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Sample records for nerve sheath meningioma

  1. Optic Nerve Sheath Meningiomas.

    PubMed

    Radhakrishnan, Sunita; Lee, Michael S

    2005-01-01

    Optic nerve sheath meningiomas (ONSMs) grow slowly and, if untreated, patients may have stable visual function for up to several years. Treatment of an ONSM may lead to vision loss (radiation retinopathy or optic neuropathy). Therefore, observation is recommended for a patient with ONSM and relatively preserved visual acuity, color vision, pupils, and visual fields. Follow-up every 4 to 6 months initially is recommended extending to annual examinations if visual function and tumor size remain stable for a few years. Neuroimaging can be repeated every 12 months. An undisputed decline in visual function or any intracranial extension warrants treatment of the ONSM. The treatment of choice for a tumor confined to the orbit is stereotactic fractionated radiation. Stereotactic fractionated radiation uses multiple small doses of radiation using tight margins. A reasonable alternative, three-dimensional conformal fractionated radiation uses computed tomography-guided planning but usually requires wider margins. Conventional radiation uses much wider margins and would not be recommended for treatment of ONSM. The radiation can be administered during 5 to 6 weeks in 28 daily fractions of 1.8 to 2 Gy/fraction to a total of 50.4 to 56 Gy. Many patients have improvement or stabilization of their visual function. Gamma knife radiosurgery does not have a role in ONSM because the required dose is toxic to the optic nerve. A tumor that extends intracranially may be treated with fractionated radiation if any vision remains. Surgical excision can be considered for significant intracranial extension but this often leads to complete vision loss in the ipsilateral eye. A blind, disfigured eye also may be treated with en bloc surgical resection of the meningioma.

  2. Diagnosis of optic nerve sheath meningioma during optic nerve sheath decompression.

    PubMed

    Meeker, Austin R; Ko, Melissa W; Carruth, Bryant P; Strumpf, Kenneth B; Bersani, Thomas A

    2017-02-01

    Optic nerve sheath meningioma (ONSM) is typically diagnosed based on clinical suspicion and imaging characteristics and is most often treated with radiation. Historically, biopsy, optic nerve sheath decompression, and debulking surgeries have been avoided for fear of optic nerve vascular disruption and tumor spread into the orbit. This is a case of a 48-year-old man who presented with unilateral optic disc edema, declining visual acuity, and a visual field defect. Despite an initial improvement with acetazolamide, his vision subsequently worsened. With an elevated lumbar puncture opening pressure and imaging showing right optic nerve sheath enhancement, the differential diagnosis included ONSM, perineuritis and idiopathic intracranial hypertension (IIH). Optic nerve sheath decompression (ONSD) with biopsy was performed, simultaneously decompressing the nerve and yielding a sample for pathologic analysis. A pathologic diagnosis of ONSM was made and treatment with radiation was subsequently initiated, but vision began to improve after the surgical decompression alone.

  3. Cystic change in primary paediatric optic nerve sheath meningioma.

    PubMed

    Narayan, Daniel; Rajak, Saul; Patel, Sandy; Selva, Dinesh

    2016-08-01

    Primary optic nerve sheath meningiomas (PONSM) are rare in children. Cystic meningiomas are an uncommon subgroup of meningiomas. We report a case of paediatric PONSM managed using observation alone that underwent cystic change and radiological regression. A 5-year-old girl presented with visual impairment and proptosis. Magnetic resonance (MR) imaging demonstrated a PONSM. The patient was left untreated and followed up with regular MR imaging. Repeat imaging at 16 years of age showed the tumour had started to develop cystic change. Repeat imaging at 21 years of age showed the tumour had decreased in size.

  4. Optic nerve sheath meningioma: current diagnosis and treatment.

    PubMed

    Eddleman, Christopher S; Liu, James K

    2007-01-01

    Optic nerve sheath meningiomas (ONSMs) are rare tumors of the anterior visual pathway and constitute approximately 2% of all orbital tumors and 1-2% of all meningiomas. Untreated ONSMs almost always lead to progressive visual decline, color blindness, and finally complete loss of vision. Although resection is warranted in cases of widespread ONSM, surgery can lead to significant morbidity. Recently, stereotactic fractionated radiotherapy has shown effectiveness in improving or stabilizing remaining visual function with minimal procedural morbidity in patients with ONSM. The authors review the incidence, histopathological characteristics, clinical presentation, neuroimaging findings, and current treatment modalities for ONSMs, with an emphasis on fractionated stereotactic radiotherapy.

  5. Optic Nerve Sheath Meningioma Masquerading as Optic Neuritis

    PubMed Central

    Alroughani, R.; Behbehani, R.

    2016-01-01

    Optic neuritis is a common presentation of demyelinating disorders such as multiple sclerosis. It typically presents with acute painful monocular vision loss, whereas chronic optic neuropathy can be caused by compressive lesions along the anterior visual pathway, genetic, toxic, or nutritional causes. We report an unusual presentation mimicking optic neuritis, which was subsequently diagnosed as optic nerve sheath meningioma (ONSM). Misinterpretation of white matter lesions on MRI of brain and the failure to image the optic nerves at the time of acute loss of vision led to the misdiagnosis of optic neuritis in this case. A comprehensive accurate history and ordering the appropriate imaging modality remain paramount in diagnosing progressive visual deterioration. PMID:26904329

  6. Fractionated Stereotactic Radiotherapy in Patients With Optic Nerve Sheath Meningioma

    SciTech Connect

    Paulsen, Frank; Doerr, Stefan; Wilhelm, Helmut; Becker, Gerd; Bamberg, Michael; Classen, Johannes

    2012-02-01

    Purpose: To evaluate the effectiveness of fractionated stereotactic radiotherapy (SFRT) in the treatment of optic nerve sheath meningioma (ONSM). Methods and Materials: Between 1993 and 2005, 109 patients (113 eyes) with primary (n = 37) or secondary (n = 76) ONSM were treated according to a prospective protocol with SFRT to a median dose of 54 Gy. All patients underwent radiographic, ophthalmologic, and endocrine analysis before and after SFRT. Radiographic response, visual control, and late side effects were endpoints of the analysis. Results: Median time to last clinical, radiographic, and ophthalmologic follow up was 30.2 months (n = 113), 42.7 months (n = 108), and 53.7 months (n = 91), respectively. Regression of the tumor was observed in 5 eyes and progression in 4 eyes, whereas 104 remained stable. Visual acuity improved in 12, deteriorated in 11, and remained stable in 68 eyes. Mean visual field defects reduced from 33.6% (n = 90) to 17.8% (n = 56) in ipsilateral and from 10% (n = 94) to 6.7% (n = 62) in contralateral eyes. Ocular motility improved in 23, remained stable in 65, and deteriorated in 3 eyes. Radiographic tumor control was 100% at 3 years and 98% at 5 years. Visual acuity was preserved in 94.8% after 3 years and in 90.9% after 5 years. Endocrine function was normal in 90.8% after 3 years and in 81.3% after 5 years. Conclusions: SFRT represents a highly effective treatment for ONSM. Interdisciplinary counseling of the patients is recommended. Because of the high rate of preservation of visual acuity we consider SFRT the standard approach for the treatment of ONSM. Prolonged observation is warranted to more accurately assess late visual impairment. Moderate de-escalation of the radiation dose might improve the preservation of visual acuity and pituitary gland function.

  7. Staged image guided robotic radiosurgery for optic nerve sheath meningiomas.

    PubMed

    Romanelli, Pantaleo; Bianchi, Livia; Muacevic, Alexander; Beltramo, Giancarlo

    2011-01-01

    Optic nerve sheath meningiomas (ONSMs) represent the most challenging lesions involving the optic pathways: Microsurgery is not indicated and classical single-stage radiosurgery appears to be too risky due to the expected destruction of the common blood supply with consequent loss of vision. Staged radiosurgery might be one treatment option because it exploits the ability of normal tissues to repair sub-lethal radiation-induced damage, offering a chance to control tumor growth while sparing function. Staged robotic radiosurgery was offered to 5 patients harboring ONSMs with the aim of sparing vision while achieving local growth control. Five patients with ONSM presenting with visual field deficits and loss of visual acuity were treated with staged CyberKnife radiosurgery, receiving 20 Gy in 4 stages (5 Gy per stage). Treatment planning was based on contrast-enhanced thin-slice CT (1.25 mm thickness for the first three cases, 0.5 mm for the last two) and volumetric MR imaging (1.5 T for the first three cases, 3 T for the last two). An interval of 24 hours was strictly observed between stages. Visual acuity and visual fields were assessed in all patients immediately prior to treatment and at intervals of 6 months thereafter. Follow-up MRIs were performed every 6 months for 2 years, then once per year. The entire procedure, inclusive of imaging, treatment planning and treatment delivery, was performed in 5 days. Irradiation required approximately 45 min per stage. Mean tumor volume was 2.94 cc (range: 0.8-6.4 cc). Treatment was well tolerated in all patients. Follow-up ranged from 36 to 74 months. Local growth control was achieved in all patients. Restoration of normal vision was experienced by 4 patients 6 to 12 months after the treatment. One patient, who was also affected by diabetic retinopathy, showed a modest improvement after 6 months, remaining stable thereafter. Staged CyberKnife radiosurgery provides a fast and well-tolerated non

  8. Optic nerve compartment syndrome in a patient with optic nerve sheath meningioma.

    PubMed

    Jaggi, G P; Mironov, A; Huber, A R; Killer, H E

    2007-01-01

    To report a patient with optic nerve (ON) sheath meningioma, unilateral optic disc swelling, and inhomogeneous cerebrospinal fluid (CSF) composition between lumbar CSF and CSF from the subarachnoid space (SAS) of the affected ON. A 39-year-old woman presented with unilateral optic disc swelling and slight deterioration of visual function in the left eye. Extensive laboratory workup and magnetic resonance imaging (MRI) of the brain and orbits were performed. As radiotherapy was refused by the patient, ON sheath fenestration (ONSF) was offered and performed in order to stop deterioration. CSF from the SAS of the ON was sampled. Laboratory workup was within normal limits. MRI of the left orbit demonstrated enhancement of the dura in the precanalicular portion of the ON and distension of the SAS, most prominent in the bulbar portion of the ON. On lumbar puncture the opening pressure measured 19 (cm H2O). Compared to the lumbar CSF the CSF of the affected ON SAS showed markedly elevated measurements for albumin, IgG, and beta-trace protein. Visual function remained stable over a follow-up time of 18 months. Composition of CSF is considered to be homogenous throughout all CSF spaces. In this patient the authors found a marked concentration-gradient of albumin, IgG, and beta-trace protein between the CSF in the spinal canal and the CSF in the SAS of the affected ON. Based on the radiologic features of the left ON and the dissociated beta-trace protein concentrations in the CSF of the SAS of the ON and the lumbar CSF, the diagnosis of an ON sheath compartment syndrome due to an ON sheath meningioma was made.

  9. Alterations of oxidative phosphorylation in meningiomas and peripheral nerve sheath tumors.

    PubMed

    Feichtinger, René G; Weis, Serge; Mayr, Johannes A; Zimmermann, Franz A; Bogner, Barbara; Sperl, Wolfgang; Kofler, Barbara

    2016-02-01

    Changes in the mode of aerobic energy production are observed in many solid tumors, though the kinds of changes differ among tumor types. We investigated mitochondrial energy metabolism in meningiomas and peripheral nerve sheath tumors, taking into consideration the histologic heterogeneity of these tumors. Oxidative phosphorylation (OXPHOS) complexes and porin (a marker for mitochondrial mass) were analyzed by immunohistochemical staining of meningiomas (n = 76) and peripheral nerve sheath tumors (schwannomas: n = 10; neurofibromas: n = 4). The enzymatic activities of OXPHOS complexes and citrate synthase were determined by spectrophotometric measurement. Western blot analysis of OXPHOS complexes, porin, and mitochondrial transcription factor A was performed. Furthermore, mitochondrial DNA copy number was determined. The tumors differed with regard to mitochondrial energy metabolism. Low levels of a subset of OXPHOS complexes were frequently observed in World Health Organization grade I meningiomas (percent of cases with a reduction; complex I: 63%; complex II: 67%; complex IV: 56%) and schwannomas (complex III: 40%, complex IV: 100%), whereas in neurofibromas a general reduction of all complexes was observed. In contrast, expression of complexes III and V was similar to that in normal brain tissue in the majority of tumors. Mitochondrial mass was comparable or higher in all tumors compared with normal brain tissue, whereas mitochondrial DNA copy number was reduced. The reduction of OXPHOS complexes in meningiomas and peripheral nerve sheath tumors has potential therapeutic implications, since respiratory chain-deficient tumor cells might be selectively starved by inhibitors of glycolysis or by ketogenic diet. © The Author(s) 2015. Published by Oxford University Press on behalf of the Society for Neuro-Oncology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

  10. Optic nerve sheath meningioma detected by single- photon emission computed tomography/computed tomography somatostatin receptor scintigraphy: a case report.

    PubMed

    Nussbaum-Hermassi, Lucie; Ahle, Guido; Zaenker, Chistophe; Duca, Camelia; Namer, Izzie Jacques

    2016-04-22

    Optic nerve sheath meningiomas account for only 2% of orbital lesions and 42% of optic nerve tumors. Diagnosis remains difficult because histologic confirmation carries a high risk of visual loss. Therefore, a less invasive and specific diagnostic method for differentiating optic nerve sheath meningiomas from other optic nerve lesions is needed to overcome the limitations of computed tomography and magnetic resonance imaging, and make the best individualized treatment decision. This case is a good illustration of the clinical and imaging difficulties inherent in this rare tumor, which may be hard to differentiate from other causes. A 51-year-old Caucasian woman developed a central scotoma, visual loss, and abnormal visual evoked potentials. The first magnetic resonance imaging scan classified the optic nerve damage as retrobulbar optic neuritis. After magnetic resonance imaging follow-up at 3 months, a negative lumbar puncture and biological workup, and clinical worsening, an optic nerve sheath meningioma was suspected. We confirmed this diagnosis with 111In-pentetreotide single-photon emission computed tomography, which is able to bind with very high affinity to somatostatin receptor subtype 2 expressed on meningiomas. In the diagnosis of optic nerve sheath meningiomas, [111In]-pentetreotide single-photon emission computed tomography-fused magnetic resonance imaging is a valuable additional tool, optimizing the diagnosis and obviating the need for a more invasive procedure.

  11. Analyses on the misdiagnoses of 25 patients with unilateral optic nerve sheath meningioma

    PubMed Central

    Mao, Jun-Feng; Xia, Xiao-Bo; Tang, Xiang-Bo; Zhang, Xue-Yong; Wen, Dan

    2016-01-01

    AIM To investigate clinical features of optic nerve sheath meningioma (ONSM) that was misdiagnosed, and to find methods to reduce the misdiagnoses. METHODS Retrospective series study. Twenty-five misdisgnosed patients with unilateral ONSM were collected from Jan. 2008 to Jan. 2015 and the clinical records reviewed. RESULTS Patients were misdiagnosed with acute papillitis most frequently (n=17), immediately followed by optic atrophy (n=8), ischemic optic neuropathy (n=5), acute retrobulbar optic neuritis (n=5), optic disc vasculitis (n=3). For each patient, the minimum frequency of misdiagnoses was once and the maximum was 4 times. As for the lasting time of being misdiagnosed, the shortest was 1.5mo and the longest was 45mo. Twenty-one cases (84%) were once treated with glucocorticoids, and its side effects was found in seventeen patients. Twenty patients (80%) complained with varying degree of vision loss. When a definite diagnosis was made, sixteen cases (64%) showed slight exophthalmos and eighteen cases (72%) had the tubular ONSM. CONCLUSION ONSM without loss obvious exophthalmos is easily misdiagnosed in clinic, and for most of these ONSMs are tubular. PMID:27672598

  12. Visual Outcome and Tumor Control After Conformal Radiotherapy for Patients With Optic Nerve Sheath Meningioma

    SciTech Connect

    Arvold, Nils D.; Lessell, Simmons; Bussiere, Marc; Beaudette, Kevin; Rizzo, Joseph F.; Loeffler, Jay S.; Shih, Helen A.

    2009-11-15

    Purpose: Optic nerve sheath meningioma (ONSM) is a rare tumor that almost uniformly leads to visual dysfunction and even blindness without intervention. Because surgical extirpation carries a high risk of postoperative blindness, vision-sparing treatment strategies are desirable. Methods and Materials: We retrospectively reviewed the outcomes of 25 patients (25 optic nerves) with ONSM, treated at a single institution with conformal fractionated radiotherapy by either stereotactic photon or proton radiation. Primary endpoints were local control and visual acuity. Results: The patients presented with symptoms of visual loss (21) or orbital pain (3) or were incidentally diagnosed by imaging (3). The mean age was 44 years, and 64% were female patients. The indication for treatment was the development or progression of symptoms. Of the patients, 13 were treated with photons, 9 were treated with protons, and 3 received a combination of photons and protons. The median dose delivered was 50.4 gray equivalents (range, 45-59.4 gray equivalents). Median follow-up after radiotherapy was 30 months (range, 3-168 months), with 3 patients lost to follow-up. At most recent follow-up, 21 of 22 patients (95%) had improved (14) or stable (7) visual acuity. One patient had worsened visual acuity after initial postirradiation improvement. Of the 22 patients, 20 (95%) had no radiographic progression. Three patients had evidence of asymptomatic, limited retinopathy on ophthalmologic examination, and one had recurrent ONSM 11 years after treatment. Conclusions: Highly conformal, fractionated radiation therapy for symptomatic primary ONSM provides tumor control and improvement in visual function in most cases, with minimal treatment-induced morbidity. Longer follow-up is needed to assess the durability of tumor control and treatment-related late effects.

  13. Standard-Fractionated Radiotherapy for Optic Nerve Sheath Meningioma: Visual Outcome Is Predicted by Mean Eye Dose

    SciTech Connect

    Abouaf, Lucie; Girard, Nicolas; Lefort, Thibaud; D'hombres, Anne; Tilikete, Caroline; Vighetto, Alain; Mornex, Francoise

    2012-03-01

    Purpose: Radiotherapy has shown its efficacy in controlling optic nerve sheath meningiomas (ONSM) tumor growth while allowing visual acuity to improve or stabilize. However, radiation-induced toxicity may ultimately jeopardize the functional benefit. The purpose of this study was to identify predictive factors of poor visual outcome in patients receiving radiotherapy for ONSM. Methods and Materials: We conducted an extensive analysis of 10 patients with ONSM with regard to clinical, radiologic, and dosimetric aspects. All patients were treated with conformal radiotherapy and subsequently underwent biannual neuroophthalmologic and imaging assessments. Pretreatment and posttreatment values of visual acuity and visual field were compared with Wilcoxon's signed rank test. Results: Visual acuity values significantly improved after radiotherapy. After a median follow-up time of 51 months, 6 patients had improved visual acuity, 4 patients had improved visual field, 1 patient was in stable condition, and 1 patient had deteriorated visual acuity and visual field. Tumor control rate was 100% at magnetic resonance imaging assessment. Visual acuity deterioration after radiotherapy was related to radiation-induced retinopathy in 2 patients and radiation-induced mature cataract in 1 patient. Study of radiotherapy parameters showed that the mean eye dose was significantly higher in those 3 patients who had deteriorated vision. Conclusions: Our study confirms that radiotherapy is efficient in treating ONSM. Long-term visual outcome may be compromised by radiation-induced side effects. Mean eye dose has to be considered as a limiting constraint in treatment planning.

  14. Somatostatin Receptor SPECT/CT using 99mTc Labeled HYNIC-TOC Aids in Diagnosis of Primary Optic Nerve Sheath Meningioma

    PubMed Central

    Chandra, Piyush; Purandare, Nilendu; Shah, Sneha; Agrawal, Archi; Rangarajan, Venkatesh

    2017-01-01

    Primary optic nerve sheath meningiomas (ONSM) are rare, benign and slow growing tumor involving the intra-orbital/intra-canalicular segment of the optic nerve. Untreated, they can potentially lead to visual deterioration. Magnetic resonance (MR) is the gold standard imaging modality for diagnosing the entity. Often, a clinical dilemma exists to narrow the differential diagnosis of an enhancing intra-orbital mass on MR. Molecular imaging provides a high degree of precision in diagnosing meningioma in view of relatively high levels of somatostatin receptor expression by these tumors. The following case demonstrates the potential clinical utility of somatostatin receptor SPECT using 99mTc- labeled HYNIC-TOC in clinical diagnosis of ONSM. PMID:28242992

  15. Somatostatin Receptor SPECT/CT using (99m)Tc Labeled HYNIC-TOC Aids in Diagnosis of Primary Optic Nerve Sheath Meningioma.

    PubMed

    Chandra, Piyush; Purandare, Nilendu; Shah, Sneha; Agrawal, Archi; Rangarajan, Venkatesh

    2017-01-01

    Primary optic nerve sheath meningiomas (ONSM) are rare, benign and slow growing tumor involving the intra-orbital/intra-canalicular segment of the optic nerve. Untreated, they can potentially lead to visual deterioration. Magnetic resonance (MR) is the gold standard imaging modality for diagnosing the entity. Often, a clinical dilemma exists to narrow the differential diagnosis of an enhancing intra-orbital mass on MR. Molecular imaging provides a high degree of precision in diagnosing meningioma in view of relatively high levels of somatostatin receptor expression by these tumors. The following case demonstrates the potential clinical utility of somatostatin receptor SPECT using (99m)Tc- labeled HYNIC-TOC in clinical diagnosis of ONSM.

  16. Radiation therapy for primary optic nerve meningiomas.

    PubMed

    Smith, J L; Vuksanovic, M M; Yates, B M; Bienfang, D C

    1981-06-01

    Optic nerve sheath meningiomas, formerly thought to be rare, have been encountered with surprising frequency since the widespread use of computed tomography. Early diagnosis led to an enthusiastic surgical approach to these lesions, but this has been tempered by the realization that even in the best of hands, blindness followed such surgery with distressing frequency. Optic nerve sheath meningiomas may be divided into primary, secondary, and multiple meningioma groups. Five patients with primary optic nerve sheath meningiomas treated with irradiation therapy are presented in this report. Improvement in visual acuity, stabilization to increase in the visual field, and decrease in size to total regression of optociliary veins, have been documented following irradiation therapy of the posterior orbital and intracanalicular portions of the optic nerve in some of these cases. Although each patient must be carefully individualized, there is no question that visual palliation can be achieved in some cases of optic nerve sheath meningioma. Further investigation of this therapeutic modality in selected cases in advised.

  17. Fractionated Conformal Radiotherapy for Management of Optic Nerve Sheath Meningiomas: Long-Term Outcomes of Tumor Control and Visual Function at a Single Institution

    SciTech Connect

    Metellus, Philippe; Kapoor, Sumit; Kharkar, Siddharth; Batra, Sachin; Jackson, Juan F.; Kleinberg, Lawrence; Miller, Neil R.; Rigamonti, Daniele

    2011-05-01

    Purpose: To provide the long-term outcomes of patients treated with fractionated conformal radiotherapy (FCRT) for presumed optic nerve sheath meningiomas (ONSMs). Patients and Methods: Between 1995 and 2002, 9 patients with a presumed ONSM were treated with FCRT at our institution. The indications for FCRT were significant visual dysfunction at presentation, progression of visual dysfunction during a period of observation, tumor growth documented by sequential imaging, or a combination of these findings. In 2 patients, FCRT was performed as adjuvant therapy, and in 7, it was the initial and primary treatment. Results: Of the 9 patients, 6 were women and 3 were men, with a mean age of 47 years. All 9 patients had evidence of optic nerve dysfunction in the affected eye, characterized by reduced visual acuity, a visual field defect, and a relative afferent pupillary defect. In addition, 2 patients had proptosis and 1 had diplopia. The mean follow-up period was 98 {+-} 31.7 months (median, 90; range, 61-151). After FCRT, the visual function improved in the 7 patients who had undergone FCRT as the primary treatment. However, 2 patients who were blind in their affected eye at FCRT remained blind. In 4 of the 7 patients with improvement, the improvement was documented within 1-3 months after FCRT. The tumor control rate was 100%. Proptosis and diplopia also regressed in 100% of patients. At 2 years after FCRT, 1 patient had developed radiation retinopathy. Conclusion: The results of our study have shown that FCRT is a safe and effective treatment of ONSMs, affording satisfactory long-term tumor control, good functional outcome, and low treatment morbidity. FCRT should be considered the treatment of choice for patients with presumed ONSMs for whom the treatment has been deemed appropriate.

  18. Meningioma

    MedlinePlus

    ... brain tissue, nerves or vessels may cause serious disability. Meningiomas occur most commonly in women, and are often discovered at older ages, but ... meningioma. Female hormones. Meningiomas are more common in women, ... tumors. Obesity. A high BMI (body mass index) is an established risk ...

  19. Intrasellar malignant peripheral nerve sheath tumor (MPNST).

    PubMed

    Krayenbühl, N; Heppner, F; Yonekawa, Y; Bernays, R L

    2007-02-01

    Intracranial malignant peripheral nerve sheath tumors (MPNST) and intrasellar schwannomas are rare tumors. We describe a case of an intrasellar schwannoma with progression to a MPNST, a finding that, although very rare, extends the differential diagnosis of intrasellar lesions.

  20. Malignant Peripheral Nerve Sheath Tumors.

    PubMed

    Durbin, Adam D; Ki, Dong Hyuk; He, Shuning; Look, A Thomas

    2016-01-01

    Malignant peripheral nerve sheath tumors (MPNST) are tumors derived from Schwann cells or Schwann cell precursors. Although rare overall, the incidence of MPNST has increased with improved clinical management of patients with the neurofibromatosis type 1 (NF1) tumor predisposition syndrome. Unfortunately, current treatment modalities for MPNST are limited, with no targeted therapies available and poor efficacy of conventional radiation and chemotherapeutic regimens. Many murine and zebrafish models of MPNST have been developed, which have helped to elucidate the genes and pathways that are dysregulated in MPNST tumorigenesis, including the p53, and the RB1, PI3K-Akt-mTOR, RAS-ERK and Wnt signaling pathways. Preclinical results have suggested that new therapies, including mTOR and ERK inhibitors, may synergize with conventional chemotherapy in human tumors. The discovery of new genome editing technologies, like CRISPR-cas9, and their successful application to the zebrafish model will enable rapid progress in the faithful modeling of MPNST molecular pathogenesis. The zebrafish model is especially suited for high throughput screening of new targeted therapeutics as well as drugs approved for other purposes, which may help to bring enhanced treatment modalities into human clinical trials for this devastating disease.

  1. Dural ectasia of the optic nerve sheath

    PubMed Central

    Kacem, Hanane Hadj; Hammani, Lehcen; Ajana, Ali; Nassar, Itimad

    2014-01-01

    Optic nerve dural ectasia is a rare cause of optic nerve sheath enlargement due to the accumulation of CSF around the optic nerve with no associated pathology. It diagnosed by MRI studies and can follow benign or sometimes an unfavorable course. We describe the case of a 24-day-old female referred for a visual blurring, which we diagnosed as a dural ectasia of the optic nerve sheath by MRI and confirmed in surgical intervention. We present this case report to illustrate the classic imaging features of the disease. PMID:25374645

  2. Optic Nerve Sheath Mechanics in VIIP Syndrome

    NASA Technical Reports Server (NTRS)

    Raykin, Julia; Feola, Andrew; Gleason, Rudy; Mulugeta, Lealem; Myers, Jerry; Nelson, Emily; Samuels, Brian; Ethier, C. Ross

    2015-01-01

    Visual Impairment and Intracranial Pressure (VIIP) syndrome results in a loss of visual function and occurs in astronauts following long-duration spaceflight. Understanding the mechanisms that lead to the ocular changes involved in VIIP is of critical importance for space medicine research. Although the exact mechanisms of VIIP are not yet known, it is hypothesized that microgravity-induced increases in intracranial pressures (ICP) drive the remodeling of the optic nerve sheath, leading to compression of the optic nerve which in turn may reduce visual acuity. Some astronauts present with a kink in the optic nerve after return to earth, suggesting that tissue remodeling in response to ICP increases may be taking place. The goal of this work is to characterize the mechanical properties of the optic nerve sheath (dura mater) to better understand its biomechanical response to increased ICP.

  3. Penetration and Splitting of Optic Nerve by Tuberculum Sellae Meningioma

    PubMed Central

    Park, Seong-Cheol

    2016-01-01

    Preservation of the optic nerves is an important issue in the resection of tuberculum sellae meningiomas. We report the case of a patient whose optic nerve was penetrated by a tuberculum sellae meningioma. During surgery, a bulging tumor was found to penetrate the right optic nerve. The tumor was gross totally removed, including tumors bulging through the optic nerve. Two trunks of the split optic nerve were preserved. The penetrated shape of the optic nerve may lead to misjudgment and its damage. Divided trunks of optic nerves are difficult to recognize and may be confused for the tumor capsule, because they may be thinned and seem to contain tumors. In addition, a single trunk may be confused for the whole nerve; thus, the other trunk may be easily damaged if not dissected cautiously. Treatment strategy according to the remnant visual acuity was suggested. PMID:27651874

  4. Optic Nerve Sheath Mechanics in VIIP Syndrome

    NASA Technical Reports Server (NTRS)

    Raykin, Julia; Forte, Taylor E.; Wang, Roy; Feola, Andrew; Samuels, Brian; Myers, Jerry; Nelson, Emily; Gleason, Rudy; Ethier, C. Ross

    2016-01-01

    Visual Impairment Intracranial Pressure (VIIP) syndrome is a major concern in current space medicine research. While the exact pathology of VIIP is not yet known, it is hypothesized that the microgravity-induced cephalad fluid shift increases intracranial pressure (ICP) and drives remodeling of the optic nerve sheath. To investigate this possibility, we are culturing optic nerve sheath dura mater samples under different pressures and investigating changes in tissue composition. To interpret results from this work, it is essential to first understand the biomechanical response of the optic nerve sheath dura mater to loading. Here, we investigated the effects of mechanical loading on the porcine optic nerve sheath.Porcine optic nerves (number: 6) were obtained immediately after death from a local abattoir. The optic nerve sheath (dura mater) was isolated from the optic nerve proper, leaving a hollow cylinder of connective tissue that was used for biomechanical characterization. We developed a custom mechanical testing system that allowed for unconfined lengthening, twisting, and circumferential distension of the dura mater during inflation and under fixed axial loading. To determine the effects of variations in ICP, the sample was inflated (0-60 millimeters Hg) and circumferential distension was simultaneously recorded. These tests were performed under variable axial loads (0.6 grams - 5.6 grams at increments of 1 gram) by attaching different weights to one end of the dura mater. Results and Conclusions: The samples demonstrated nonlinear behavior, similar to other soft connective tissue (Figure 1). Large increases in diameter were observed at lower transmural pressures (approximately 0 to 5 millimeters Hg), whereas only small diameter changes were observed at higher pressures. Particularly interesting was the existence of a cross-over point at a pressure of approximately 11 millimeters Hg. At this pressure, the same diameter is obtained for all axial loads applied

  5. Coincidental Optic Nerve Meningioma and Thyroid Eye Disease.

    PubMed

    Garg, Aakriti; Patel, Payal; Lignelli, Angela; Baron, Edward; Kazim, Michael

    2015-01-01

    A 57-year-old woman with diabetes mellitus, hypertension, obesity, and Graves disease presented with clinical evidence of thyroid eye disease (TED) and optic neuropathy. She was referred when a tapered dose of steroids prompted worsening of her TED. CT and MRI were consistent with TED and bilateral optic nerve meningioma. To the authors' knowledge, this is the first reported case of concurrent TED and unsuspected bilateral optic nerve meningioma. When investigating the etiology of TED-associated optic neuropathy, careful attention to orbital imaging is required because coexisting pathology may exist.

  6. ULTRASTRUCTURE OF THE PRAWN NERVE SHEATHS

    PubMed Central

    Doggenweiler, C. F.; Heuser, John E.

    1967-01-01

    The sheaths from freshly teased nerve fibers of the prawn exhibit a positive radial birefringence, consistent with their EM appearance as highly organized laminated structures composed of numerous thin cytoplasmic sheets or laminae bordered by unit membranes and arranged concentrically around the axon. The closely apposed membranes in these sheaths are fragile and often break down into rows of vesicles during fixation. Desmosome-like attachment zones occur in many regions of the sheath. The membranes within these zones resist vesiculation and thereby provide a "control" region for relating the type of vesicles formed in the fragile portions of the sheaths to the specific fixation conditions. It is proposed that during fixation the production of artifactual vesicles is governed by an interplay of three factors: (a) direct chemical action of the fixative on the polar strata of adjacent unit membranes, (b) osmotic forces applied to membranes during fixation, and (c) the pre-existing natural relations between adjacent membranes. It is found that permanganate best preserves the continuity of the membranes but will still produce vesicles if the fixative exerts severe osmotic forces. These results support other reports (19) of the importance of comparing tissues fixed by complementary procedures so that systematic artifacts will not be described as characteristic of the natural state. PMID:4166578

  7. Nerve Sheath Myxoma: Report of A Rare Case

    PubMed Central

    Bhat, Amoolya; C, Vijaya; VK, Sundeep

    2015-01-01

    Nerve sheath myxoma defined by Harkin and Reed is an uncommon benign neoplasm with nerve sheath like features. It has several cytological and histological differential diagnoses. One such lesion is neurothekeoma, which can be differentiated using immunohistochemistry. In most of the previous reports nerve sheath myxoma and neurothekeoma were considered synonymous and were often confused for one another. This case report separates the two using immunohistochemistry. Also, the cytological features of nerve sheath myxoma are not well documented in the past. This case report attempts to display the cyto-morphology of nerve sheath myxoma. We report a rare case of nerve sheath myxoma diagnosed on cytological features confirmed by histopathology and immunohistochemistry in a 32-year-old lady who presented with an asymptomatic nodule over the left cervical area and discuss its cyto-histological mimics. PMID:26023558

  8. Nerve sheath myxoma: report of a rare case.

    PubMed

    Bhat, Amoolya; Narasimha, Apaparna; C, Vijaya; Vk, Sundeep

    2015-04-01

    Nerve sheath myxoma defined by Harkin and Reed is an uncommon benign neoplasm with nerve sheath like features. It has several cytological and histological differential diagnoses. One such lesion is neurothekeoma, which can be differentiated using immunohistochemistry. In most of the previous reports nerve sheath myxoma and neurothekeoma were considered synonymous and were often confused for one another. This case report separates the two using immunohistochemistry. Also, the cytological features of nerve sheath myxoma are not well documented in the past. This case report attempts to display the cyto-morphology of nerve sheath myxoma. We report a rare case of nerve sheath myxoma diagnosed on cytological features confirmed by histopathology and immunohistochemistry in a 32-year-old lady who presented with an asymptomatic nodule over the left cervical area and discuss its cyto-histological mimics.

  9. Malignant peripheral nerve sheath tumour of penis.

    PubMed

    Kaur, J; Madan, R; Singh, L; Sharma, D N; Julka, P K; Rath, G K; Roy, S

    2015-04-01

    Malignant peripheral nerve sheath tumour (MPNST) is a rare variety of soft tissue sarcoma that originates from Schwann cells or pluripotent cells of neural crest origin. They have historically been difficult tumours to diagnose and treat. Surgery is the mainstay of treatment with a goal to achieve negative margins. Despite aggressive surgery and adjuvant therapy, the prognosis of patients with MPNST remains poor. MPNST arising from penis is a very rare entity; thus, it presents a diagnostic and therapeutic challenge. We present a case of penile MPNST in a 38-year-old man in the absence of neurofibromatosis treated with surgery followed by post-operative radiotherapy to a dose of 60 Gray in 30 fractions and adjuvant chemotherapy with ifosfamide and adriamycin.

  10. Radiation-induced malignant and atypical peripheral nerve sheath tumors

    SciTech Connect

    Foley, K.M.; Woodruff, J.M.; Ellis, F.T.; Posner, J.B.

    1980-04-01

    The reported peripheral nerve complications of therapeutic irradiation in humans include brachial and lumbar plexus fibrosis and cranial and peripheral nerve atrophy. We have encountered 9 patients with malignant (7) and atypical (2) peripheral nerve tumors occurring in an irradiated site suggesting that such tumors represent another delayed effect of radiation treatment on peripheral nerve. In all instances the radio-theray was within an acceptable radiation dosage, yet 3 patients developed local radiation-induced skin and bony abnormalities. The malignant peripheral nerve sheath tumors developed only in the radiation port. Animal studies support the clinical observation that malignant peripheral nerve sheath tumors can occur as a delayed effect of irradiation.

  11. Melanocytic Malignant Peripheral Nerve Sheath Tumor of the Male Breast.

    PubMed

    Wang, Haijun; Ge, Jing; Chen, Lirong; Xie, Panpan; Chen, Fangfang; Chen, Yiding

    2009-01-01

    SUMMARY: BACKGROUND: Malignant peripheral nerve sheath tumors are rare tumor entities that originate from peripheral nerve sheaths and have an unfavorable prognosis. Common sites include deeper soft tissues, usually in the proximity of a nerve trunk. Breast is an absolutely rare location of this lesion, and presentation as a breast lump in the male breast is even rarer. CASE REPORT: A 65-year-old man presented with a 6-month history of a painless mass of the left breast. Tissue biopsy was performed. Histopathology revealed a malignant spindle cell tumor which was confirmed to be a melanocytic malignant peripheral nerve sheath tumor on the basis of immunopositivity for HMB45 and S-100. CONCLUSION: There are no generally accepted guidelines for the treatment of malignant peripheral nerve sheath tumors in the male breast. The patient was referred for radiation therapy after simple mastectomy.

  12. Synovial sarcoma mimicking benign peripheral nerve sheath tumor.

    PubMed

    Larque, Ana B; Bredella, Miriam A; Nielsen, G Petur; Chebib, Ivan

    2017-07-08

    To assess the radiographic and clinicopathologic features of synovial sarcoma of the nerve that were clinically or radiologically interpreted as benign peripheral nerve sheath tumor. Five patients with synovial sarcoma arising from the peripheral nerve and interpreted clinically and radiologically as peripheral nerve sheath tumors were identified. Clinicopathologic and imaging features were evaluated. There were three females and two males, ranging in age from 28 to 50 (mean 35.8) years. Most patients (4/5) complained of a mass, discomfort or pain. MR images demonstrated a heterogeneous, enhancing, soft tissue mass contiguous with the neurovascular bundle. On histologic examination, most tumors were monophasic synovial sarcoma (4/5). At the time of surgery, all tumors were noted to arise along or within a peripheral nerve. All patients were alive with no evidence of disease with median follow-up of 44 (range 32-237) months. For comparison, approximately 775 benign peripheral nerve sheath tumors of the extremities were identified during the same time period. Primary synovial sarcoma of the nerve can mimic peripheral nerve sheath tumors clinically and on imaging and should be included in the differential diagnosis for tumors arising from peripheral nerves.

  13. Coexistent intracerebral metastatic melanoma and meningioma.

    PubMed

    Shinde, Sweety V; Shenoy, Asha S; Savant, Hemant V; Balasubramaniam, Srikant B

    2017-01-01

    Coexistence of multifocal neural crest tumors, namely meningioma, melanoma, and nerve sheath tumors, is termed as neurocristopathy. Neurofibromatosis is the commonest form of neurocristopathy. We report a rare case of frontal lobe metastatic melanoma coexistent with a parietal lobe meningioma, in the absence of any stigmata of neurofibromatosis.

  14. Malignant peripheral nerve sheath tumour presenting with Horner's syndrome.

    PubMed

    Basuthakur, Sumitra; Sengupta, Amitava; Bandyopadhyay, Ankan; Banerjee, Arpita

    2013-09-01

    A young male presented with clinical and radiological features of right apical lung mass and Horner's syndrome. Subsequently the patient was diagnosed as a case of malignant peripheral nerve sheath tumour (MPNST) at the apex of right lung originating from an intercostal nerve and compressing ipsilateral cervical sympathetic plexus and lower cord of brachial plexus, in a case of neurofibromatosis type 1.

  15. [Transformation of trigeminal nerve tumor into malignant peripheral nerve sheath tumor (MPNST)].

    PubMed

    Nenashev, E A; Cherekaev, V A; Kadasheva, A B; Kozlov, A V; Rotin, D L; Stepanian, M A

    2012-01-01

    Malignant peripheral nerve sheath tumor (MPNST) is a rare entity with only 18 cases of trigeminal nerve MPNST described by now and only one report of malignant transformation of trigeminal nerve tumor into MPNST published up to date. One more case of malignant transformation of trigeminal nerve (1st division) tumor into MPNST is demonstrated.

  16. Peripheral Nerve Sheath Tumor of the Vagus Nerve in a Dog.

    PubMed

    Yap, Fui; Pratschke, Kathryn

    2016-01-01

    A peripheral nerve sheath tumor was diagnosed in a female, neutered Labrador retriever with a 6 mo history of coughing, retching, ptyalism, and left-sided Horner's syndrome. Computed tomography scan of the neck revealed a mass lesion between the carotid artery and esophagus in the mid-cervical region. Exploratory surgery was performed and an 18 cm section of thickened vagus nerve was excised. Histopathological findings and immunochemistry staining confirmed a malignant peripheral nerve sheath tumor. The tumor showed microscopic signs of malignancy, but there were no macroscopic signs of local extension or distant metastasis. This report documents a peripheral nerve sheath tumor of rare origin in dogs.

  17. Malignant Peripheral Nerve Sheath Tumor -A Rare Malignancy in Mandible

    PubMed Central

    Majumdar, Sumit; Kotina, Sreekanth; Uppala, Divya; Kumar, Singam Praveen

    2016-01-01

    Malignant Peripheral Nerve Sheath Tumor (MPNST) is biologically an aggressive tumor that is usually found in the extremities, trunk and infrequently found in the head and neck area particularly in the jaws, arising from the cells allied with nerve sheath. Mandibular MPNST may either arise from a preexisting neurofibroma or develop de novo. Because of the greater variability from case to case in overall appearance both clinically and histologically, a case of MPNST of the mandible in a 25-year-old female patient is reported. The lesion was excised and immunohistological studies (S-100 & Neuron specific enolase) were conducted to confirm the neural origin. PMID:27504425

  18. Leptomeningeal metastasis of an intradural malignant peripheral nerve sheath tumor.

    PubMed

    Stark, Andreas M; Mehdorn, H Maximilian

    2013-08-01

    Malignant peripheral nerve sheath tumors (MPNST) are defined as any malignant tumor arising from or differentiating towards the peripheral nerve sheath. Intradural MPNST metastases are very rare. We report, to our knowledge, the first case of leptomeningeal metastasis of a MPNST to the spine and intracranial space. A 56-year-old woman with primary intradural MPNST of the S1 nerve root developed leptomeningeal metastases as well as brain metastases 19 months after diagnosis. The patient had a history of non-Hodgkins lymphoma for which she had received irradiation to the spine 15 years prior to this presentation. She had no stigmata of neurofibromatosis type 1. Patients with MPNST may also develop leptomeningeal metastases as demonstrated in this patient with intradural post-radiation MPNST.

  19. MENINGIOMAS

    PubMed Central

    Rogers, Leland; Barani, Igor; Chamberlain, Marc; Kaley, Thomas; McDermott, Michael; Raizer, Jeffrey; Schiff, David; Weber, Damien C.; Wen, Patrick Y.; Vogelbaum, Michael A.

    2016-01-01

    Evolving interest in meningioma, the most common primary brain tumor, has refined contemporary management of these tumors. Problematic, however, is the paucity of prospective clinical trials that provide an evidence-based algorithm for managing meningioma. The current review summarizes the published literature regarding the treatment of newly diagnosed and recurrent meningioma, with an emphasis on outcomes stratified by World Health Organization (WHO) tumor grade. In particular this review focuses on patient outcomes following treatment (either adjuvant or at recurrence) with surgery or radiation therapy inclusive of radiosurgery and fractionated irradiation. Phase II trials for patients with meningioma have recently completed accrual within the Radiation Therapy Oncology Group (RTOG) and the European Organization for Research and Treatment of Cancer (EORTC) consortia, and phase III studies are being developed. However, at present, there are no completed prospective, randomized trials assessing the role of either surgery or radiotherapy. Successful completion of future studies will require a multidisciplinary effort, dissemination of the current knowledge base, improved implementation of WHO grading criteria, standardization of response criteria and other outcome endpoints, and concerted efforts to address weaknesses in present treatment paradigms, particularly for patients with progressive or recurrent low grade meningioma, or with high-grade meningioma. In parallel efforts, Response Assessment in Neuro-Oncology (RANO) subcommittees are developing a manuscript on systemic therapies for meningioma, and a separate article proposing standardized endpoint and response criteria for meningioma. PMID:25343186

  20. Effect of optic nerve sheath fenestration for idiopathic intracranial hypertension on retinal nerve fiber layer thickness.

    PubMed

    Starks, Victoria; Gilliland, Grant; Vrcek, Ivan; Gilliland, Connor

    2016-01-01

    The objective of the study was to evaluate whether optic nerve sheath fenestration in patients with idiopathic intracranial hypertension was associated with improvement in visual field pattern deviation and optical coherence tomography retinal nerve fiber layer thickness.The records of 13 eyes of 11 patients who underwent optic nerve sheath fenestration were reviewed. The subjects were patients of a clinical practice in Dallas, Texas. Charts were reviewed for pre- and postoperative visual field pattern deviation (PD) and retinal nerve fiber layer thickness (RNFL).PD and RNFL significantly improved after surgery. Average PD preoperatively was 8.51 DB and postoperatively was 4.80 DB (p = 0.0002). Average RNFL preoperatively was 113.63 and postoperatively was 102.70 (p = 0.01). The preoperative PD and RNFL did not correlate strongly.Our results demonstrate that PD and RNFL are improved after optic nerve sheath fenestration. The pre- and postoperative RNFL values were compared to the average RNFL value of healthy optic nerves obtained from the literature. Post-ONSF RNFL values were significantly closer to the normal value than preoperative. RNFL is an objective parameter for monitoring the optic nerve after optic nerve sheath fenestration. This study adds to the evidence that OCT RNFL may be an effective monitoring tool for patients with IIH and that it continues to be a useful parameter after ONSF.

  1. Therapy of Experimental Nerve Sheath Tumors Using Oncolytic Viruses

    DTIC Science & Technology

    2005-06-01

    SUPPLEMENTARY NOTES 14. ABSTRACT Abstract follows. 15. SUBJECT TERMS Oncolytic HSV, angiogenesis, MPNST , mouse model 16. SECURITY CLASSIFICATION OF: 17...reliable tumor models for malignant peripheral nerve sheath tumors ( MPNST ). Several existing and novel oncolytic HSV vectors will then be tested on these...from G47A increases cytotoxicity in vitro to human endothelial cells and murine Nfl" MPNST cell lines. Inhibition of MPNST M2 tumor growth in vivo was

  2. Differential expression of angiogenic factors in peripheral nerve sheath tumors.

    PubMed

    Wasa, Junji; Nishida, Yoshihiro; Suzuki, Yoshitaka; Tsukushi, Satoshi; Shido, Yoji; Hosono, Kozo; Shimoyama, Yoshie; Nakamura, Shigeo; Ishiguro, Naoki

    2008-01-01

    It is difficult to differentiate some malignant peripheral nerve sheath tumors (MPNST) from benign peripheral nerve sheath tumors (BPNST) histologically, and to predict the clinical outcome of patients with MPNST. In this study, the expression of VEGF and MVD were evaluated immunohistochemically in 22 cases of MPNST, 14 of neurofibroma and 19 of schwannoma and correlation of the staining grade of VEGF or MVD and the various clinical factors were analyzed, and statistically evaluated. Levels of VEGF mRNA expression were also determined with real-time RT-PCR. Statistically higher positive staining for VEGF was observed in MPNST compared to neurofibroma (P=0.004) and schwannoma (P<0.001). Even low grade MPNST showed higher VEGF positive staining than neurofibroma. Moreover, high VEGF expression statistically correlated with the poor prognosis of the patients with MPNST (P=0.015). Although MVD in MPNST was significantly higher than that in neurofibroma (P=0.038) and schwannoma (P<0.001), MVD could not predict the prognosis of the patients with MPNST. Although VEGF mRNA expression tended to be higher in MPNST compared to neurofibroma, the difference was not significant. Levels of VEGF protein expression serve as a novel diagnostic and prognostic tools for peripheral nerve sheath tumors.

  3. Meningioma

    MedlinePlus

    ... based dye is used to augment the picture. Magnetic resonance imaging (MRI). With this imaging study, a magnetic field ... Accessed Dec. 2, 2013. Pinto PS, et al. Magnetic resonance imaging features of meningioma in children and young adults: ...

  4. Characterization of the Biomechanical Behavior of the Optic Nerve Sheath

    NASA Technical Reports Server (NTRS)

    Raykin, Julia; Wang, Roy; Forte, Taylor E.; Feola, Andrew; Samuels, Brian; Myers, Jerry; Nelson, Emily; Gleason, Rudy; Ethier, C. Ross

    2016-01-01

    A major priority in current space medicine research is to characterize the mechanisms in Visual Impairment and Intracranial Pressure (VIIP) syndrome, a group of ophthalmic changes that occur in some astronauts following long-duration spaceflight [1]. It is hypothesized that microgravity-induced cephalad fluid shifts lead to increases in intracranial pressure (ICP), which drives maladaptive remodeling of the optic nerve sheath (ONS). In this study, we investigated the effects of mechanical loading on the porcine ONS to better understand the mechanical response of the ONS to increased ICP.

  5. [Intrathoracic giant peripheral nerve sheath tumor during Von Recklinghausen disease].

    PubMed

    Ngabou, U D; Mounguengui, D; Owono Mbouengou, J P; El Wali, A; Nguema Edzang, B; Boguikouma, J B; Tchoua, R; Aziz, N E

    2014-06-01

    We report the case of a patient aged 23, admitted for bilateral intrathoracic tumor, including a giant right. Surgery was performed by right sternothoracotomy. After 7 days, she presented an irreversible cardiac arrest. The malignant peripheral nerve sheath tumors are rare and aggressive. Their incidence is 0.001% in the general population and 0.16% in patients with neurofibromatosis type 1. These tumors are characterized by their risk of recurrence and poor prognosis. The treatment is the surgical resection. We analyze incidence, diagnosis and prognosis of these tumors.

  6. Aggressive orbital optic nerve meningioma with benign microscopic features: a case report.

    PubMed

    Amoli, F Asadi; Mehrabani, P Mansouri; Tari, A Sadeghi

    2007-12-01

    Primary optic nerve meningiomas occur at lower ages than meningiomas arising from the coverings of the brain and spinal cord. Here we report the case of a 20-year-old female with an aggressive orbital meningioma referred to the Ophthalmology Department of the Farabi Hospital in Tehran. The patient had a history of orbital meningioma from 10 years ago and several surgical resections due to tumor recurrence during these 10 years. On admission, the patient had a large orbital mass and severe proptosis. MRI images revealed a large left orbital mass with optic nerve involvement and extension to the left maxillary sinus, pterygoid fossa and the dura in the floor of the anterior fossa. Fine-needle aspiration cytology of the mass confirmed tumor recurrence. The patient first received radiotherapy due to the inoperable mass, and the tumor was resected 1.5 month later. Microscopic study showed meningotheliomatous meningioma with extensive involvement of the optic nerve and invasion of the optic disc, sclera and choroid. The interesting aspect of this case was the aggressive behavior of the tumor with intraocular invasion, despite its benign histopathological features, which led to wide exenteration of the eye together with resection of the upper and lower lids.

  7. Microgravity-Driven Optic Nerve/Sheath Biomechanics Simulations

    NASA Technical Reports Server (NTRS)

    Ethier, C. R.; Feola, A.; Myers, J. G.; Nelson, E.; Raykin, J.; Samuels, B.

    2016-01-01

    Visual Impairment and Intracranial Pressure (VIIP) syndrome is a concern for long-duration space flight. Current thinking suggests that the ocular changes observed in VIIP syndrome are related to cephalad fluid shifts resulting in altered fluid pressures [1]. In particular, we hypothesize that increased intracranial pressure (ICP) drives connective tissue remodeling of the posterior eye and optic nerve sheath (ONS). We describe here finite element (FE) modeling designed to understand how altered pressures, particularly altered ICP, affect the tissues of the posterior eye and optic nerve sheath (ONS) in VIIP. METHODS: Additional description of the modeling methodology is provided in the companion IWS abstract by Feola et al. In brief, a geometric model of the posterior eye and optic nerve, including the ONS, was created and the effects of fluid pressures on tissue deformations were simulated. We considered three ICP scenarios: an elevated ICP assumed to occur in chronic microgravity, and ICP in the upright and supine positions on earth. Within each scenario we used Latin hypercube sampling (LHS) to consider a range of ICPs, ONH tissue mechanical properties, intraocular pressures (IOPs) and mean arterial pressures (MAPs). The outcome measures were biomechanical strains in the lamina cribrosa, optic nerve and retina; here we focus on peak values of these strains, since elevated strain alters cell phenotype and induce tissue remodeling. In 3D, the strain field can be decomposed into three orthogonal components, denoted as first, second and third principal strains. RESULTS AND CONCLUSIONS: For baseline material properties, increasing ICP from 0 to 20 mmHg significantly changed strains within the posterior eye and ONS (Fig. 1), indicating that elevated ICP affects ocular tissue biomechanics. Notably, strains in the lamina cribrosa and retina became less extreme as ICP increased; however, within the optic nerve, the occurrence of such extreme strains greatly increased as

  8. Study of malignant peripheral nerve sheath tumor in cerebellopontine angle.

    PubMed

    Hong, WenMing; Cheng, HongWei; Wang, XiaoJie; Hu, XiaoPeng; Feng, ChunGuo

    2014-01-01

    Malignant peripheral nerve sheath tumors (MPNSTs) are very rare soft tissue sarcomas, usually arising from somatic soft tissues or peripheral nerves. Primary MPNST of the cerebellopontine angle is extremely rare, with only a single case reported so far. Here, we report an unusual case of MPNST in cerebellopontine angle in a 25-year-old man presented with dizziness, left facial numbness, and tinnitus. After hospitalization, the tumor was treated with complete surgical excision followed by adjuvant chemotherapy and radiotherapy. Histologically, the tumor showed malignant spindle cells, which were with focal S-100 positivity on immunohistochemistry, and a diagnosis of the MPNST was made. This case is being reported for its rarity and presence in cerebellopontine and illustrated the difficulties in the diagnosis and treatment of MPNST, which to the best of our knowledge, has not been described before in the soft tissue sarcomas.

  9. Skull Base Meningiomas and Cranial Nerves Contrast Using Sodium Fluorescein: A New Application of an Old Tool

    PubMed Central

    da Silva, Carlos Eduardo; da Silva, Vinicius Duval; da Silva, Jefferson Luis Braga

    2014-01-01

    Objective The identification of cranial nerves is one of the most challenging goals in the dissection of skull base meningiomas. The authors present an application of sodium fluorescein (SF) in skull base meningiomas with the purpose of improving the identification of cranial nerves. Design A prospective study within-subjects design. Setting Hospital Ernesto Dornelles, Porto Alegre, Brazil. Participants Patients with skull base meningiomas. Main Outcomes Measures Cranial nerve identification. Results The group of nine meningiomas was composed of one cavernous sinus, three petroclival, one tuberculum sellae, two sphenoid wing, one olfactory groove, and one temporal floor meningioma. The SF enhancement in all tumors was strong, and the contrast with cranial nerves clearly evident. There were one definite olfactory nerve deficit, one transient abducens deficit, and one definite hemiparesis. All lesions were resected (Simpson grades 1 and 2). The analysis of the difference of the delta SF wavelength between the meningiomas and cranial nerve contrast was performed by the Wilcoxon signed rank test and showed p = 0.011. Conclusions The contrast between the enhanced meningiomas and cranial nerves was evident and assisted in the visualization and microsurgical dissection of these structures. The anatomical preservation of these structures was improved using the contrast. PMID:27054056

  10. Skull Base Meningiomas and Cranial Nerves Contrast Using Sodium Fluorescein: A New Application of an Old Tool.

    PubMed

    da Silva, Carlos Eduardo; da Silva, Vinicius Duval; da Silva, Jefferson Luis Braga

    2014-08-01

    Objective The identification of cranial nerves is one of the most challenging goals in the dissection of skull base meningiomas. The authors present an application of sodium fluorescein (SF) in skull base meningiomas with the purpose of improving the identification of cranial nerves. Design A prospective study within-subjects design. Setting Hospital Ernesto Dornelles, Porto Alegre, Brazil. Participants Patients with skull base meningiomas. Main Outcomes Measures Cranial nerve identification. Results The group of nine meningiomas was composed of one cavernous sinus, three petroclival, one tuberculum sellae, two sphenoid wing, one olfactory groove, and one temporal floor meningioma. The SF enhancement in all tumors was strong, and the contrast with cranial nerves clearly evident. There were one definite olfactory nerve deficit, one transient abducens deficit, and one definite hemiparesis. All lesions were resected (Simpson grades 1 and 2). The analysis of the difference of the delta SF wavelength between the meningiomas and cranial nerve contrast was performed by the Wilcoxon signed rank test and showed p = 0.011. Conclusions The contrast between the enhanced meningiomas and cranial nerves was evident and assisted in the visualization and microsurgical dissection of these structures. The anatomical preservation of these structures was improved using the contrast.

  11. Finite Element Biomechanics of Optic Nerve Sheath Traction in Adduction.

    PubMed

    Shin, Andrew; Yoo, Lawrence; Park, Joseph; Demer, Joseph L

    2017-10-01

    Historical emphasis on increased intraocular pressure (IOP) in the pathogenesis of glaucoma has been challenged by the recognition that many patients lack abnormally elevated IOP. We employed finite element analysis (FEA) to infer contribution to optic neuropathy from tractional deformation of the optic nerve head (ONH) and lamina cribrosa (LC) by extraocular muscle (EOM) counterforce exerted when optic nerve (ON) redundancy becomes exhausted in adduction. We characterized assumed isotropic Young's modulus of fresh adult bovine ON, ON sheath, and peripapillary and peripheral sclera by tensile elongation in arbitrary orientations of five specimens of each tissue to failure under physiological temperature and humidity. Physical dimensions of the FEA were scaled to human histological and magnetic resonance imaging (MRI) data and used to predict stress and strain during adduction 6 deg beyond ON straightening at multiple levels of IOP. Young's modulus of ON sheath of 44.6 ± 5.6 MPa (standard error of mean) greatly exceeded that of ON at 5.2 ± 0.4 MPa, peripapillary sclera at 5.5 ± 0.8 MPa, and peripheral sclera at 14.0 ± 2.3 MPa. FEA indicated that adduction induced maximum stress and strain in the temporal ONH. In the temporal LC, the maximum stress was 180 kPa, and the maximum strain was ninefold larger than produced by IOP elevation to 45 mm Hg. The simulation suggests that ON sheath traction by adduction concentrates far greater mechanical stress and strain in the ONH region than does elevated IOP, supporting the novel concept that glaucomatous optic neuropathy may result at least partly from external traction on the ON, rather than exclusively on pressure on the ON exerted from within the eye.

  12. Composite pheochromocytoma with a malignant peripheral nerve sheath tumor: Case report and review of the literature.

    PubMed

    Namekawa, Takeshi; Utsumi, Takanobu; Imamoto, Takashi; Kawamura, Koji; Oide, Takashi; Tanaka, Tomoaki; Nihei, Naoki; Suzuki, Hiroyoshi; Nakatani, Yukio; Ichikawa, Tomohiko

    2016-07-01

    Adrenal tumors with more than one cellular component are uncommon. Furthermore, an adrenal tumor composed of a pheochromocytoma and a malignant peripheral nerve sheath tumor is extremely rare. A composite pheochromocytoma with malignant peripheral nerve sheath tumor in a 42-year-old man is reported here. After adequate preoperative control, left adrenalectomy was performed simultaneously with resection of the ipsilateral kidney for spontaneous rupture of the left adrenal tumor. Pathological findings demonstrated pheochromocytoma and malignant peripheral nerve sheath tumor in a ruptured adrenal tumor. To date, there have been only four reported cases of composite pheochromocytoma with malignant peripheral nerve sheath tumor, so the present case is only the fifth case in the world. Despite the very poor prognosis of patients with pheochromocytoma and malignant peripheral nerve sheath tumors reported in the literature, the patient remains well without evidence of recurrence or new metastatic lesions at 36 months postoperatively. Copyright © 2012. Published by Elsevier Taiwan.

  13. Feline cutaneous nerve sheath tumours: histological features and immunohistochemical evaluations.

    PubMed

    Mandara, M T; Fabriani, E; Pavone, S; Pumarola, M

    2013-10-01

    Feline cutaneous nerve sheath tumours (CNSTs) are uncommonly reported in the skin, since they are underestimated relative to the more common spindle cell tumours of soft tissue. In this study, 26 nerve sheath tumours selected from 337 skin neoplasms of cats were examined. Histologically, they were classified into malignant (MPNSTs) and benign tumours (BPNSTs) based on degree of cellular atypia and polymorphism as well as mitotic rate and diffuse necrosis. CPNSTs were tipically characterised by Antoni A pattern, in some cases associated with Antoni B pattern. In the malignant peripheral nerve sheath tumours (MPNSTs) the polymorphism was marked, while it was mild to moderate in the benign forms (BPNSTs). In the MPNSTs the mitotic activity was generally higher than in the BPNSTs. In five cases, including three MPNSTs and two BPNSTs, there were multinucleated giant cells. Necrotic foci occurred in a BPNST and in two MPNSTs, while osseous/chondroid metaplasia was found in two cases. Immunohistochemically, all the tumours showed a marked diffuse vimentin expression. S-100 protein was expressed in 17 cases, including 81.8% of BPNSTs and 57.14% of MPNSTs. Twenty-five tumours expressed NSE and twenty-four cases showed immunoreaction for laminin. Thirteen tumours were positive for GFAP, while five tumours were positive for SMA. PGP 9.5 expression was detected in all cases, except for two MPNSTs. NGFR was expressed in eleven cases, including four MPNSTs and seven BPNSTs. Ki67 was expressed in twenty tumours without any relationship with morphologic malignancy of the neoplasm. In this case series we confirmed neoplastic spindloid cells with wavy cytoplasm arranged in compact areas, with occasional nuclear palisading or whirls, and interchanged with loosely arranged areas, as the morphological features supporting a diagnosis of CPNST. A constant concurrent expression of vimentin, NSE, and laminin might confirm the diagnosis of PNST in the absence of clear S-100 protein

  14. Malignant peripheral nerve sheath tumour in a sow.

    PubMed

    Resende, Talita P; Pereira, Carlos E R; Vannucci, Fabio A; Araujo, Fernando S; dos Santos, José Lúcio; Cassali, Geovanni D; Damasceno, Karine A; Guedes, Roberto M C

    2015-09-25

    Nodular lung lesions in swine are frequently due to abscesses or granulomatous pneumonia. Although tumours are rarely reported in modern pig farming, they should be considered as a differential diagnosis when nodular lung lesions are found. A first-parity sow exhibiting respiratory signs was euthanized. Several whitish firm nodules, not encapsulated, ranging in diameter from 0.5 to 5 cm were present in all lung lobes. Microscopically, the nodules were composed of dense neoplastic cells, mainly in Antoni types A and B patterns, infiltrative and with development of emboli. All neoplastic cells stained positively by immunohistochemistry for vimentin and S-100 protein, with variable immunostaining for glial fibrillary acidic protein and stained negative for cytokeratin. Based on the gross, histological and immunohistochemical features, the tumor was diagnosed as malignant peripheral nerve sheath tumour.

  15. Sarcoma of possible nerve sheath origin in a captive muskrat.

    PubMed

    Borucinska, J D; Trettel, J; Knibbs, D

    2000-07-01

    A captive adult female muskrat (Ondatra zibethicus) was found dead without previous signs of disease. At necropsy, abdominal organs were infiltrated with a poorly demarcated, soft, tan tissue. Microscopically this tissue was composed of neoplastic cells assuming two distinct growth characteristics consistent with Antoni A and B patterns. Ultrastructurally, the neoplastic cells were pleomorphic, lacked junctional devices, had abundant mitochondria and ergastoplasm, and frequently were closely associated with extracellular collagen. Immunocytochemical examination of tumor cells demonstrated sporadic expression of neuron specific enolase. Microscopic tumor metastases to the myocardium, ascending aorta, lungs and visceral pleura were present. This is the first report of a sarcoma compatible with a malignant peripheral nerve sheath tumor in a muskrat.

  16. Cerebral malignant nerve sheath tumor, triton tumor variant: case report.

    PubMed

    Bornstein-Quevedo, Leticia; Peralta-Olvera, Fabiola; Marhx-Bracho, Alfonso; Rodríguez-Jurado, Rodolfo; De Leon-Bojorge, Beatriz

    2003-01-01

    A case of a cerebral malignant triton tumor in a 3-year-old boy with a 2-month history of frontal headache and no clinical evidence of neurofibromatosis is reported. The computed tomography (CT) scan showed a large, irregular tumor in the right parietooccipital lobe. A partial surgical resection was performed. Histologically, the tumor was highly cellular and consisted of spindle cells with hyperchromatic and pleomorphic nuclei. Focally, neoplastic cells with rhabdomyoblastic features were found. The immunohistochemical study showed that tumor cells were positive for S-100 protein and CD57, and the rhabdomyoblasts expressed desmin, Myo-D1, and myoglobin. During the postoperative period, a massive intraparenchymal hemorrhage was identified and surgical drainage was performed. The patient worsened and died 10 days after the first surgery. Postmortem study was not authorized. Six cases of cerebral malignant nerve sheath tumor have been described; however, primary intraparenchymal malignant triton tumor has not been previously described.

  17. Nerve sheath myxoma of the dorsal paravertebral space

    PubMed Central

    Malkoc, Melih; Ormeci, Tugrul; Keskinbora, Mert; Yılmaz, Adem; Korkmaz, Ozgur; Tanik, Canan Besleyici

    2014-01-01

    INTRODUCTION Nerve sheath myxomas (NSM) are rare benign soft tissue tumors. The dorsal paravertebral placed NMS diagnosis can be difficult. PRESENTATION OF CASE This article presents clinical, radiological findings and treatment of the NSM of the dorsal paravertebral space in a 32-year-old man presented with a right shoulder and back pain for 4 years. DISCUSSION NSM is a rare and benign tumor and that most often occurs in the skin of the head, neck or upper limbs of younger patients. Rare locations such as intracranial, spinal canal, trunk, lower limb and oral cavity were also reported. The appropriate treatment of NSM is surgical excision. Diagnosis is difficult in an uncommon presentation. CONCLUSION Although the most presented case of NMS are dermal tumors, it may also be found extremely rare locations. We conclude that, the definitive treatment of NSM is surgical excision with safe margins even when it is possible. PMID:25462051

  18. Targeting the ECM to Enhance Drug Delivery in Nf1-Associated Nerve Sheath Tumors

    DTIC Science & Technology

    2016-10-01

    AWARD NUMBER: W81XWH-15-1-0114 TITLE: Targeting the ECM to Enhance Drug Delivery in Nf1-Associated Nerve Sheath Tumors PRINCIPAL INVESTIGATOR...14 Sep 2016 4. TITLE AND SUBTITLE 5a. CONTRACT NUMBER NF140089 Targeting the ECM to Enhance Drug Delivery in Nf1-Associated Nerve Sheath Tumors 5b...to determine what factors limit delivery of drugs to tumors of the peripheral nerves, namely neurofibromas (NFs) and their derivative, the malignant

  19. Optic Nerve Vascular Compression in a Patient with a Tuberculum Sellae Meningioma

    PubMed Central

    Mizrahi, Cezar José; Moscovici, Samuel; Dotan, Shlomo; Spektor, Sergey

    2015-01-01

    Background. Optic nerve vascular compression in patients with suprasellar tumor is a known entity but is rarely described in the literature. Case Description. We present a unique, well-documented case of optic nerve strangulation by the A1 segment of the anterior cerebral artery in a patient with a tuberculum sellae meningioma. The patient presented with pronounced progressive visual deterioration. Following surgery, there was immediate resolution of her visual deficit. Conclusion. Vascular strangulation of the optic nerve should be considered when facing progressive and/or severe visual field deterioration in patients with tumors proximal to the optic apparatus. PMID:25705535

  20. Optic nerve vascular compression in a patient with a tuberculum sellae meningioma.

    PubMed

    Mizrahi, Cezar José; Moscovici, Samuel; Dotan, Shlomo; Spektor, Sergey

    2015-01-01

    Background. Optic nerve vascular compression in patients with suprasellar tumor is a known entity but is rarely described in the literature. Case Description. We present a unique, well-documented case of optic nerve strangulation by the A1 segment of the anterior cerebral artery in a patient with a tuberculum sellae meningioma. The patient presented with pronounced progressive visual deterioration. Following surgery, there was immediate resolution of her visual deficit. Conclusion. Vascular strangulation of the optic nerve should be considered when facing progressive and/or severe visual field deterioration in patients with tumors proximal to the optic apparatus.

  1. Ectopic orbital meningioma: Fact or fiction?

    PubMed

    Tan, Lee Teak; Stewart, Christopher M; Sheerin, Fintan; MacDonald, Brendan; Silva, Priy; Norris, Jonathan H

    2017-06-01

    Primary intraorbital ectopic meningiomas are rare and their existence remains controversial. We present a 30-year-old female with painless, non-axial proptosis and a palpable superomedial mass. The MRI demonstrated that the mass had no optic nerve sheath or sphenoid wing involvement and was initially reported to have no intracranial extension. The patient was initially thought to have an ectopic orbital meningioma. Subsequent multidisciplinary team (MDT) consultation and further specialist review of the MRI revealed a subtle dural tail connecting to an enhancing mass in the olfactory groove. Biopsy revealed a WHO Grade 1 transitional meningioma with an infiltrative pattern. We argue that some previously reported cases of ectopic meningioma may lack the requisite imaging to discover the primary disease. Our report highlights the importance of MRI in this group of patients and the role of a skull-base MDT with specialist neuroradiology input to determine the true origin and extent of these extradural orbital meningiomas.

  2. Concurrent spinal nerve root schwannoma and meningioma mimicking single-component schwannoma.

    PubMed

    Nakamizo, Akira; Suzuki, Satoshi O; Shimogawa, Takafumi; Amano, Toshiyuki; Mizoguchi, Masahiro; Yoshimoto, Koji; Sasaki, Tomio

    2012-04-01

    We present a first case of concurrent tumors consisting of schwannoma and meningioma arising at the same spinal level in a patient without neurofibromatosis. A 49-year-old man without clinical evidence of neurofibromatosis presented with a 5-month history of right neck pain. MRI demonstrated an extradural tumor involving the right-sided C2 nerve root with a small intradural component. T1- and T2-weighted and contrast-enhanced MRI could not differentiate the intradural tumor as different from the extradural tumor. Total removal of the tumors was performed. No contiguity of the extradural tumor with the intradural tumor was seen. The intradural tumor attached strongly to the dura mater around the C2 nerve root exits. Intraoperative pathological diagnosis confirmed the extradural tumor as schwannoma and the intradural tumor as meningioma. We therefore thoroughly coagulated the dura mater adjacent to the intradural tumor and resected the dura mater around the nerve root exits together with the tumor. Pathological examination revealed that the resection edge of the extradural component consisted of a spinal nerve with thickened epineurium and was free of neoplastic cells. No schwannoma component was evident in the intradural tumor. No obvious transition thus existed between the extra- and intradural tumors. Distinguishing these tumors prior to surgery is critical for determining an optimal surgical plan, as schwannoma and meningioma require different surgical procedures. We therefore recommend a careful review of preoperative imaging with the possibility of concurrent tumors in mind. © 2011 Japanese Society of Neuropathology.

  3. Reconstruction of nerve root sheaths for sacral extradural spinal meningeal cysts with spinal nerve root fibers.

    PubMed

    Sun, Jianjun; Wang, Zhenyu; Li, Zhendong; Wu, Haibo; Yen, Ruyu; Zheng, Mei; Chang, Qing; Liu, Isabelle Yisha

    2013-11-01

    This study analyzed the clinical characteristics and outcomes of sacral extradural spinal meningeal cysts with spinal nerve root fibers treated by reconstruction of the nerve root sheaths. The relationships between the cysts and spinal nerve root fibers were examined microscopically, the cysts were partially excised, and the defects were oversewn to reconstruct the nerve root sheaths. The Improved Japanese Orthopedic Association (IJOA) scoring system was used to evaluate preoperative and postoperative neurological function. Thirty-eight patients were included in this study, with a mean age of 41.4 ± 15.57 years. The mean IJOA score was 18.8 ± 1.32 preoperatively and 19.6 ± 0.65 postoperatively, which was a significant difference (t=-3.77, P=0.001). These results indicate a significant improvement in neurological function after surgery. The most significant improvement in neurological function was sensation (z=-2.86, P=0.004), followed by bowel/bladder function (z=-2.31, P=0.02).

  4. Biomechanics of the Optic Nerve Sheath in VIIP Syndrome

    NASA Technical Reports Server (NTRS)

    Ethier, C. Ross; Raykin, Julia; Gleason, Rudy; Mulugeta, Lealem; Myers, Jerry; Nelson, Emily; Samuels, Brian C.

    2014-01-01

    Long-duration space flight carries the risk of developing Visual Impairment and Intracranial Pressure (VIIP) syndrome, a spectrum of ophthalmic changes including posterior globe flattening, choroidal folds, distension of the optic nerve sheath (ONS), optic nerve kinking and potentially permanent degradation of visual function. The slow onset of VIIP, its chronic nature, and certain clinical features strongly suggest that biomechanical factors acting on the ONS play a role in VIIP. Here we measure several relevant ONS properties needed to model VIIP biomechanics. The ONS (meninges) of fresh porcine eyes (n7) was reflected, the nerve proper was truncated near the sclera, and the meninges were repositioned to create a hollow cylinder of meningeal connective tissue attached to the posterior sclera. The distal end was cannulated, sealed, and pressure clamped (mimicking cerebrospinal fluid [CSF] pressure), while the eye was also cannulated for independent control of intraocular pressure (IOP). The meninges were inflated (CSF pressure cycling 7-50 mmHg) while ONS outer diameter was imaged. In another set of experiments (n4), fluid permeation rate across the meninges was recorded by observing the drainage of an elevated fluid reservoir (30 mmHg) connected to the meninges. The ONS showed behavior typical of soft tissues: viscoelasticity, with hysteresis in early preconditioning cycles and repeatable behavior after 4 cycles, and nonlinear stiffening, particularly at CSF pressures 15 mmHg (Figure). Tangent moduli measured from the loading curve were 372 101, 1199 358, and 2050 379 kPa (mean SEM) at CSF pressures of 7, 15 and 30 mmHg, respectively. Flow rate measurements through the intact meninges at 30mmHg gave a permeability of 1.34 0.46 lmincm2mmHg (mean SEM). The ONS is a tough, strain-stiffening connective tissue that is surprisingly permeable. The latter observation suggests that there could be significant CSF drainage through the ONS into the orbit, likely important

  5. Optic Nerve Sheath Mechanics and Permeability in VIIP Syndrome

    NASA Technical Reports Server (NTRS)

    Raykin, Julia; Best, Lauren; Gleason, Rudy; Mulugeta, Lealem; Myers, Jerry; Nelson, Emily; Samuels, Brian C.; Ethier, C. R.

    2014-01-01

    Long-duration space flight carries the risk of developing Visual Impairment and Intracranial Pressure (VIIP) syndrome, a spectrum of ophthalmic changes including posterior globe flattening, choroidal folds, distension of the optic nerve sheath (ONS), optic nerve kinking and potentially permanent degradation of visual function. The slow onset of VIIP, its chronic nature, and certain clinical features strongly suggest that biomechanical factors acting on the ONS play a role in VIIP. Here we measure several relevant ONS properties needed to model VIIP biomechanics. The ONS (meninges) of fresh porcine eyes (n7) was reflected, the nerve proper was truncated near the sclera, and the meninges were repositioned to create a hollow cylinder of meningeal connective tissue attached to the posterior sclera. The distal end was cannulated, sealed, and pressure clamped (mimicking cerebrospinal fluid [CSF] pressure), while the eye was also cannulated for independent control of intraocular pressure (IOP). The meninges were inflated (CSF pressure cycling 7-50 mmHg) while ONS outer diameter was imaged. In another set of experiments (n4), fluid permeation rate across the meninges was recorded by observing the drainage of an elevated fluid reservoir (30 mmHg) connected to the meninges. The ONS showed behavior typical of soft tissues: viscoelasticity, with hysteresis in early preconditioning cycles and repeatable behavior after 4 cycles, and nonlinear stiffening, particularly at CSF pressures 15 mmHg (Figure). Tangent moduli measured from the loading curve were 372 101, 1199 358, and 2050 379 kPa (mean SEM) at CSF pressures of 7, 15 and 30 mmHg, respectively. Flow rate measurements through the intact meninges at 30mmHg gave a permeability of 1.34 0.46 lmincm2mmHg (mean SEM). The ONS is a tough, strain-stiffening connective tissue that is surprisingly permeable. The latter observation suggests that there could be significant CSF drainage through the ONS into the orbit, likely important

  6. Prevention and Treatment of Neurofibromatosis Type 1-Associated Malignant Peripheral Nerve Sheath Tumors

    DTIC Science & Technology

    2016-04-01

    notwithstanding any other provision of law, no person shall be subject to any penalty for failing to comply with a collection of information if it does...ABSTRACT The most common cause of death in Neurofibromatosis Type 1 (NF1) patients is malignant peripheral nerve sheath tumor (MPNST). MPNSTs are...expectancy by ten to twenty years. Malignant peripheral nerve sheath tumors (MPNSTs) are the leading cause of death in NF1 patients and typically arise

  7. Receptor Tyrosine Kinases as Targets for Treatment of Peripheral Nerve Sheath Tumors in NF 1 Patients

    DTIC Science & Technology

    2010-03-01

    by interphase cytogenetics (FISH) in malignant peripheral nerve sheath tumor (MPNST) and morphologically similar spindle cell neoplasms . J...Chronic myeloproliferative disorders with rearrangement of the platelet-derived growth factor alpha receptor: a new clinical target for STI571/Glivec...malignant peripheral nerve sheath tumor (MPNST) and morphologically similar spindle cell neoplasms . J Neuropathol Exp Neurol. 2002;61:702–709. 8

  8. Optic Nerve Sheath Diameter Increase on Ascent to High Altitude: Correlation With Acute Mountain Sickness.

    PubMed

    Kanaan, Nicholas C; Lipman, Grant S; Constance, Benjamin B; Holck, Peter S; Preuss, James F; Williams, Sarah R

    2015-09-01

    Elevated optic nerve sheath diameter on sonography is known to correlate with increased intracranial pressure and is observed in acute mountain sickness. This study aimed to determine whether optic nerve sheath diameter changes on ascent to high altitude are associated with acute mountain sickness incidence. Eighty-six healthy adults enrolled at 1240 m (4100 ft), drove to 3545 m (11,700 ft) and then hiked to and slept at 3810 m (12,500 ft). Lake Louise Questionnaire scores and optic nerve sheath diameter measurements were taken before, the evening of, and the morning after ascent. The incidence of acute mountain sickness was 55.8%, with a mean Lake Louise Questionnaire score ± SD of 3.81 ± 2.5. The mean maximum optic nerve sheath diameter increased on ascent from 5.58 ± 0.79 to 6.13 ± 0.73 mm, a difference of 0.91 ± 0.55 mm (P = .09). Optic nerve sheath diameter increased at high altitude regardless of acute mountain sickness diagnosis; however, compared to baseline values, we observed a significant increase in diameter only in those with a diagnosis of acute mountain sickness (0.57 ± 0.77 versus 0.21 ± 0.76 mm; P = .04). This change from baseline, or Δ optic nerve sheath diameter, was associated with twice the odds of developing acute mountain sickness (95% confidence interval, 1.08-3.93). The mean optic nerve sheath diameter increased on ascent to high altitude compared to baseline values, but not to a statistically significant degree. The magnitude of the observed Δ optic nerve sheath diameter was positively associated with acute mountain sickness diagnosis. No such significant association was found between acute mountain sickness and diameter elevation above standard cutoff values, limiting the utility of sonography as a diagnostic tool. © 2015 by the American Institute of Ultrasound in Medicine.

  9. Astounding recovery after resection of an intradural nerve sheath tumor in an adult male from Vietnam

    PubMed Central

    King, Paul; Khan, Saleen; Inamullah, Ovais

    2015-01-01

    Background: Spinal cord tumors can be classified as intramedullary, intradural extramedullary, or extradural. The differential diagnosis of spinal cord tumors includes meningiomas, astrocytomas, ependymomas, metastasis, nerve sheath tumors such as schwannomas or neurofibromas, and multiple sclerosis plaques. Radiology can provide clues to the type of tumor, but a pathology evaluation of a specimen is necessary to provide an accurate diagnosis. These tumors can cause a variety of neurological symptoms from spinal cord compression including pain, weakness, and paresthesia. They are treated by surgical resection, with a variety of outcomes possible depending on the severity of the preoperative symptoms, location and extent of the tumor, and efficacy of the surgery performed. Case Description: A 59-year-old male from Vietnam came to the Atlanta Medical Center for evaluation of severe ride sided hemiparesis and paresthesias. He first noticed alterations in his handwriting and quickly deteriorated to the point of being unable to walk or move his right arm. A cervical spinal mass was identified and analyzed on magnetic resonance imaging. Surgical resection was performed under a microscope in a joint operation between an orthopedic surgeon and neurosurgeon. A specimen of the tumor was sent to pathology for further evaluation. Conclusion: The mass was determined to be an intradural extramedullary schwannoma. The severity of the patient's symptoms and the location and size of the tumor made full recovery unlikely and postoperative quadriplegia a real possibility. The tumor was surgically resected, which led surprisingly, however, to a full and prompt resolution of the patient's symptoms. Less than 2 weeks after surgery, the patient was able to walk and had almost fully regained use of his hands. PMID:26425395

  10. [Malignant intracerebral nerve sheath tumours: Two case reports and complete review of the literature cases].

    PubMed

    Le Fèvre, C; Castelli, J; Perrin, C; Hénaux, P L; Noël, G

    2016-04-01

    Malignant peripheral nerve sheath tumours are extremely rare and can be associated with neurofibramatosis type 1. Their prognosis is poor and surgery remains the mainstay of therapy and should be the first line of treatment. Radiotherapy and chemotherapy are second line treatment and their effectiveness remains to demonstrate. The diagnosis is clinical, radiological, histological and immunohistochemical. Malignant peripheral nerve sheath tumours have a potential of local tumour recurrence very high and can metastasize. They often occur in extremity of the members but also rarely into brain. We report two cases of intracerebral nerve sheath tumour. The first was a 68-year-old woman who was admitted with progressive symptoms of headache and diplopia. A left frontotemporal malignant peripheral nerve sheath tumours was diagnosed and was treated by surgery and irradiation. Ten months later, she presented a local recurrence and spine bone's metastases were treated by vertebroplasty and irradiation. The patient died 15 months after the diagnosis. The second case was a 47-year-old woman who was referred because headache and vomiting symptoms. A right frontal malignant peripheral nerve sheath tumours was diagnosed and treated by surgery and irradiation. After that, the patient had three local recurrence operated and pulmonary and cranial bone's metastases. She was still alive after 20 months. We propose a literature review with 25 cases of intracerebral nerve sheath tumour identified, including the two current cases.

  11. Palliative Epineurotomy for Focal Radial Malignant Peripheral Nerve Sheath Tumor in a Dog.

    PubMed

    Gibson, Andrew David; Davies, Emma; Lara-Garcia, Ana; Lafuente, Pilar

    2016-01-01

    This case report describes the diagnosis of a peripheral nerve sheath tumor of the deep branch of the radial nerve distal to the elbow in a dog. The lesion was identified using computed tomography and ultrasonography and confirmed as sarcoma on histopathological analysis of incisional biopsies. Clinical signs dramatically improved following surgical biopsy before recurring three months later. Repeat epineurotomy of the deep branch of the radial nerve resulted in clinical improvement for a further month before signs once again returned. Epineurotomy as a palliative treatment for peripheral nerve sheath tumors has not been previously described, but may have a place in palliation of clinical signs in specific cases of peripheral nerve sheath tumors in which limb amputation is not an option.

  12. Therapeutic efficacy of G207 in a novel peripheral nerve sheath tumor model.

    PubMed

    Mashour, G A; Moulding, H D; Chahlavi, A; Khan, G A; Rabkin, S D; Martuza, R L; Driever, P H; Kurtz, A; Chalavi, A

    2001-05-01

    Nerve involvement poses a significant obstacle for the management of peripheral nervous system tumors, and nerve injury provides a frequent source of postoperative morbidity. The lack of suitable animal models for peripheral nerve tumors has impeded the development of alternative nerve-sparing therapies. To evaluate the effect of a multimutated replication-competent herpes simplex virus (G207) on the growth of peripheral nerve tumors and on nerve function, we developed a novel peripheral nerve sheath tumor model. Human neuroblastoma-derived cells injected into murine sciatic nerve consistently caused tumor development within the nerve sheath after 2 weeks followed by increasingly severe impairment of nerve function. Tumor treatment by a single intratumoral injection of G207 resulted in significant reduction of functional impairment, inhibition of tumor growth and prolonged survival. Direct injection of G207 viral particles into the healthy nerve sheath caused no obvious neurologic sequelae, whereas injections of wild-type virus resulted in uniform lethality. The results indicate that viral therapy might be considered as a safe alternative to surgical removal of tumors with peripheral nerve involvement. Copyright 2001 Academic Press.

  13. Alterations at chromosome 17 loci in peripheral nerve sheath tumors

    SciTech Connect

    Lothe, R.A.; Slettan, A.; Saeter, G.

    1995-01-01

    Little is known about the molecular genetic changes in malignant peripheral nerve sheath tumors (MPNST). Inactivation of the TP53 gene in l7p has been reported in a few tumors. The MPNST is one of the manifestations of neurofibromatosis 1 (NF1), suggesting that the NF1 gene in 17q might be important. We present a study of 15 neurofibromas and MPNST from nine individuals. Seven patients had NF1 and six of these developed MPNST. Genetic alterations at nine polymorphic loci on chromosome 17 were examined. Allelic imbalance was detected only in the malignant tumors from NF1 patients (4/6). Complete loss of heterozygosity of 17q loci was found in three of these tumors, all including loci within the NF1 gene. Two of the malignant tumors also showed deletions on 17p. No mutations were detected within exon 5-8 of the TP53 in any of the MPNST, and none of them were TP53 protein-positive using immunostaining with mono- and polyclonal antibodies against TP53. The numbers of chromosome 17 present in each tumor were evaluated by use of fluorescence in situ hybridization (FISH) on interphase nuclei with a centromere-specific probe. A deviation from the disomic status of chromosome 17 was observed in two of the MPNST from NF1 patients. These results support the hypothesis of inactivation of both NF1 gene alleles during development of MPNST in patients with NF1. In contrast to other reports, we did not find evidence for a homozygous mutated condition of the TP53 gene in the same tumors. Finally, FISH analysis was in accordance with the DNA analysis in the deduction of the numbers of chromosome 17 in these tumors. 29 refs., 3 figs., 2 tabs.

  14. Giant intrapelvic malignant peripheral nerve sheath tumor mimicking disc herniation: A case report

    PubMed Central

    Wang, Peng; Chen, Cong; Xin, Xiaotang; Liu, Bo; Li, Wei; Yin, Dezhen; Mu, Weidong

    2016-01-01

    Giant intrapelvic malignant peripheral nerve sheath tumors arising in the sciatic nerve in the pelvic cavity are a rare occurrence and their symptomatology is usually misdiagnosed as intervertebral disc herniation. We herein report the case of a 46-year old woman presenting with pain, hypesthesia and weakness of the left lower extremity due to a giant intrapelvic malignant peripheral nerve sheath tumor of the sciatic nerve. Prior to being referred to our institution, the patient was misdiagnosed as a case of sciatica due to a lumbar disc herniation and underwent an operation unsuccessfully, as there was little symptomatic improvement 2 months after the surgery. A magnetic resonance imaging examination of the pelvic cavity revealed a tumor of the sciatic nerve. The mass was resected via the posterior approach and histopathological examination confirmed the diagnosis of malignant peripheral nerve sheath tumor. Intrapelvic malignant peripheral nerve sheath tumors are an uncommon cause of sciatica and are commonly misdiagnosed as lumbar intervertebral disc herniation. Accurate diagnosis and complete surgical excision prior to metastasis are crucial for effective management of this condition. PMID:27900106

  15. A Rare Malignant Peripheral Nerve Sheath Tumor of the Maxilla Mimicking a Periapical Lesion

    PubMed Central

    Álvares, Pamella; Silva, Luciano; Pereira dos Santos Neto, Alexandrino; Rodrigues, Cleomar Donizeth; Caubi, Antônio; Silveira, Marcia; Sayão, Sandra; Sobral, Ana Paula

    2016-01-01

    Malignant peripheral nerve sheath tumor is a malignant neoplasm that is rarely found in the oral cavity. About 50% of this tumor occurs in patients with neurofibromatosis type I and comprises approximately 10% of all soft tissue sarcomas of head and neck region. Intraosseous malignant peripheral nerve sheath tumor of the maxilla is rare. This article is the first to address malignant peripheral nerve sheath tumor of the maxilla presenting as a periapical radiolucency on nonvital endodontically treated teeth in the English medical literature. Surgical approaches to malignant soft tissue tumor vary based on the extent of the disease, age of the patient, and pathological findings. A rare case of intraosseous malignant peripheral nerve sheath tumor is reported in a 16-year-old woman. The patient presented clinically with a pain involving the upper left incisors region and with defined unilocular periapical radiolucency lesion involved between the upper left incisors. An incisional biopsy was made. Histological and immunohistochemical examination were positive for S-100 protein and glial fibrillary acidic protein showed that the lesion was an intraosseous malignant peripheral nerve sheath tumor of the maxilla. Nine years after the surgery, no regional recurrence was observed. PMID:27994888

  16. Usefulness of intraoperative monitoring of oculomotor and abducens nerves during surgical treatment of the cavernous sinus meningiomas.

    PubMed

    Kaspera, Wojciech; Adamczyk, Piotr; Ślaska-Kaspera, Aleksandra; Ładziński, Piotr

    2015-03-01

    We analyzed the usefulness and prognostic value of intraoperative monitoring for identification of the oculomotor (III) and the abducens (VI) nerve in patients with cavernous sinus meningiomas. 43 patients diagnosed with cavernous sinus meningiomas were divided according to their topography. Function of the nerves was scored on original clinical and neurophysiological scales. The percentage of nerves identified correctly with the monitoring was significantly higher (91% vs. 53% for nerve III and 70% vs. 23% for nerve VI, p<0.001). The fractions of nerves III and VI identified correctly by means of the monitoring were significantly higher in the case of tumors with intra- and extracavernous location (89% vs. 32%, p<0.01) and intracavernous tumors (80% vs. 20%, p<0.05), respectively. The quality of post-resection recording correlated with functional status of both the nerves determined 9 months after the surgery (R=0.51, p<0.001 for nerve III and R=0.57, p<0.01 for nerve VI). Even a trace or pathological response to the post-resection stimulation was associated with improved functional status (90% vs. 50%, p<0.05 for nerve III and 93% vs. 38%, p<0.01 for nerve VI). Neurophysiological monitoring of ocular motor nerves enables their intraoperative identification during resections of the cavernous sinus meningiomas. Intraoperative monitoring of nerve III is particularly important in the case of tumors with extra- and intracavernous location, and the monitoring of nerve VI in the case of intracavernous tumors. The outcome of the post-resection monitoring has prognostic value with regard to the clinical status of the nerves on long-term follow-up. Copyright © 2014 Medical University of Bialystok. Published by Elsevier Urban & Partner Sp. z o.o. All rights reserved.

  17. Accuracy and complications of CT-guided core needle biopsy of peripheral nerve sheath tumours.

    PubMed

    Pianta, Marcus; Chock, Eric; Schlicht, Stephen; McCombe, David

    2015-09-01

    This single-centre study retrospectively reviews the complications in patients that have occurred following peripheral nerve sheath tumour biopsy, and assesses whether there is an association with biopsy technique or underlying lesion characteristics. 41 consecutive core needle biopsies of proven peripheral nerve sheath tumours over a 2-year period in a tertiary teaching hospital were reviewed. Patient demographics and symptoms, tumour characteristics and radiological appearances were recorded. Biopsy and surgical histology were correlated, and post-biopsy and surgical complications analyzed. 41 biopsies were performed in 38 patients. 68% schwannomas, 24% neurofibromas and 7% malignant peripheral nerve sheath tumours. Biopsy histology correlated with surgery in all cases. 71% of lesions were surgically excised. 60% of patients reported pain related to their lesion. Following the biopsy, 12% reported increased pain, which resolved in all cases. Pain exacerbation was noted in tumours smaller in size, more superficial and in closer proximity of the biopsy needle tip to the traversing nerve. Number of biopsy needle passes was not associated with an increased incidence of procedure-related pain. Core biopsy of a suspected peripheral nerve sheath tumour may be performed safely before excisional surgery to confirm lesion histology and assist prognosis. There is excellent correlation between core biopsy and excised surgical specimen histology. The most common complication of pain exacerbation is seen in a minority and is temporary, and more likely with smaller, more superficial lesions and a closer needle-tip to traversing nerve distance during biopsy.

  18. Vagal nerve stimulation without dissecting the carotid sheath during intraoperative neuromonitoring of the recurrent laryngeal nerve in thyroid surgery.

    PubMed

    Wu, Che-Wei; Dionigi, Gianlorenzo; Chen, Hui-Chun; Chen, Hsiu-Ya; Lee, Ka-Wo; Lu, I-Cheng; Chang, Pi-Ying; Hsiao, Pi-Jung; Ho, Kuen-Yao; Chiang, Feng-Yu

    2013-10-01

    Vagal nerve stimulation (VNS) has been recommended as a routine procedure during intraoperative neuromonitoring (IONM) of the recurrent laryngeal nerve (RLN). However, many surgeons have been discouraged from performing VNS because of the need for opening the carotid sheath. The purpose of this study was to investigate the feasibility and reliability of VNS without carotid sheath dissection. Two hundred twenty patients with 376 nerves at risk were enrolled in this study. VNS without nerve exposure during IONM was applied by simply pressing a ball-tip stimulator on the space between the carotid artery and jugular vein. VNS without nerve exposure was feasible in all cases and did not result in any morbidity. All VNS signals were successfully obtained within 30 minutes of the start of the operation and all showed a clear and reliable laryngeal electromyography (EMG) response that was similar to that from the conventional method in which nerve exposure for VNS is applied. VNS without dissecting the carotid sheath is feasible and reliable, rendering it a simple, safe, and surgeon-friendly procedure during IONM. Copyright © 2013 Wiley Periodicals, Inc.

  19. Ultrastructure of the extracellular matrix of bovine dura mater, optic nerve sheath and sclera.

    PubMed Central

    Raspanti, M; Marchini, M; Della Pasqua, V; Strocchi, R; Ruggeri, A

    1992-01-01

    The sclera, the outermost sheath of the optic nerve and the dura mater have been investigated histologically and ultrastructurally. Although these tissues appear very similar under the light microscope, being dense connective tissues mainly composed of collagen bundles and a limited amount of cells and elastic fibres, they exhibit subtle differences on electron microscopy. In the dura and sclera collagen appears in the form of large, nonuniform fibrils, similar to those commonly found in tendons, while in the optic nerve sheath the fibrils appear smaller and uniform, similar to those commonly observed in reticular tissues, vessel walls and skin. Freeze-fracture also reveals these fibrils to have different subfibrillar architectures, straight or helical, which correspond to 2 distinct forms of collagen fibril previously described (Raspanti et al. 1989). The other extracellular matrix components also vary with the particular collagen fibril structure. Despite their common embryological derivation, the dura mater, optic nerve sheath and sclera exhibit diversification of their extracellular matrix consistent with the mechanical loads to which these tissues are subjected. Our observations indicate that the outermost sheath of the optic nerve resembles the epineurium of peripheral nerves rather than the dura to which it is commonly likened. Images Fig. 1 Fig. 2 Fig. 3 Fig. 4 Fig. 5 Fig. 6 Fig. 7 Fig. 8 Fig. 9 PMID:1295858

  20. [Indications and technique for transconjunctival optic nerve sheath fenestration : Video article].

    PubMed

    Lagrèze, W A; Gross, N; Biermann, J; Joachimsen, L

    2017-09-13

    Placement of a ventricular shunt is the primary surgical procedure for lowering intracranial pressure in pseudotumor cerebri syndrome; however, if ophthalmological symptoms prevail over neurological symptoms or if there are no neurological symptoms at all, optic nerve sheath fenestration may be a valuable option for relief of pressure on the retrobulbar optic nerve when papilledema caused by pseudotumor cerebri syndrome threatens vision despite previous conservative measures. This review covers the indications, technique and results of optic nerve sheath fenestration compared to competing procedures based on a systematic literature search, analysis of own cases and a documentation of the surgical technique. After performing a medial transconjunctival orbitotomy the medial rectus muscle tendon is temporarily detached and the eye abducted by traction sutures. Using confocal illumination under a surgical microscope, the optic nerve can be visualized using orbital spatulas and the sheath can be punctured with a microscalpel. A video of this operation is available online. Transconjunctival optic nerve sheath fenestration is a relatively safe method to reduce the rate of visual loss in pseudotumor cerebri syndrome. In selected cases it can be a useful alternative to ventriculoperitoneal/atrial shunts or venous stents.

  1. Genetic profiling by single-nucleotide polymorphism-based array analysis defines three distinct subtypes of orbital meningioma.

    PubMed

    Ho, Cheng-Ying; Mosier, Stacy; Safneck, Janice; Salomao, Diva R; Miller, Neil R; Eberhart, Charles G; Gocke, Christopher D; Batista, Denise A S; Rodriguez, Fausto J

    2015-03-01

    Orbital meningiomas can be classified as primary optic nerve sheath (ON) meningiomas, primary intraorbital ectopic (Ob) meningiomas and spheno-orbital (Sph-Ob) meningiomas based on anatomic site. Single-nucleotide polymorphism (SNP)-based array analysis with the Illumina 300K platform was performed on formalin-fixed, paraffin-embedded tissue from 19 orbital meningiomas (5 ON, 4 Ob and 10 Sph-Ob meningiomas). Tumors were World Health Organization (WHO) grade I except for two grade II meningiomas, and one was NF2-associated. We found genomic alterations in 68% (13 of 19) of orbital meningiomas. Sph-Ob tumors frequently exhibited monosomy 22/22q loss (70%; 7/10) and deletion of chromosome 1p, 6q and 19p (50% each; 5/10). Among genetic alterations, loss of chromosome 1p and 6q were more frequent in clinically progressive tumors. Chromosome 22q loss also was detected in the majority of Ob meningiomas (75%; 3/4) but was infrequent in ON meningiomas (20%; 1/5). In general, Ob tumors had fewer chromosome alterations than Sph-Ob and ON tumors. Unlike Sph-Ob meningiomas, most of the Ob and ON meningiomas did not progress even after incomplete excision, although follow-up was limited in some cases. Our study suggests that ON, Ob and Sph-Ob meningiomas are three molecularly distinct entities. Our results also suggest that molecular subclassification may have prognostic implications.

  2. Pathology of Peripheral Nerve Sheath Tumors: Diagnostic Overview and Update on Selected Diagnostic Problems

    PubMed Central

    Rodriguez, Fausto J.; Folpe, Andrew L.; Giannini, Caterina; Perry, Arie

    2013-01-01

    Peripheral nerve sheath tumors are common neoplasms, with classic identifiable features, but on occasion, they are diagnostically challenging. Although well defined subtypes of peripheral nerve sheath tumors were described early in the history of surgical pathology, controversies regarding the classification and grading of these tumors persist. Advances in molecular biology have provided new insights into the nature of the various peripheral nerve sheath tumors, and have begun to suggest novel targeted therapeutic approaches. In this review we discuss current concepts and problematic areas in the pathology of peripheral nerve sheath tumors. Diagnostic criteria and differential diagnosis for the major categories of nerve sheath tumors are proposed, including neurofibroma, schwannoma, and perineurioma. Diagnostically challenging variants, including plexiform, cellular and melanotic schwannomas are highlighted. A subset of these affects the childhood population, and has historically been interpreted as malignant, although current evidence and outcome data suggests they represent benign entities. The growing current literature and the authors experience with difficult to classify borderline or “hybrid tumors” are discussed and illustrated. Some of these classification gray zones occur with frequency in the gastrointestinal tract, an anatomical compartment that must always be entertained when examining these neoplasms. Other growing recent areas of interest include the heterogeneous group of pseudoneoplastic lesions involving peripheral nerve composed of mature adipose tissue and/or skeletal muscle, such as the enigmatic neuromuscular choristoma. Malignant peripheral nerve sheath tumors (MPNST) represent a diagnostically controversial group; difficulties in grading and guidelines to separate “atypical neurofibroma” from MPNST are provided. There is an increasing literature of MPNST mimics which neuropathologists must be aware of, including synovial sarcoma and

  3. Changes in myelin sheath thickness and internode geometry in the rabbit phrenic nerve during growth.

    PubMed Central

    Friede, R L; Brzoska, J; Hartmann, U

    1985-01-01

    The rabbit phrenic nerve was studied at seven phases of growth from the newborn to the adult to determine the length of the nerve fibres, the length of the internodes, the fibre calibre, the geometric proportions of the internodes and the thickness of the myelin sheaths. The elongation of the internodes corresponded precisely to the elongation of the nerve, indicating a constant number of approximately 140 internodes per fibre, each internode elongating commensurate with body growth. Internode elongation was accompanied by increases in fibre calibre, but these parameters did not change in precise proportion. The internodes of thick fibres were relatively short for calibre, as defined by the length/diameter quotient. This trend of foreshortening changed during growth. Sheath thickness, defined by the quotient axon diameter/fibre diameter, was determined with a computer-assisted method. Fibres of young rabbits had relatively thin sheaths for axon calibre, compared with adult rabbits. The changes in sheath thickness corresponded to the changes in internode geometry. This was consistent with previous studies showing that elongation or foreshortening of an internode of a given calibre has a slight, but definite effect on the thickness of its myelin sheath. PMID:3870716

  4. Role of CD44 in Malignant Peripheral Nerve Sheath Tumor Growth and Metastasis

    DTIC Science & Technology

    2002-09-01

    Malignant peripheral nerve sheath tumors ( MPNSTs ) are aggressive malignancies that arise within peripheral nerves. These tumors occur with increased...and abnormal expression of the epidermal growth factor receptor (EGFR). We previously found that MPNSTs express increased levels of the CD44 family...kinase activity (and not increased Ras-GTP) contributes to MPNST cell invasion. We further find that EGFR contributes at least part of the elevated Src

  5. Receptor Tyrosine Kinases as Targets for Treatment of Peripheral Nerve Sheath Tumors in NF 1 Patients

    DTIC Science & Technology

    2007-03-01

    EGFR patterns by interphase cytogenetics (FISH) in malignant peripheral nerve sheath tumor (MPNST) and morphologically similar spindle cell neoplasms ...Armstrong,F., Delsol,G., Dastugue,N. and Brousset,P. (2003) Chronic myeloproliferative disorders with rearrangement of the platelet-derived growth

  6. Direct visualization of improved optic nerve pial vascular supply following tuberculum meningioma resection: case report

    PubMed Central

    Han, Seunggu J.; Magill, Stephen T.; Tarapore, Phiroz E.; Horton, Jonathan C.; McDermott, Michael W.

    2016-01-01

    Tuberculum sellae meningiomas frequently produce visual loss by direct compression from tumor, constriction of the optic nerve (ON) under the falciform ligament, and/or ON ischemia. The authors hypothesized that changes in visual function after tumor removal may be related to changes in blood supply to the ON that might be seen in the pial circulation at surgery. Indocyanine green (ICG) angiography was used to attempt to document these changes at surgery. The first patient in whom the technique was used had a left-sided, 1.4-cm, tuberculum meningioma. Time-lapse comparison of images was done postsurgery, and the comparison of video images revealed both faster initial filling and earlier complete filling of the ON pial circulation, suggesting improved pial blood flow after surgical decompression. In follow-up the patient had significant improvements in both visual acuity and visual fields function. Intraoperative ICG angiography of the ON can demonstrate measurable changes in pial vascular flow that may be predictive of postoperative visual outcome. The predictive value of this technique during neurosurgical procedures around the optic apparatus warrants further investigation in a larger cohort. PMID:26684783

  7. Peripheral Nerve Diffusion Tensor Imaging: Assessment of Axon and Myelin Sheath Integrity.

    PubMed

    Heckel, A; Weiler, M; Xia, A; Ruetters, M; Pham, M; Bendszus, M; Heiland, S; Baeumer, P

    2015-01-01

    To investigate the potential of diffusion tensor imaging (DTI) parameters as in-vivo biomarkers of axon and myelin sheath integrity of the median nerve in the carpal tunnel as validated by correlation with electrophysiology. MRI examinations at 3T including DTI were conducted on wrists in 30 healthy subjects. After manual segmentation of the median nerve quantitative analysis of fractional anisotropy (FA) as well as axial, radial and mean diffusivity (AD, RD, and MD) was carried out. Pairwise Pearson correlations with electrophysiological parameters comprising sensory nerve action potential (SNAP) and compound muscle action potential (CMAP) as markers of axon integrity, and distal motor latency (dml) and sensory nerve conduction velocity (sNCV) as markers of myelin sheath integrity were computed. The significance criterion was set at P=0.05, Bonferroni corrected for multiple comparisons. DTI parameters showed a distinct proximal-to-distal profile with FA, MD, and RD extrema coinciding in the center of the carpal tunnel. AD correlated with CMAP (r=0.50, p=0.04, Bonf. corr.) but not with markers of myelin sheath integrity. RD correlated with sNCV (r=-0.53, p=0.02, Bonf. corr.) but not with markers of axon integrity. FA correlated with dml (r=-0.63, p=0.002, Bonf. corr.) and sNCV (r=0.68, p=0.001, Bonf. corr.) but not with markers of axon integrity. AD reflects axon integrity, while RD (and FA) reflect myelin sheath integrity as validated by correlation with electrophysiology. DTI parameters consistently indicate a slight decrease of structural integrity in the carpal tunnel as a physiological site of median nerve entrapment. DTI is particularly sensitive, since these findings are observed in healthy participants. Our results encourage future studies to evaluate the potential of DTI in differentiating axon from myelin sheath injury in patients with manifest peripheral neuropathies.

  8. Methylation-based classification of benign and malignant peripheral nerve sheath tumors.

    PubMed

    Röhrich, Manuel; Koelsche, Christian; Schrimpf, Daniel; Capper, David; Sahm, Felix; Kratz, Annekathrin; Reuss, Jana; Hovestadt, Volker; Jones, David T W; Bewerunge-Hudler, Melanie; Becker, Albert; Weis, Joachim; Mawrin, Christian; Mittelbronn, Michel; Perry, Arie; Mautner, Victor-Felix; Mechtersheimer, Gunhild; Hartmann, Christian; Okuducu, Ali Fuat; Arp, Mirko; Seiz-Rosenhagen, Marcel; Hänggi, Daniel; Heim, Stefanie; Paulus, Werner; Schittenhelm, Jens; Ahmadi, Rezvan; Herold-Mende, Christel; Unterberg, Andreas; Pfister, Stefan M; von Deimling, Andreas; Reuss, David E

    2016-06-01

    The vast majority of peripheral nerve sheath tumors derive from the Schwann cell lineage and comprise diverse histological entities ranging from benign schwannomas and neurofibromas to high-grade malignant peripheral nerve sheath tumors (MPNST), each with several variants. There is increasing evidence for methylation profiling being able to delineate biologically relevant tumor groups even within the same cellular lineage. Therefore, we used DNA methylation arrays for methylome- and chromosomal profile-based characterization of 171 peripheral nerve sheath tumors. We analyzed 28 conventional high-grade MPNST, three malignant Triton tumors, six low-grade MPNST, four epithelioid MPNST, 33 neurofibromas (15 dermal, 8 intraneural, 10 plexiform), six atypical neurofibromas, 43 schwannomas (including 5 NF2 and 5 schwannomatosis associated cases), 11 cellular schwannomas, 10 melanotic schwannomas, 7 neurofibroma/schwannoma hybrid tumors, 10 nerve sheath myxomas and 10 ganglioneuromas. Schwannomas formed different epigenomic subgroups including a vestibular schwannoma subgroup. Cellular schwannomas were not distinct from conventional schwannomas. Nerve sheath myxomas and neurofibroma/schwannoma hybrid tumors were most similar to schwannomas. Dermal, intraneural and plexiform neurofibromas as well as ganglioneuromas all showed distinct methylation profiles. Atypical neurofibromas and low-grade MPNST were indistinguishable with a common methylation profile and frequent losses of CDKN2A. Epigenomic analysis finds two groups of conventional high-grade MPNST sharing a frequent loss of neurofibromin. The larger of the two groups shows an additional loss of trimethylation of histone H3 at lysine 27 (H3K27me3). The smaller one retains H3K27me3 and is found in spinal locations. Sporadic MPNST with retained neurofibromin expression did not form an epigenetic group and most cases could be reclassified as cellular schwannomas or soft tissue sarcomas. Widespread immunohistochemical loss

  9. Surgical strategies for managing foraminal nerve sheath tumors: the emerging role of CyberKnife ablation

    PubMed Central

    Murovic, Judith A.; Charles Cho, S.

    2009-01-01

    Sixteen Stanford University Medical Center (SUMC) patients with foraminal nerve sheath tumors had charts reviewed. CyberKnife radiosurgery was innovative in management. Parameters were evaluated for 16 foraminal nerve sheath tumors undergoing surgery, some with CyberKnife. Three neurofibromas had associated neurofibromatosis type 1 (NF1). Eleven patients had one resection; others had CyberKnife after one (two) and two (three) operations. The malignant peripheral nerve sheath tumor (MPNST) had prior field-radiation and adds another case. Approaches included laminotomy and laminectomies with partial (three) or total (two) facetectomies/fusions. Two cases each had supraclavicular, lateral extracavitary, retroperitoneal and Wiltze and costotransversectomy/thoracotomy procedures. Two underwent a laminectomy/partial facetectomy, then CyberKnife. Pre-CyberKnife, one of two others had a laminectomy/partial facetectomy, then total facetectomy/fusion and the other, two supraclavicular approaches. The MPNST had a hemi-laminotomy then laminectomy/total facetectomy/fusion, followed by CyberKnife. Roots were preserved, except in two. Of 11 single-operation-peripheral nerve sheath tumors, the asymptomatic case remained stable, nine (92%) improved and one (9%) worsened. Examinations remained intact in three (27%) and improved in seven (64%). Two having a single operation then CyberKnife had improvement after both. Of two undergoing two operations, one had symptom resolution post-operatively, worsened 4 years post-CyberKnife then has remained unchanged after re-operation. The other such patient improved post-operatively, had no change after re-operation and improved post-CyberKnife. The MPNST had presentation improvement after the first operation, worsened and after the second surgery \\and CyberKnife, the patient expired from tumor spread. In conclusion, surgery is beneficial for pain relief and function preservation in foraminal nerve sheath tumors. Open surgery with Cyber

  10. Surgical strategies for managing foraminal nerve sheath tumors: the emerging role of CyberKnife ablation.

    PubMed

    Murovic, Judith A; Charles Cho, S; Park, Jon

    2010-02-01

    Sixteen Stanford University Medical Center (SUMC) patients with foraminal nerve sheath tumors had charts reviewed. CyberKnife radiosurgery was innovative in management. Parameters were evaluated for 16 foraminal nerve sheath tumors undergoing surgery, some with CyberKnife. Three neurofibromas had associated neurofibromatosis type 1 (NF1). Eleven patients had one resection; others had CyberKnife after one (two) and two (three) operations. The malignant peripheral nerve sheath tumor (MPNST) had prior field-radiation and adds another case. Approaches included laminotomy and laminectomies with partial (three) or total (two) facetectomies/fusions. Two cases each had supraclavicular, lateral extracavitary, retroperitoneal and Wiltze and costotransversectomy/thoracotomy procedures. Two underwent a laminectomy/partial facetectomy, then CyberKnife. Pre-CyberKnife, one of two others had a laminectomy/partial facetectomy, then total facetectomy/fusion and the other, two supraclavicular approaches. The MPNST had a hemi-laminotomy then laminectomy/total facetectomy/fusion, followed by CyberKnife. Roots were preserved, except in two. Of 11 single-operation-peripheral nerve sheath tumors, the asymptomatic case remained stable, nine (92%) improved and one (9%) worsened. Examinations remained intact in three (27%) and improved in seven (64%). Two having a single operation then CyberKnife had improvement after both. Of two undergoing two operations, one had symptom resolution post-operatively, worsened 4 years post-CyberKnife then has remained unchanged after re-operation. The other such patient improved post-operatively, had no change after re-operation and improved post-CyberKnife. The MPNST had presentation improvement after the first operation, worsened and after the second surgery \\and CyberKnife, the patient expired from tumor spread. In conclusion, surgery is beneficial for pain relief and function preservation in foraminal nerve sheath tumors. Open surgery with Cyber

  11. Peripheral Nerve Diffusion Tensor Imaging: Assessment of Axon and Myelin Sheath Integrity

    PubMed Central

    Heckel, A.; Weiler, M.; Xia, A.; Ruetters, M.; Pham, M.; Bendszus, M.; Heiland, S.; Baeumer, P.

    2015-01-01

    Purpose To investigate the potential of diffusion tensor imaging (DTI) parameters as in-vivo biomarkers of axon and myelin sheath integrity of the median nerve in the carpal tunnel as validated by correlation with electrophysiology. Methods MRI examinations at 3T including DTI were conducted on wrists in 30 healthy subjects. After manual segmentation of the median nerve quantitative analysis of fractional anisotropy (FA) as well as axial, radial and mean diffusivity (AD, RD, and MD) was carried out. Pairwise Pearson correlations with electrophysiological parameters comprising sensory nerve action potential (SNAP) and compound muscle action potential (CMAP) as markers of axon integrity, and distal motor latency (dml) and sensory nerve conduction velocity (sNCV) as markers of myelin sheath integrity were computed. The significance criterion was set at P=0.05, Bonferroni corrected for multiple comparisons. Results DTI parameters showed a distinct proximal-to-distal profile with FA, MD, and RD extrema coinciding in the center of the carpal tunnel. AD correlated with CMAP (r=0.50, p=0.04, Bonf. corr.) but not with markers of myelin sheath integrity. RD correlated with sNCV (r=-0.53, p=0.02, Bonf. corr.) but not with markers of axon integrity. FA correlated with dml (r=-0.63, p=0.002, Bonf. corr.) and sNCV (r=0.68, p=0.001, Bonf. corr.) but not with markers of axon integrity. Conclusion AD reflects axon integrity, while RD (and FA) reflect myelin sheath integrity as validated by correlation with electrophysiology. DTI parameters consistently indicate a slight decrease of structural integrity in the carpal tunnel as a physiological site of median nerve entrapment. DTI is particularly sensitive, since these findings are observed in healthy participants. Our results encourage future studies to evaluate the potential of DTI in differentiating axon from myelin sheath injury in patients with manifest peripheral neuropathies. PMID:26114630

  12. Acute optic nerve sheath fenestration in humans using the free electron laser (FEL): a case report

    NASA Astrophysics Data System (ADS)

    Joos, Karen M.; Mawn, Louise A.; Shen, Jin-Hui; Jansen, E. Duco; Casagrande, Vivien A.

    2002-06-01

    Our previous studies using rabbits and monkeys showed that the Amide II wavelength (6.45 micrometers ) produced by the FEL could efficiently produce an optic nerve sheath fenestration with minimal damage. In order to determine if the technology safely could be applied to human surgery, we used 2 blind human eyes during enucleation to compare the results of producing fenestrations with the FEL or a scissors. FDA and Vanderbilt IRB approvals, and individual patient consents were obtained. The FEL energy was transmitted to a human operating room. After disinsertion of the medial rectus muscle, an optic nerve sheath fenestration (2 mm diameter) was made with either the FEL (6.45 micrometers , 325 micrometers spot size, 30 Hz, 3 mJ) through a hollow waveguide surgical probe or with a scissors. The enucleation was then completed. The optic nerve was dissected from the globe and fixed. Specimens were examined histologically. Dural incisions were effective with both methods. FEL energy at 6.45 micrometers can be transmitted to an operating room and delivered to human ocular tissue through a hollow waveguide surgical probe. This FEL wavelength can produce an optic nerve sheath fenestration without acute direct damage to the nerve in this case report.

  13. Morphometric Analysis of Connective Tissue Sheaths of Sural Nerve in Diabetic and Nondiabetic Patients

    PubMed Central

    Kundalić, Braca; Ugrenović, Slađana; Jovanović, Ivan; Stefanović, Natalija; Petrović, Vladimir; Kundalić, Jasen; Stojanović, Vesna; Živković, Vladimir; Antić, Vladimir

    2014-01-01

    One of the most common complications of diabetes mellitus is diabetic neuropathy. It may be provoked by metabolic and/or vascular factors, and depending on duration of disease, various layers of nerve may be affected. Our aim was to investigate influence of diabetes on the epineurial, perineurial, and endoneurial connective tissue sheaths. The study included 15 samples of sural nerve divided into three groups: diabetic group, peripheral vascular disease group, and control group. After morphological analysis, morphometric parameters were determined for each case using ImageJ software. Compared to the control group, the diabetic cases had significantly higher perineurial index (P < 0.05) and endoneurial connective tissue percentage (P < 0.01). The diabetic group showed significantly higher epineurial area (P < 0.01), as well as percentage of endoneurial connective tissue (P < 0.01), in relation to the peripheral vascular disease group. It is obvious that hyperglycemia and ischemia present in diabetes lead to substantial changes in connective tissue sheaths of nerve, particularly in peri- and endoneurium. Perineurial thickening and significant endoneurial fibrosis may impair the balance of endoneurial homeostasis and regenerative ability of the nerve fibers. Future investigations should focus on studying the components of extracellular matrix of connective tissue sheaths in diabetic nerves. PMID:25147820

  14. Superficial malignant peripheral nerve sheath tumor arising from diffuse neurofibroma in a neurofibromatosis type 1 patient.

    PubMed

    Inoue, Takuya; Kuwashiro, Maki; Misago, Noriyuki; Narisawa, Yutaka

    2014-07-01

    Malignant peripheral nerve sheath tumors (MPNST) are regarded as sarcomas that arise from peripheral nerves or that display differentiation along the lines of the various elements of the nerve sheath. These tumors occur in deep soft tissues, but superficial primary MPNST with a cutaneous or subcutaneous origin have rarely been reported. A 70-year-old woman presented with a 3-4-year history of a slowly enlarging soft nodule on the left side of her neck. The histopathological diagnosis of the nodule was low-grade MPNST arising from diffuse neurofibroma. There was increased cellularity, but no necrosis or mitotic activity. These histopathological findings pose difficulties in differential diagnosis from a neurofibroma with atypical histological features. We report a rare case of superficial MPNST arising from diffuse neurofibroma associated with underlying occipital bone dysplasia in a neurofibromatosis type 1 patient.

  15. Visual outcome after fronto-temporo-orbito-zygomatic approach combined with early extradural and intradural optic nerve decompression in tuberculum and diaphragma sellae meningiomas.

    PubMed

    Mortini, Pietro; Barzaghi, Lina Raffaella; Serra, Carlo; Orlandi, Vittoria; Bianchi, Stefania; Losa, Marco

    2012-07-01

    The surgical challenge of the treatment of tuberculum (TSMs) and diaphragma sellae meningiomas (DSMs) is to preserve or improve the visual function. Extradural and intradural optic nerve decompression should reduce surgical trauma of the nerve achieving a good visual result. We reported 37 consecutive TSMs and DSMs operated through fronto-temporo-orbito-zygomatic approach with extradural unroofing of the optical canal and early intradural incision of the dural sheath. Visual data were recorded measuring the visual impairment score (VIS), the visual acuity (VA), the visual field (VF) and the postoperative improvement. A good visual outcome (VIS improved or unchanged) was obtained in 97.2% of patients (35/36). The evaluation of 72 eyes showed a good outcome (VA and VF unchanged or improved) in 98.6% (71/72 eyes). The degree of preoperative VA and VF impairment was the only factor correlating with the postoperative improvement of VA (P<.001 and P=.018) and VF defect (P<.001). Worsening of visual function occurred in 1/37 patient (2.7%). Using this surgical technique we achieved a high improvement rate of visual defects and a low frequency of worsening. Copyright © 2011 Elsevier B.V. All rights reserved.

  16. Epicardial Ablation: Prevention of Phrenic Nerve Damage by Pericardial Injection of Saline and the Use of a Steerable Sheath

    PubMed Central

    Neven, Kars; Fernandez-Armenta, Juan; Andreu, David; Berruezo, Antonio

    2014-01-01

    Because of the close proximity of the phrenic nerve to the pericardium, phrenic nerve damage caused by epicardial ablation can easily occur. We report two cases of epicardial VT ablation where pericardial injection of saline, combined with the use of a steerable sheath, successfully prevents the phrenic nerve from being damaged. PMID:24669108

  17. Epicardial ablation: prevention of phrenic nerve damage by pericardial injection of saline and the use of a steerable sheath.

    PubMed

    Neven, Kars; Fernandez-Armenta, Juan; Andreu, David; Berruezo, Antonio

    2014-03-01

    Because of the close proximity of the phrenic nerve to the pericardium, phrenic nerve damage caused by epicardial ablation can easily occur. We report two cases of epicardial VT ablation where pericardial injection of saline, combined with the use of a steerable sheath, successfully prevents the phrenic nerve from being damaged.

  18. Cluster analysis of immunohistochemical profiles in synovial sarcoma, malignant peripheral nerve sheath tumor, and Ewing sarcoma.

    PubMed

    Olsen, Stephen H; Thomas, Dafydd G; Lucas, David R

    2006-05-01

    As a result of overlapping morphologic and immunohistochemical features, it can be difficult to distinguish synovial sarcoma, malignant peripheral nerve sheath tumor, and Ewing sarcoma/primitive neuroectodermal tumor in core biopsies. To analyze and compare immunohistochemical profiles, we stained tissue microarrays of 23 synovial sarcomas, 23 malignant peripheral nerve sheath tumors, and 27 Ewing sarcomas with 22 antibodies potentially useful in the differential diagnosis, and analyzed the data with cluster analysis. Stain intensity was scored as none, weak, or strong. For CD99, tumors with membranous accentuation were independently categorized. Cluster analysis sorted five groups, with like tumors clustering together. Synovial sarcoma clustered into two groups: one cytokeratin and EMA positive (n = 11), the other mostly cytokeratin negative, EMA positive, bcl-2 positive and mostly CD56 positive (n = 9). Malignant peripheral nerve sheath tumor clustered into two groups: one S100 positive, with nestin and NGFR positivity in most (n = 10), the other mostly S100 negative, and variably but mostly weakly positive for nestin and NGFR (n = 11). Ewing sarcomas clustered into a single group driven by membranous CD99 staining. Thirteen cases failed to cluster (outliers), while three Ewing sarcomas clustered into groups of other tumor types. Paired antibodies for each tumor type determined by visual assessment of cluster analysis data and statistical calculations of specificity, sensitivity, and predictive values showed that EMA/CK7 for synovial sarcoma, nestin/S100 for malignant peripheral nerve sheath tumor, and membranous CD99/Fli-1 for Ewing sarcoma yielded high specificity and positive predictive values. Cluster analysis also highlighted aberrant staining reactions and diagnostic pitfalls in these tumors. Hierarchical cluster analysis is an effective method for analyzing high-volume immunohistochemical data.

  19. Sporadic Multifocal Malignant Peripheral Nerve Sheath Tumor-A Rare Presentation: Multifocal MPNST.

    PubMed

    Leena, J B; Fernandes, Hilda; Swethadri, G K

    2013-06-01

    Malignant peripheral nerve sheath tumors(MPNST) are uncommon neoplasms with an incidence of 0.001% in general population. Multifocality is a rare manifestation of MPNST . A case of a 65 year old patient who presented with multiple swellings involving the neck, extremity and back without associated neurofibromatosis is reported for its rarity of presentation.. Diagnosis was made by FNAC and confirmed by peroperative findings and histopathology.

  20. Role of CD44 in Malignant Peripheral Nerve Sheath Tumor Growth and Metastasis

    DTIC Science & Technology

    2003-09-01

    Malignant peripheral nerve sheath tumors ( MPNST ) are aggressive, difficult to treat tumors that occur in type I neurofibromatosis patients with an...survival rate. We previously found that MPNSTs overexpress the CD44 tranmembrane glycoprotein and that reducing CD44 expression partially inhibits MPNST ...depends on Src kinase and that Src kinase activity promotes MPNST invasion (Su et al., 2003a) . Furthermore, we show that MPNST cell invasion depends on

  1. Role of CD44 in Malignant Peripheral Nerve Sheath Tumor Growth and Metastasis

    DTIC Science & Technology

    2001-09-01

    Malignant peripheral nerve sheath tumors ( MPNST ) are aggressive, difficult to treat tumors that occur in type I neurofibromatosis patients with an...survival rate. We previously found that MPNSTs overexpress the CD44 tranmembrane glycoprotein and that reducing Cc44 expression inhibits MPNST cell...Src kinase. Furthermore, we show that MPNST cell invasion depends on an autocrine loop involving MCF, an MCF activating enzyme (MGFA), and c-Met, all of

  2. Genetic Evaluation of Peripheral Nerve Sheath Tumors in Neurofibromatosis Type I

    DTIC Science & Technology

    2003-10-01

    neurofibromatosis type 1 (NF1). Archival and prospectively acquired plexiform neurofibromas and malignant peripheral nerve sheath tumors ( MPNSTs ) were collected...evaluated, whereas a relatively small number of MPNSTs have been collected for complete analysis. Immunohistochemical stains have been developed to...distinguish -high-grade versus low-grade MPNSTs and plexiform neurofibromas. The genome of plexiform neurofibromas is relatively stable, compared to the

  3. Novel Therapeutic Development of NF1-Associated Malignant Peripheral Nerve Sheath Tumor (MPNST)

    DTIC Science & Technology

    2016-08-01

    AWARD NUMBER: W81XWH-15-1-0124 TITLE: Novel Therapeutic Development of NF1- Associated Malignant Peripheral Nerve Sheath Tumor (MPNST...COVERED 15 Jul 2015 - 14 Jul 2016 4. TITLE AND SUBTITLE 5a. CONTRACT NUMBER Novel Therapeutic Development of NF1- Associated Malignant Peripheral...components (EED or SUZ12), CDKN2A (81%) and NF1 (72% of non-NF1- associated ) in all MPNSTs, and the three components significantly co-occur, suggesting their

  4. Lost in translation: ambiguity in nerve sheath tumor nomenclature and its resultant treatment effect.

    PubMed

    Bernthal, Nicholas M; Jones, Kevin B; Monument, Michael J; Liu, Ting; Viskochil, David; Randall, R Lor

    2013-05-08

    There is much ambiguity surrounding the diagnosis of nerve sheath tumors, including atypical neurofibroma and low-grade MPNST, and yet, the distinction between these entities designates either benign or malignant behavior and thus carries presumed profound prognostic importance that often guides treatment. This study reviews the diagnostic criteria used to designate atypical neurofibroma from low-grade MPNSTs and reviews existing literature the natural history of each of these tumors to see if the distinction is, in fact, of importance.

  5. Malignant peripheral nerve sheath tumour in the ischio-rectal fossa.

    PubMed

    Teoh, K H; Reddy, S; Beggs, I; Al-Nafussi, A; Mander, B J; Porter, D E

    2009-06-01

    Primary sarcomas in the ischiorectal fossa are occasionally reported and represent a significant challenge due to the proximity of rectum, levator muscles and pudendal neurovascular structures. We report a case in which the diagnosis changed between biopsy (desmoid tumour) and resection (malignant peripheral nerve sheath tumour), requiring a multidisciplinary surgical approach involving different sub-specialties. It also illustrates the importance of undertaking sarcoma surgery in a recognized sarcoma centre with sarcoma expertise available across a range of disciplines.

  6. Engineered Herpes Simplex Viruses for the Treatment of Malignant Peripheral Nerve Sheath Tumors

    DTIC Science & Technology

    2012-09-01

    AD_________________ Award Number: W81XWH-11-1-0498 TITLE: Engineered Herpes Simplex Viruses for the...August 2012 4. TITLE AND SUBTITLE Engineered Herpes Simplex Viruses for the Treatment of Malignant Peripheral Nerve Sheath Tumors 5a. CONTRACT NUMBER...for each blot. Glyco-protein D is produced at extraordinarily high levels by our herpes simplex virus, and thus, it is quite common in herpes simplex

  7. [Malignant peripheral nerve sheath tumor with perineural differentiation (malignant perineurinoma) of the cervix uteri].

    PubMed

    Dolzhikov, A A; Mukhina, T S

    2014-01-01

    The paper describes a case of a malignant peripheral nerve sheath tumor with perineural differentiation and at the rare site of the cervix uteri in a 57-year-old patient. The diagnosis was established on the basis of extensive immunohistochemical examination, by excluding the similar neoplasms and detecting an immunophenotype characteristic of perineural differentiation. There are data available in the literature on the morphological and immunophenotypical characteristics of this tumor.

  8. Induction of malignant peripheral nerve sheath tumors in European hamsters with 1,1-dimethylhydrazine (UDMH).

    PubMed

    Ernst, H; Rittinghausen, S; Wahnschaffe, U; Mohr, U

    1987-06-01

    A rate of up to 43% of malignant peripheral nerve sheath tumors (PNST) was induced in European hamsters (EH) after weekly s.c. administration of 1,1-dimethylhydrazine (UDMH). The overall neoplastic response in the treated EH was also elevated as compared to the untreated controls. Histologically, the malignant PNST were neurofibrosarcomas and melanotic as well as unpigmented schwannomas. The occurrence of melanotic schwannomas is briefly discussed with regard to the histogenesis of this rare tumor type.

  9. Quantitative relationships between axoplasm and Schwann cell sheath in unmyelinated nerve fibres. An electron microscope study.

    PubMed Central

    Pannese, E; Arcidiacono, G; Frattola, D; Rigamonti, L; Procacci, P; Ledda, M

    1988-01-01

    The quantitative relationships between the size of the Schwann cell sheath and that of its related axoplasm were studied by electron microscopy in cross sections of bundles of unmyelinated axons (Remak bundles) of the spinal roots of lizard (Lacerta muralis). It was found that (i) the cross sectional area of the Schwann cell sheath is directly proportional to that of its related axoplasm (correlation coefficient 0.84), and (ii) the ratio between the cross sectional area of the Schwann cell sheath and that of its related axoplasm tends to diminish as the cross sectional area of the latter increases. Thus, under normal conditions, in the bundles of unmyelinated axons of the spinal roots of lizard a quantitative balance exists between the nerve tissue and its associated glial tissue. These results agree with those previously obtained in the myelinated fibres of the same region and in the spinal ganglia of the lizard, gecko, cat and rabbit. Some of the mechanisms probably involved in the control of the quantitative balance between nerve tissue and its associated glial tissue in peripheral nerves are listed. Images Fig. 1 PMID:3248972

  10. A Vascular Malformation Presenting as a Peripheral Nerve Sheath Tumor

    PubMed Central

    Parmar, Vikas; Haldeman, Clayton; Amaefuna, Steve; Hanna, Amgad S.

    2016-01-01

    We present the case of a venous malformation (VM) masquerading as a schwannoma. VMs are thin-walled vascular dilations of various sizes that typically present as soft, compressible, blue masses that are associated with pain or dysesthesia. VMs are commonly found in the head and neck as well as the distal extremities. Notably, slow-flow VMs are hypointense on T1-weighted imaging, hyperintense on T2-weighted imaging, and enhance markedly with contrast. However, VMs tend to be poorly circumscribed and fraught with venous lakes and phleboliths. Conservative therapy and sclerotherapy are the primary treatment options. In this case report, we present a VM presenting near the neurovascular bundle of the upper extremity axilla. Our case is unique in that the patient presented with symptoms and imaging qualities characteristic for a peripheral nerve schwannoma. PMID:28077959

  11. Growth-associated protein 43 in differentiating peripheral nerve sheath tumors from other non-neural spindle cell neoplasms.

    PubMed

    Chen, Wei-Shen; Chen, Pei-Ling; Lu, Dongsi; Lind, Anne C; Dehner, Louis P

    2014-02-01

    The malignant peripheral nerve sheath tumor is a relatively uncommon type of soft tissue sarcoma arising from a peripheral nerve or extraneural soft tissues and showing nerve sheath differentiation. The diagnosis of malignant peripheral nerve sheath tumor is one of the most challenging tasks in surgical pathology because of its uncommon type (5-10% soft tissue sarcomas), morphologic resemblance to other spindle cell neoplasms and lack of sensitive and specific immunohistochemical markers. The pathologic diagnosis is more straightforward in the clinical setting of neurofibromatosis-1, but problems are mainly centered on the non-neurofibromatosis-1 malignant peripheral nerve sheath tumors. To date, S100 protein is the most widely applied marker in the case of a suspected malignant peripheral nerve sheath tumor, yet its suboptimal sensitivity and its expression in other spindle cell neoplasms, including spindle cell melanoma, clear-cell sarcoma, leiomyosarcoma and monophasic synovial sarcoma, add to the diagnostic conundrum. Growth-associated protein 43 (GAP43), a membrane-associated phosphoprotein expressed in neuronal growth cones and Schwann cell precursors during neural development and axonal regeneration, was applied to a set of nerve sheath and non-nerve sheath spindle cell neoplasms. The findings in this study indicate that GAP43 is expressed in malignant peripheral nerve sheath tumors (n=18/21; 86%) and demonstrates a sensitivity superior to S100 protein (n=13/21; 62%). GAP43 is also positive in neurofibromas (n=17/18; 94%), schwannomas (n=11/12; 92%) and desmoplastic melanomas (n=7/10; 70%). In contrast, it is negative in the non-desmoplastic spindle cell melanomas (n=20/22; 91%). Of the other non-neural soft tissue sarcomas, GAP43 is non-reactive in most leiomyosarcomas (n=14/16; 88%) and clear-cell sarcomas (n=8/8), and only focally positive in monophasic synovial sarcomas (n=3/7; 43%). GAP43 is seemingly a highly sensitive marker for peripheral nerve

  12. Comparison of Outside Versus Inside Brachial Plexus Sheath Injection for Ultrasound-Guided Interscalene Nerve Blocks.

    PubMed

    Maga, Joni; Missair, Andres; Visan, Alex; Kaplan, Lee; Gutierrez, Juan F; Jain, Annika R; Gebhard, Ralf E

    2016-02-01

    Ultrasound-guided interscalene brachial plexus blocks are commonly used to provide anesthesia for the shoulder and proximal upper extremity. Some reviews identify a sheath that envelops the brachial plexus as a potential tissue plane target, and current editorials in the literature highlight the need to establish precise and reproducible injection targets under ultrasound guidance. We hypothesize that an injection of a local anesthetic inside the brachial plexus sheath during ultrasound-guided interscalene nerve blocks will result in enhanced procedure success and provide a consistent tissue plane target for this approach with a reproducible and characteristic local anesthetic spread pattern. Sixty patients scheduled for shoulder surgery with a preoperative interscalene block for postoperative pain management were enrolled in this prospective randomized observer-blinded study. Each patient was randomly assigned to receive a single-shot interscalene block either inside or outside the brachial plexus sheath. The rate of complete motor and sensory blocks of the axillary nerve territory 10 minutes after local anesthetic injection for the inside group was 70% versus 37% for the outside group (P < .05). At all measurement intervals beyond 10 minutes, however, neither group showed a statistically significant difference in complete sensory blockade. The incidence rates of transient paresthesia during needle passage were 6.7% for the outside group and 96.7% for the inside group (P < .05). Except for faster onset, this prospective randomized trial did not find any advantages to performing an interscalene block inside the brachial plexus sheath. There was a higher incidence of transient paresthesia when injections were performed inside compared to outside the sheath. © 2016 by the American Institute of Ultrasound in Medicine.

  13. Malignant peripheral nerve sheath tumors of the eighth cranial nerve arising without prior irradiation.

    PubMed

    Carlson, Matthew L; Jacob, Jeffrey T; Habermann, Elizabeth B; Glasgow, Amy E; Raghunathan, Aditya; Link, Michael J

    2016-11-01

    OBJECTIVE Malignant peripheral nerve sheath tumors (MPNSTs) of the eighth cranial nerve (CN) are exceedingly rare. To date the literature has focused on MPNSTs occurring after radiation therapy for presumed benign vestibular schwannomas (VSs), while MPNSTs arising without prior irradiation have received little attention. The objectives of the current study are to characterize the epidemiology, clinical presentation, disease course, and outcome using a large national cancer registry database and a systematic review of the English literature. Additionally, a previously unreported case is presented. METHODS The authors conducted an analysis of the Surveillance, Epidemiology, and End Results (SEER) database, a systematic review of the literature, and present a case report. Data from all patients identified in the SEER database with a diagnosis of MPNST involving the eighth CN, without a history of prior radiation, were analyzed. Additionally, all cases reported in the English literature between January 1980 and March 2015 were reviewed. Finally, 1 previously unreported case is presented. RESULTS The SEER registries identified 30 cases between 1992 and 2012. The average incidence was 0.017 per 1 million persons per year (range 0.000-0.0687 per year). The median age at diagnosis was 55 years, and 16 (53%) were women. Thirteen cases were diagnosed upon autopsy. Of the 17 cases diagnosed while alive, the median follow-up was 118 days, with 3 deaths (18%) observed. When compared with the incidence of benign VS, 1041 VSs present for every 1 MPNST arising from the eighth CN. Including a previously unreported case from the authors' center, a systematic review of the English literature yielded 24 reports. The median age at diagnosis was 44 years, 50% were women, and the median tumor size at diagnosis was 3 cm. Eleven patients (46%) reported isolated audiovestibular complaints typical for VS while 13 (54%) exhibited facial paresis or other signs of a more aggressive process

  14. Metastatic Neuroendocrine Carcinoma of Unknown Origin Arising in the Femoral Nerve Sheath.

    PubMed

    Candy, Nicholas; Young, Adam; Allinson, Kieren; Carr, Oliver; McMillen, Jason; Trivedi, Rikin

    2017-08-01

    Metastatic neuroendocrine carcinoma of unknown origin is a rare condition, usually presenting with lesions in the liver and/or lung. We present the first reported case of a metastatic neuroendocrine carcinoma of unknown origin arising in the femoral nerve sheath. Magnetic resonance imaging demonstrated what was thought to be a schwannoma in the left femoral nerve sheath in the proximal femoral triangle, immediately inferior to the anterior inferior iliac spine. At the time of operation, the tumor capsule was invading surrounding tissue, as well as three trunks of the femoral nerve. The patient underwent a subtotal resection, preserving the integrity of the residual functioning femoral nerve trunks. Histologic evaluation determined that the tumor had features consistent with a metastatic neuroendocrine carcinoma of unknown primary origin. The patient recovered well postoperatively, and subsequent radiologic evaluation failed to demonstrate a potential primary site. Unfortunately, the patient re-presented with disease progression and was subsequently referred to palliative care. We recommend that there is a definite role for surgery in the management of solitary neuroendocrine carcinoma of unknown origin. Copyright © 2017 Elsevier Inc. All rights reserved.

  15. Optic nerve sheath fenestration using a Raman-shifted alexandrite laser

    PubMed Central

    Kozub, John; Shen, Jin-H.; Joos, Karen M.; Prasad, Ratna; Hutson, M. Shane

    2016-01-01

    Background and Objective Optic nerve sheath fenestration is an established procedure for relief of potentially damaging overpressure on the optic nerve resulting from idiopathic intracranial hypertension. Prior work showed that a mid-IR free-electron laser could be delivered endoscopically and used to produce an effective fenestration. This study evaluates the efficacy of fenestration using a table-top mid-IR source based on a Raman-shifted alexandrite (RSA) laser. Study Design/Materials and Methods Porcine optic nerves were ablated using light from an RSA laser at wavelengths of 6.09, 6.27 and 6.43 μm and pulse energies up to 3 mJ using both free-space and endoscopic beam delivery through 250-μm I.D. hollow-glass waveguides. Waveguide transmission was characterized, ablation thresholds and etch rates were measured, and the efficacy of endoscopic fenestration was evaluated for ex vivo exposures using both optical coherence tomography and histological analysis. Results Using endoscopic delivery, the RSA laser can effectively fenestrate porcine optic nerves. Performance was optimized at a wavelength of 6.09 μm and delivered pulse energies of 0.5-0.8 mJ (requiring 1.5-2.5 mJ to be incident on the waveguide). Under these conditions, the ablation threshold fluence was 0.8 ± 0.2 J/cm2, the ablation rate was 1-4 μm/pulse, and the margins of ablation craters showed little evidence of thermal or mechanical damage. Nonetheless, nominally identical exposures yielded highly variable ablation rates. This led to fenestrations that ranged from too deep to too shallow – either damaging the underlying optic nerve or requiring additional exposure to cut fully through the sheath. Of 48 excised nerves subjected to fenestration at 6.09 μm, 16 ex vivo fenestrations were judged as good, 23 as too deep, and 9 as too shallow. Conclusions Mid-IR pulses from the RSA laser, propagated through a flexible hollow waveguide, are capable of cutting through porcine optic nerve sheaths in

  16. Optic nerve sheath fenestration using a Raman-shifted alexandrite laser.

    PubMed

    Kozub, John; Shen, Jin H; Joos, Karen M; Prasad, Ratna; Hutson, M Shane

    2016-03-01

    Optic nerve sheath fenestration is an established procedure for relief of potentially damaging overpressure on the optic nerve resulting from idiopathic intracranial hypertension. Prior work showed that a mid-IR free-electron laser could be delivered endoscopically and used to produce an effective fenestration. This study evaluates the efficacy of fenestration using a table-top mid-IR source based on a Raman-shifted alexandrite (RSA) laser. Porcine optic nerves were ablated using light from an RSA laser at wavelengths of 6.09, 6.27, and 6.43 μm and pulse energies up to 3 mJ using both free-space and endoscopic beam delivery through 250-μm I.D. hollow-glass waveguides. Waveguide transmission was characterized, ablation thresholds and etch rates were measured, and the efficacy of endoscopic fenestration was evaluated for ex vivo exposures using both optical coherence tomography and histological analysis. Using endoscopic delivery, the RSA laser can effectively fenestrate porcine optic nerves. Performance was optimized at a wavelength of 6.09 μm and delivered pulse energies of 0.5-0.8 mJ (requiring 1.5-2.5 mJ to be incident on the waveguide). Under these conditions, the ablation threshold fluence was 0.8 ± 0.2 J/cm(2) , the ablation rate was 1-4 μm/pulse, and the margins of ablation craters showed little evidence of thermal or mechanical damage. Nonetheless, nominally identical exposures yielded highly variable ablation rates. This led to fenestrations that ranged from too deep to too shallow-either damaging the underlying optic nerve or requiring additional exposure to cut fully through the sheath. Of 48 excised nerves subjected to fenestration at 6.09 μm, 16 ex vivo fenestrations were judged as good, 23 as too deep, and 9 as too shallow. Mid-IR pulses from the RSA laser, propagated through a flexible hollow waveguide, are capable of cutting through porcine optic nerve sheaths in surgically relevant times with reasonable accuracy and low

  17. Optic Nerve Sheath as a Novel Mechanical Load on the Globe in Ocular Duction

    PubMed Central

    Demer, Joseph L.

    2016-01-01

    Purpose The optic nerve (ON) sheath's role in limiting duction has been previously unappreciated. This study employed magnetic resonance imaging (MRI) to demonstrate this constraint on adduction. Methods High-resolution, surface coil axial MRI was obtained in 11 normal adults, 14 subjects with esotropia (ET) having normal axial length (AL) < 25.8 mm, 13 myopic subjects with ET and mean AL 29.3 ± 3.3 (SD) mm, and 7 subjects with exotropia (XT). Gaze angles and ON lengths were measured for scans employing eccentric lateral fixation in which an ON became completely straightened. Results In all groups, ON straightening occurred only in the adducting, not abducting, eye. Adduction at ON straightening was 26.0 ± 8.8° in normal subjects, not significantly different from XT at 22.2 ± 11.8°. However, there was significant increase in comparable adduction in ET to 36.3 ± 9.3°, and in myopic ET to 33.6 ± 10.7° (P < 0.04). Optic nerve length at straightening was 27.6 ± 2.7 mm in normals, not significantly different from 28.2 ± 2.8 mm in ET and 27.8 ± 2.7 mm in XT. In myopic ET, ON length at straightening was significantly reduced to 24.0 ± 2.9 mm (P < 0.002) and was associated with globe retraction in adduction, suggesting ON tethering. Conclusions Large adduction may exhaust length redundancy in the normally sinuous ON and sheath, so that additional adduction must stretch the sheath and retract or deform the globe. These mechanical effects are most significant in ET with axial myopia, but may also exert traction on the posterior sclera absent strabismus or myopia. Tethering by the ON sheath in adduction is an important, novel mechanical load on the globe. PMID:27082297

  18. Pulsatile Dynamics of the Optic Nerve Sheath and Intracranial Pressure: An Exploratory In Vivo Investigation

    PubMed Central

    Brekken, Reidar; Fieggen, Graham; Selbekk, Tormod

    2016-01-01

    BACKGROUND: Raised intracranial pressure (ICP) may lead to increased stiffness of the optic nerve sheath (ONS). OBJECTIVE: To develop a method for analyzing ONS dynamics from transorbital ultrasound and investigate a potential difference between patients with raised ICP vs normal ICP. METHODS: We retrospectively analyzed data from 16 patients (≤12 years old) for whom ultrasound image sequences of the ONS had been acquired from both eyes just before invasive measurement of ICP. Eight patients had an ICP ≥20 mm Hg. The transverse motion on each side of the ONS was estimated from ultrasound, and Fourier analysis was used to extract the magnitude of the displacement corresponding to the heart rate. By calculating the normalized absolute difference between the displacements on each side of the ONS, a measure of ONS deformation was obtained. This parameter was referred to as the deformability index. According to our hypothesis, because deformability is inversely related to stiffness, we expected this parameter to be lower for ICP ≥20 mm Hg compared with ICP <20 mm Hg. The one-sided Mann-Whitney U test was used for statistical comparison. RESULTS: The deformability index was significantly lower in the group with ICP ≥20 mm Hg (median value 0.11 vs 0.24; P = .002). CONCLUSION: We present a method for assessment of ONS pulsatile dynamics using transorbital ultrasound imaging. A significant difference was noted between the patient groups, indicating that deformability of the ONS may be relevant as a noninvasive marker of raised ICP. The clinical implications are promising and should be investigated in future clinical studies. ABBREVIATIONS: AUC, area under curve ICP, intracranial pressure ONS, optic nerve sheath ONSD, optic nerve sheath diameter ROC, receiver operating characteristic PMID:26813857

  19. Malignant peripheral nerve sheath tumour (MPNST) of mandible: solving the perplexity.

    PubMed

    Patel, Shilpa; Pathak, Jigna; Dekate, Kamlesh; Mohanty, Neeta

    2015-03-11

    We present an extremely rare case of malignant peripheral nerve sheath tumour (MPNST) in a 30-year-old woman without associated neurofibromatosis 1. The patient presented with an 8 cm×4 cm lesion extending from 46 to the retro molar region involving the ramus of the right mandible associated with regional paraesthesia. Incisional biopsy revealed spindle cells with vesicular nuclei arranged in fascicles leading to a diagnosis of spindle cell lesion. Posterior segmental mandibulectomy was performed under general anaesthesia. On excisional biopsy, a definitive diagnosis of low-grade MPNST was established on the basis of immunohistochemistry. The patient was then lost to follow-up.

  20. Potential of boron neutron capture therapy (BNCT) for malignant peripheral nerve sheath tumors (MPNST).

    PubMed

    Fujimoto, Takuya; Andoh, Tooru; Sudo, Tamotsu; Fujita, Ikuo; Fukase, Naomasa; Takeuchi, Tamotsu; Sonobe, Hiroshi; Inoue, Masayoshi; Hirose, Tkanori; Sakuma, Toshiko; Moritake, Hiroshi; Sugimoto, Tohru; Kawamoto, Teruya; Fukumori, Yoshinobu; Yamamoto, Satomi; Atagi, Shinji; Sakurai, Yoshinori; Kurosaka, Masahiro; Ono, Koji; Ichikawa, Hideki; Suzuki, Minoru

    2015-12-01

    Malignant peripheral nerve sheath tumors (MPNST) are relatively rare neoplasms with poor prognosis. At present there is no effective treatment for MPNST other than surgical resection. Nonetheless, the anti-tumor effect of boron neutron capture therapy (BNCT) was recently demonstrated in two patients with MPNST. Subsequently, tumor-bearing nude mice subcutaneously transplanted with a human MPNST cell line were injected with p-borono-L-phenylalanine (L-BPA) and subjected to BNCT. Pathological studies then revealed that the MPNST cells were selectively destroyed by BNCT.

  1. Optic Nerve Sheath Diameter Ultrasound and the Diagnosis of Increased Intracranial Pressure.

    PubMed

    Hylkema, Christopher

    2016-03-01

    Ultrasound has been used for almost 30 years in a wide variety of clinical applications and environments. From the austerity of battlefields to the labor and delivery ward, ultrasound has the ability to give clinicians real-time, noninvasive diagnostic imaging. Ultrasound by emergency physicians (and all nonradiologists) has become more prevalent and has been used for examinations such as the transcranial Doppler to evaluate for stroke, cardiac function, FAST and EFAST examinations for trauma, and now increased intracranial pressure (ICP) via Optic Nerve Sheath Diameter Ultrasound (ONSD). The ONSD is a valid and reliable indicator of ICP.

  2. Giant Sporadic Low Grade Malignant Peripheral Nerve Sheath (MPNST) of Left Thigh.

    PubMed

    Nikumbh, Dhiraj B; Suryawanshi, K H; Dravid, N V; Patil, T B; Rokade, C M

    2013-06-01

    Malignant Peripheral Nerve Sheath Tumours (MPNST) are rare spindle- cell sarcomas which are derived from the schwann cells or the pleuripotent cells of the neural crest. MPNSTs are usually located in the peripheral nerve trunk roots, extremities and in the head and neck region. These tumours may arise as sporadic variants or in patients with neurofibromatosis1 (NF1). The estimated incidence of MPNSTs in the patients with NF1is 2-5% as compared to a 0.001% incidence in the general population. Herein, we are reporting a case of a giant sporadic (NF1 independent) low grade MPNST of the left thigh in 65 year female patient, which had a good prognosis.

  3. Composite phaeochromocytoma with malignant peripheral nerve sheath tumour and rhabdomyosarcomatous differentiation in a patient without von Recklinghausen disease.

    PubMed

    Gupta, R; Sharma, A; Arora, R; Vijayaraghavan, M

    2009-07-01

    The coexistence of adrenal phaeochromocytoma with non-chromaffin tumours is a rare fascinating occurrence. This category of tumours is subdivided into "composite" and "mixed". The coexistence of adrenal phaeochromocytoma with a malignant Triton tumour does not appear to have been described in the available literature so far. A unique case of composite phaeochromocytoma in a 26-year-old male patient, where the non-chromaffin component was a malignant Triton tumour composed of peripheral nerve sheath tumour and skeletal muscle differentiation, is reported. This admixture was confirmed with immunohistochemical pattern of expression. This is the first case of such a phenomenon in a composite phaeochromocytoma. The present case further widens the histomorphological range of composite phaeochromocytoma of the adrenal gland, which the histopathologist should be aware of. Since the prognosis of composite phaeochromocytoma with malignant nerve sheath tumour would be determined by the nerve sheath component, recognition of this tumour is imperative.

  4. Effects of pigment epithelium derived factor (PEDF) on malignant peripheral nerve sheath tumours (MPNSTs).

    PubMed

    Demestre, Maria; Terzi, Menderes Yusuf; Mautner, Victor; Vajkoczy, Peter; Kurtz, Andreas; Piña, Ana Luisa

    2013-12-01

    Neurofibromatosis type 1 (NF1) is an inherited genetic disease affecting 1 in 3,500 individuals. A prominent feature of NF1 is the formation of benign tumours of the peripheral nerve sheath (neurofibromas). However, these can become malignant and form highly metastatic malignant peripheral nerve sheath tumours (MPNST), which are usually fatal despite aggressive surgery, chemotherapy, and radiotherapy. Recent studies have shown that pigment epithelium-derived factor (PEDF) can induce differentiation and inhibit angiogenesis in several kinds of tumours. The present study was designed to determine the in vitro and in vivo effects of PEDF on MPNST angiogenesis and tumour growth. PEDF inhibited proliferation and augmented apoptosis in S462 MPNST cells after 48 h of treatment in culture. In xenografts of S462 MPNST cells in athymic nude mice, PEDF suppressed MPNST tumour burden, due mainly to inhibition of angiogenesis. These results demonstrate for the first time inhibitory effects of PEDF on the growth of human MPNST via induction of anti-angiogenesis and apoptosis. Our results suggest that PEDF could be a novel approach for future therapeutic purposes against MPNST.

  5. Feline peripheral nerve sheath tumors: histologic, immunohistochemical, and clinicopathologic correlation (59 tumors in 53 cats).

    PubMed

    Schulman, F Y; Johnson, T O; Facemire, P R; Fanburg-Smith, J C

    2009-11-01

    Feline peripheral nerve sheath tumors are uncommonly reported, and their clinical behavior has not been well documented. Fifty-nine peripheral nerve sheath tumors were collected from 53 cats. All of the tumors involved skin, subcutis, skeletal muscle, and/or mucous membranes. Histologically, the tumors were composed of compact to loosely arranged streams and fascicles of spindled cells with eosinophilic, often wavy cytoplasmic processes; small to occasionally moderate amounts of collagenous to myxoid matrix; and nuclear palisading. Immunohistochemically, all tumors were positive for vimentin and S-100 protein, 44 of 59 were positive for glial fibrillary acidic protein (GFAP), and all were negative for muscle specific actin. The tumors fell into 3 histologic categories: 34 benign tumors with Antoni A areas that were S-100 protein and GFAP positive, 9 benign tumors that lacked Antoni A areas and were S-100 protein positive and GFAP negative, and 16 tumors with features of malignancy. Seventy-five percent of these cases involved the head, neck, or limbs. Recurrent tumors were submitted or tumors were reported to have recurred in 9 cases. Tumor recurrence was reported for all 3 of the histologic subtypes. None was documented as having metastasized.

  6. Breast metastases from a malignant peripheral nerve sheath tumor of the kidney: An unusual presentation

    PubMed Central

    Koppisetty, Shalini; Alessio, Ricardo C.; Rajpurkar, Atul

    2016-01-01

    Malignant peripheral nerve sheath tumors (MPNSTs) are extremely rare soft tissue sarcomas of ectomesenchymal origin. They are commonly seen in association with neurofibromatosis type 1 (NF-1), but can also occur without a history of NF (isolated MPNST). MPNSTs are most commonly located on the extremities (brachial and sacral plexus), head and neck, and trunk regions and are rarely reported in genitourinary organs. These tumors are aggressive, with a high recurrence rate and distant metastases. MPNST involving the kidney is extremely rare, and review of the literature using PubMed from 2001 to 2014 revealed eight cases of MPNST involving the kidney (seven, primarily involving the kidney and one metastatic MPNST of the kidney). Herein, we describe a case of breast metastases from an MPNST of the kidney without a history of NF-1. The patient was initially diagnosed with a spindle cell neoplasm of the kidney with peripheral nerve sheath differentiation. Eventually, the patient developed a right breast mass that was diagnosed as metastatic MPNST. The patient refused any kind of treatment and died 6 months later in hospice care. PMID:27453670

  7. Diffusion tensor tractography for the surgical management of peripheral nerve sheath tumors.

    PubMed

    Schmidt, Manfred; Kasprian, Gregor; Amann, Gabriele; Duscher, Dominik; Aszmann, Oskar C

    2015-09-01

    OBJECT Peripheral nerve sheath tumors (PNSTs) are uncommon but bear a significant risk of malignancy. High-resolution MRI is the standard technique for characterizing PNSTs. However, planning the appropriate extent of resection and subsequent reconstructive strategies is highly dependent on the intraoperative findings because preoperative MRI evaluation can be insufficient. Diffusion tensor tractography (DTT) represents a recently developed advanced MRI technique that reveals the microstructure of tissues based on monitoring the random movement of water molecules. DTT has the potential to provide diagnostic insights beyond conventional MRI techniques due to its mapping of specific fibrillar nerve structures. Here, DTT was applied to evaluate PNSTs and to examine the usefulness of this method for the correct delineation of tumor and healthy nerve tissue and the value of this information in the preoperative planning of surgical interventions. METHODS In this prospective study, patients with the clinical symptoms of a PNST were investigated using DTT 3-Tesla MRI scans. Image data processing and tractography were performed using the FACT (fiber assessment by continuous tracking) algorithm and multiple-regions-of-interest approach. The surgical findings were then compared with the results of the DTT MRI scans. Preoperative fascicle visualization and the correlation with the intraoperative findings were graded. RESULTS In a 21-month period, 12 patients with PNSTs were investigated (7 female and 5 male patients with a mean age of 46.2 ± 19.2 years). All patients underwent surgical removal of the tumor. Schwannoma was the most common benign histopathological finding (n = 7), whereas 2 malignant lesions were detected. In 10 of 12 patients, good preoperative nerve fascicle visualization was achieved using DTT scans. In 9 of 10 patients with good preoperative fascicle visualization, good intraoperative correlation between the DTT scans and surgical anatomy was found

  8. [Ultrasonographic measurement of the optical nerve sheath for the diagnosis of intracranial hypertension in the emergency room : a case report].

    PubMed

    Levy, R; Kerzmann, B; Franssen, V; Schwab, A S; Adam, J F; Sottiaux, Th

    2016-06-01

    Early diagnosis and treatment of intracranial hypertension (ICHT) are major components of the management of neurological emergencies. The optic nerve sheath diameter is closely dependent on intracranial pressure and can be measured by bedside ultrasound (US). We report the story of a 70-year-old COPD patient initially admitted to the emergency room for a sepsis of pulmonary origin. An unusual confusion prompted us to perform an US of the optic nerve sheath. This exam clearly suggested the presence of an ICHT. Hence, the diagnostic approach was proceeded and a herpetic encephalitis was demonstrated and successfully treated. In this clinical report, the optic nerve sheath US guided the diagnostic approach and, eventually, therapeutic decision. Several papers have shown the close relationship between increased optic nerve sheath diameter and intracranial hypertension, but we still need further studies to validate a threshold value of this diameter. The clinical relevance of the US optic nerve diameter measure appears interesting. However, further studies on larger samples of patients are needed to confirm this and to establish a validated threshold value.

  9. Optic nerve sheath diameter measurements by CT scan in ventriculoperitoneal shunt obstruction.

    PubMed

    Zaidi, Syed Javed H; Yamamoto, Loren G

    2014-08-01

    The objective of the study was to determine differences in optic nerve sheath diameter (ONSD) measurements taken from computed tomography (CT) scans of patients with ventriculoperitoneal shunt (VPS) obstruction versus controls. Inpatients 0-15 years with confirmed VPS obstruction requiring neurosurgical intervention were identified using ICD9 codes. ONSDs, orbit, cranium, and foramen magnum sizes were measured on their pre-surgical CT. Controls included cases at times when their VPS was not obstructed and age and gender matched patients with a CT scan done in the emergency room for head trauma (normal CT findings). Paired T-tests were used for both case-control comparisons. In order to compare the optic nerve sheath size more accurately, the ONSD width was divided by the width of the orbit and by the foramen magnum (antero-posterior) length. Twenty patients were identified with 25 events of VPS obstruction. The right ONSD (RON) was chosen to study. RON/orbit width and RON/foramen magnum diameter for the VPS obstruction versus self-controls, were 0.22 and 0.22, compared to 0.19 and 0.18, respectively, for the non-obstructed self-controls (P = .044 and P = .008, respectively). The same measurements for the VPS obstruction versus age and gender matched controls were 0.22 and 0.21 for the VPS obstruction cases, respectively, compared to 0.17 and 0.16, respectively for the age and gender matched controls (P < .001 and P < .001, respectively). This data confirms that the optic nerve diameter increases during a VPS obstruction. ONSD measurements by ultrasound could add to the evaluation for VPS obstruction.

  10. Malignant nerve-sheath neoplasms in neurofibromatosis: distinction from benign tumors by using imaging techniques

    SciTech Connect

    Levine, E.; Huntrakoon, M.; Wetzel, L.H.

    1987-11-01

    Malignant peripheral nerve-sheath neoplasms frequently complicate neurofibromatosis causing pain, enlarging masses, or neurologic deficits. However, similar findings sometimes also occur with benign nerve neoplasms. Our study was done retrospectively to determine if imaging techniques can differentiate malignant from benign nerve tumors in neurofibromatosis. Eight patients with symptomatic neoplasms (three benign, five malignant) were studied by CT in eight, MR in six, and /sup 67/Ga-citrate scintigraphy in seven. Uptake of /sup 67/Ga occurred in all five malignant lesions but not in two benign neoplasms studied. On CT or MR, all eight lesions, including three benign neoplasms, showed inhomogeneities. Of five lesions with irregular, infiltrative margins on CT or MR, four were malignant and one was benign. Of three lesions with smooth margins, one was malignant and two were benign. One malignant neoplasm caused irregular bone destruction. Accordingly, CT and MR could not generally distinguish malignant from benign lesions with certainty. However, both CT and MR provided structural delineation to help surgical planning for both types of lesion. /sup 67/Ga scintigraphy appears promising as a screening technique to identify lesions with malignant degeneration in patients with neurofibromatosis. Any area of abnormal radiogallium uptake suggests malignancy warranting further evaluation by CT or MR. Biopsy of any questionable lesion is essential.

  11. Magnetic resonance imaging characteristics of peripheral nerve sheath tumors of the canine brachial plexus in 18 dogs.

    PubMed

    Kraft, Susan; Ehrhart, E J; Gall, David; Klopp, Lisa; Gavin, Patrick; Tucker, Russ; Bagley, Rod; Kippenes, Hege; DeHaan, Constance; Pedroia, Vince; Partington, Beth; Olby, Natasha

    2007-01-01

    Magnetic resonance imaging (MRI) examinations from 18 dogs with a histologically confirmed peripheral nerve sheath tumor (PNST) of the brachial plexus were assessed retrospectively. Almost half (8/18) had a diffuse thickening of the brachial plexus nerve(s), six of which extended into the vertebral canal. The other 10/18 dogs had a nodule or mass in the axilla (1.2-338 cm3). Seven of those 10 masses also had diffuse nerve sheath thickening, three of which extended into the vertebral canal. The majority of tumors were hyperintense to muscle on T2-weighted images and isointense on T1-weighted images. Eight of 18 PNSTs had only minimal to mild contrast enhancement and many (13/18) enhanced heterogeneously following gadolinium DTPA administration. Transverse plane images with a large enough field of view (FOV) to include both axillae and the vertebral canal were essential, allowing in-slice comparison to detect lesions by asymmetry of structures. Higher resolution, smaller FOV, multiplanar examination of the cervicothoracic spine was important for appreciating nerve root and foraminal involvement. Short tau inversion recovery, T2-weighted, pre and postcontrast T1-weighted pulse sequences were all useful. Contrast enhancement was critical to detecting subtle diffuse nerve sheath involvement or small isointense nodules, and for accurately identifying the full extent of disease. Some canine brachial plexus tumors can be challenging to detect, requiring a rigorous multiplanar multi-pulse sequence MRI examination.

  12. Malignant peripheral nerve sheath tumor of the third eyelid in a 3-year-old Rhodesian Ridgeback

    PubMed Central

    vom Hagen, Franziska; Romkes, Gwendolyna; Kershaw, Olivia; Eule, J Corinna

    2015-01-01

    Key Clinical Message A 3-year-old Rhodesian Ridgeback was presented with conjunctivitis, enlargement of the third eyelid and a dorsotemporal deviation of the right eye. A mass within the third eyelid was detected and excised. The histopathologic examination showed a malignant peripheral nerve sheath tumor, which most likely is a neurofibrosarcoma based on immunohistochemistry. PMID:25678975

  13. Ultrasound-guided fine needle aspiration in the diagnosis of peripheral nerve sheath tumors in 4 dogs

    PubMed Central

    da Costa, Ronaldo C.; Parent, Joane M.; Dobson, Howard; Ruotsalo, Kristiina; Holmberg, David; Duque, M. Carolina; Poma, Roberto

    2008-01-01

    Ultrasound-guided fine needle aspiration was used in establishing the diagnosis in 4 cases of malignant peripheral nerve sheath tumor. Sonographic and cytologic characteristics are discussed. Because of its availability and ease of use, axillary ultrasonography with fine needle aspiration can be an initial diagnostic step for suspected brachial plexus tumors. PMID:18320983

  14. Convection-Enhanced Delivery (CED) in an Animal Model of Malignant Peripheral Nerve Sheath (MPNST) Tumors and Plexiform Neurofibromas (PN)

    DTIC Science & Technology

    2012-09-01

    convection enhanced delivery in malignant peripheral nerve sheath tumors or plexiform neurofibromas at this time. References Perrin GQ, Fishbein L...2007. 85(6): p. 1347-1357. Perrin GQ, Li H, Fishbein L, et al., An orthotopic xenograft model of intraneural NF1 MPNST suggests a potential

  15. Preoperative simulation of the running course of the abducens nerve in a large petroclival meningioma: a case report and literature review.

    PubMed

    Yang, Kaichuang; Ikawa, Fusao; Onishi, Shumpei; Kolakshyapati, Manish; Takeda, Masaaki; Yamaguchi, Satoshi; Ishifuro, Minoru; Akiyama, Yuji; Morishige, Mizuki; Kurisu, Kaoru

    2017-04-01

    One of the most important and useful pieces of information in the preoperative evaluation of a large petroclival meningioma is the running course of the abducens nerve. The abducens nerve is small and has a long intracranial course, making it prone to compression by the tumor at various anatomical points. In relatively large tumors, it is difficult to confirm the entire course of the abducens nerve, even by heavy T2-thin slice imaging. We report a case of successful preoperative estimation of the course of the abducens nerve that aided in its complete preservation during the resection of a large petroclival tumor.

  16. Primary peripheral nerve sheath tumors of the thyroid gland: A case report and literature review

    PubMed Central

    CHEN, GUANG; LIU, ZENGGUANG; SU, CHANG; GUAN, QIANG; WAN, FANG; DONG, BINGFEI; BAO, LIANG; ZHANG, WENXIN; WANG, YINPING; WANG, GUIMIN

    2016-01-01

    Primary peripheral nerve sheath tumors (PNSTs) of the thyroid gland are rare, with fewer than 30 cases reported in the medical literature to date. Primary PNSTs of the thyroid gland are classified into malignant and benign PNSTs. The benign PNSTs may be further subclassified into neurofibromas and Schwannomas. This is the case report of a 51-year-old male patient presenting with multiple primary PNSTs involving the left lobe of the thyroid gland. The patient underwent total excision of the thyroid gland and the pathological results indicated a Schwannoma with Antoni type A and B cells. The literature was reviewed briefly and, to the best of our knowledge, this is the first case report of multiple primary PNSTs of the thyroid gland. PMID:26893864

  17. A Case of Hypertensive Encephalopathy with Enlarged Optic Nerve Sheath Measured by Transorbital Sonography.

    PubMed

    Kitano, Takaya; Nezu, Tomohisa; Mukai, Tomoya; Uemura, Jyunichi; Wada, Yuko; Yagita, Yoshiki

    2017-01-01

    This case report describes our experience in using transorbital sonography to evaluate pathological changes in the central nervous system in hypertensive encephalopathy. A 49-year-old man with nausea, headache, and mild confusion was diagnosed with hypertensive encephalopathy by brain magnetic resonance imaging (MRI), which revealed vasogenic edema in the bilateral thalamus and the brain stem. Lumbar puncture showed no severe intracranial hypertension. Transorbital sonography showed an increase in the optic nerve sheath diameter (ONSD). Repeated examination revealed a return of the ONSD to an almost normal range after a reduction in blood pressure and a resolution of symptoms. An improvement in cerebral vasogenic edema was confirmed by brain MRI. ONSD might be related to the severity of cerebral vasogenic edema. Repeated measurement of ONSD by transorbital sonography may be useful to assess the pathological course and the effect of treatment in hypertensive encephalopathy. Copyright © 2017 National Stroke Association. Published by Elsevier Inc. All rights reserved.

  18. Primary peripheral nerve sheath tumors of the thyroid gland: A case report and literature review.

    PubMed

    Chen, Guang; Liu, Zengguang; Su, Chang; Guan, Qiang; Wan, Fang; Dong, Bingfei; Bao, Liang; Zhang, Wenxin; Wang, Yinping; Wang, Guimin

    2016-02-01

    Primary peripheral nerve sheath tumors (PNSTs) of the thyroid gland are rare, with fewer than 30 cases reported in the medical literature to date. Primary PNSTs of the thyroid gland are classified into malignant and benign PNSTs. The benign PNSTs may be further subclassified into neurofibromas and Schwannomas. This is the case report of a 51-year-old male patient presenting with multiple primary PNSTs involving the left lobe of the thyroid gland. The patient underwent total excision of the thyroid gland and the pathological results indicated a Schwannoma with Antoni type A and B cells. The literature was reviewed briefly and, to the best of our knowledge, this is the first case report of multiple primary PNSTs of the thyroid gland.

  19. BNIP3 regulates AT101 [(-)-gossypol] induced death in malignant peripheral nerve sheath tumor cells.

    PubMed

    Kaza, Niroop; Kohli, Latika; Graham, Christopher D; Klocke, Barbara J; Carroll, Steven L; Roth, Kevin A

    2014-01-01

    Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive Schwann cell-derived sarcomas and are the leading cause of mortality in patients with neurofibromatosis type 1 (NF1). Current treatment modalities have been largely ineffective, resulting in a high rate of MPNST recurrence and poor five-year patient survival. This necessitates the exploration of alternative chemotherapeutic options for MPNST patients. This study sought to assess the cytotoxic effect of the BH3-mimetic AT101 [(-)-gossypol] on MPNST cells in vitro and to identify key regulators of AT101-induced MPNST cell death. We found that AT101 caused caspase-independent, non-apoptotic MPNST cell death, which was accompanied by autophagy and was mediated through HIF-1α induced expression of the atypical BH3-only protein BNIP3. These effects were mediated by intracellular iron chelation, a previously unreported mechanism of AT101 cytotoxicity.

  20. Synchronous Malignant Peripheral Nerve Sheath Tumor and Adenocarcinoma of the Prostate: Case Report and Literature Review

    PubMed Central

    Bouropoulos, Konstantinos; Farmakis, Antonios

    2016-01-01

    Malignant Peripheral Nerve Sheath Tumors (MPNSTs) of the prostate are extremely rare. A very unusual case of simultaneous adenocarcinoma and MPNST of the prostate is reported. A 60-year-old Caucasian male presented for annual urologic examination. Digital rectal examination revealed a painless, toughish, and asymmetrically enlarged prostate. Serum prostate-specific antigen was 1 ng/mL. Radiologic examinations demonstrated a large mass, which was arising from the left peripheral lobe of the prostate. The patient underwent transrectal ultrasound-guided biopsy of the prostate which revealed a smooth muscle tumor of uncertain malignant potential. Radical retropubic prostatectomy with en bloc removal of the mass and the seminal vesicles was performed and histology demonstrated low-grade MPNST and adenocarcinoma of the prostate. To the best of our knowledge, this is the first report of simultaneous prostatic adenocarcinoma and MPNST in the English literature. PMID:27872787

  1. Malignant Peripheral Nerve Sheath Tumor of Prostate: A Rare Case Report and Literature Review

    PubMed Central

    Lu, Chih-Cheng; Li, Chien-Feng

    2016-01-01

    A mid-aged male presented with progressive lower urinary tract symptoms (LUTS) for years. Huge prostate with low serum prostate-specific antigen (PSA) level was detected. The specimen from transurethral resection revealed surprising pathology finding as malignant peripheral nerve sheath tumor (MPNST). Considering its huge size (more than 300 gm) and location, we prescribed neoadjuvant chemotherapy firstly. The tumor became regressive and then radical surgical resection was achieved. Adjuvant multimodality treatment including concurrent chemoradiotherapy (CCRT) and target therapy was given. However, he expired about one year later. MPNST originating from prostate is very rare and seldom reported before. We here present this extremely rare disease and share our treatment experience. PMID:27872789

  2. Epigenetic mechanisms drive the progression of neurofibromas to malignant peripheral nerve sheath tumors

    PubMed Central

    Suresh, Krish; Kliot, Tamara; Piunti, Andrea; Kliot, Michel

    2016-01-01

    Thinking Outside the Box: The polycomb repressive complex 2 (PRC2) is a histone methyltransferase complex known to repress gene expression. There is a large body of experimental evidence that supports its role in promoting tumorigenicity by suppressing tumor suppressor genes. Here, we discuss the surprising findings that, in neurofibromas, it may have a completely different role as a tumor suppressor; mutations of PRC2 lead to conversion of benign neurofibromas into malignant peripheral nerve sheath tumors (MPNSTs) by de-repressing and thereby activating genes driving cell growth and development. These findings have potentially powerful clinical applications in both diagnosing and treating MPNSTs. Hypothesis: PRC2 loss drives malignant transformation of neurofibromas. PMID:27920939

  3. Optic Nerve Sheath Diameter: Translating a Terrestrial Focused Technique into a Clinical Monitoring Tool for Spaceflight

    NASA Technical Reports Server (NTRS)

    Mason, Sara; Foy, Millennia; Sargsyan, Ashot; Garcia, Kathleen; Wear, Mary L.; Bedi, Deepak; Ernst, Randy; Van Baalen, Mary

    2015-01-01

    Ultrasonography is increasingly used to quickly measure optic nerve sheath diameter (ONSD) when increased intracranial pressure (ICP) is suspected. NASA Space and Clinical Operations Division has been using ground and on-orbit ultrasound since 2009 as a proxy for ICP in non-acute monitoring for space medicine purposes. In the terrestrial emergency room population, an ONSD greater than 0.59 cm is considered highly predictive of elevated intracranial pressure. However, this cut-off limit is not applicable to the spaceflight setting since over 50% of US Operating Segment (USOS) astronauts have an ONSD greater than 0.60 cm even before launch. Crew Surgeon clinical decision-making is complicated by the fact that many astronauts have history of previous spaceflights. Our data characterize the distribution of baseline ONSD in the astronaut corps, its longitudinal trends in long-duration spaceflight, and the predictive power of this measure related to increased ICP outcomes.

  4. A huge malignant peripheral nerve sheath tumor with hepatic metastasis arising from retroperitoneal ganglioneuroma.

    PubMed

    Meng, Z H; Yang, Y S; Cheng, K L; Chen, G Q; Wang, L P; Li, W

    2013-01-01

    Ganglioneuromas (GNs) are the rarest and most benign of the neuroblastic tumors. We experienced a case of huge retroperitoneal GN which differentiated into malignant peripheral nerve sheath tumors (MPNST) with hepatic metastasis. The tumor was located in the upper right quarter of the abdomen and pressed the right lobe of the liver, which was initially misdiagnosed as a liver carcinoma. The tumor shared blood supply with the right liver lob and had rich blood supplies from the abdominal aorta, renal artery and hepatic artery. It was also associated with skin pigment and recurrence shortly following resection. Our finding demonstrated that MPNST is a potent invasive malignant tumor and metastasis earlier with very poor prognosis.

  5. An atypical peripheral nerve sheath tumour with pseudoglandular architecture in a dog.

    PubMed

    Volmer, Christelle; Caplier, Laura; Reyes-Gomez, Edouard; Huet, Hélène; Owen, Roger A; Fontaine, Jean-Jacques

    2010-02-01

    This case describes a subcutaneous soft tissue tumour in a German Shepherd dog. Histologically, the lesion was characterized by proliferating ovoid cells, loosely arranged in a collagenous to myxoid stroma, and by numerous pseudoglandular structures lined by neoplastic cells. Immunohistochemically, neoplastic cells were labelled with vimentin, glial fibrillary acidic protein and S100 antibodies, but not with cytokeratin, desmin and smooth muscle actin antibodies. Ultrastructurally, neoplastic cells were characterized by numerous mitochondria surrounded by endoplasmic reticulum and contained few secondary lysosomes. This tumour was diagnosed as a subcutaneous peripheral nerve sheath tumour (PNST) with pseudoglandular architecture. This case illustrates the morphological diversity of PNST and provides new insight into the differential diagnosis of cutaneous tumours of similar morphology in the dog.

  6. Clinical course of a malignant peripheral nerve sheath tumor in a Siberian tiger (Panthera tigris altaica).

    PubMed

    Steinmetz, Hanspeter W; Rütten, Maja; Ruess-Melzer, Katja; Ohlerth, Stefanie; Lischer, Christoph; Oevermann, Anna; Bode-Lesniewska, Beata; Hatt, Jean-Michel

    2010-11-01

    A 14-year-old male Siberian tiger (Panthera tigris altaica) was admitted with an ulcerating mass on the right thoracic wall. Radiographic and computed tomographic evaluation indicated 2 isolated cutaneous masses without any signs of metastasis. Histology of a Tru-Cut biopsy revealed an anaplastic sarcoma with giant cells. Both tumors were resected with appropriate normal tissue margins. The size of the defect did not allow primary closure of the wound; therefore, a mesh expansion technique was attempted. Three months later, the tiger had to be euthanized due to extensive metastasis to the lungs. Histomorphological features and immunohistochemical results confirmed the diagnosis of malignant peripheral nerve sheath tumor. In contrast to domestic animal experience, the tumor had spread extensively to the lungs without local reccurrence in a short period of time. Correct diagnosis requires various immunohistochemical evaluations of the tumor tissue.

  7. Malignant peripheral nerve sheath tumor presenting as orbito temporal lump: Case report and review of literature

    PubMed Central

    Panigrahi, Souvagya; Mishra, Sudhansu S.; Mishra, Sanjib; Das, Srikant

    2016-01-01

    Malignant peripheral nerve sheath tumor (MPNST) is a rare soft tissue sarcoma. The most common anatomical sites include the upper and lower extremities and trunk and less commonly the head and neck. To our knowledge, few patients with a cranial or facial MPNST have been reported. We report such a lesion in a 35-year-old woman who presented with left sided rapidly progressive proptosis and visual loss due to an orbital lump extending up to the temporal lobe. Cranial imaging showed a huge mass invading the orbital wall and temporal bone. The presumptive diagnosis was a malignant orbital tumor. Preoperative fine needle aspiration cytology of the orbital mass came to be neurofibroma. Near total resection of the tumor was done. Histopathology revealed MPNST which was subsequently confirmed on the basis of immunopositivity for S-100. The patient recovered uneventfully and was discharged 8 days after surgery with an advice to attend cancer institute for possible radiotherapy. PMID:27057226

  8. Multicentric benign peripheral nerve sheath tumors in two related bearded dragons, Pogona vitticeps.

    PubMed

    Lemberger, K Y; Manharth, A; Pessier, A P

    2005-07-01

    Multiple subcutaneous masses from two sibling bearded dragons were removed. Nodules were well demarcated, restricted to the subcutis, and soft, white to yellow, resembling adipose tissue. Histologically, the masses were composed of short interlacing streams and bundles of spindle cells, with regions of vague nuclear palisading. Two of the tumors contained a subpopulation of polygonal cells with abundant periodic acid-Schiff (PAS)-positive cytoplasmic granules. Neoplastic cells were immunohistochemically positive for S100 and neuron-specific enolase (NSE) but negative for desmin and smooth muscle actin. Electron microscopy and reticulin stains demonstrated a continuous basal lamina separating intertwining cells. Histologic, ultrastructural, and immunohistochemical features were consistent with a peripheral nerve sheath origin. At 1 year postexcision, local reoccurrence of a single incompletely excised mass from the left shoulder was noted.

  9. Epineural Sheath Jacket as a New Surgical Technique for Neuroma Prevention in the Rat Sciatic Nerve Model.

    PubMed

    Siemionow, Maria; Bobkiewicz, Adam; Cwykiel, Joanna; Uygur, Safak; Francuzik, Wojciech

    2017-10-01

    Terminal neuromas resulting from severe nerve injuries and traumatic or surgical limb amputations can become a source of pain, and significantly impair patients' quality of life. Recently, the number of patients with peripheral nerve injuries increased due to modern war conflicts, natural disasters, and traffic accidents. This study investigated the efficacy of the epineural sheath jacket (ESJ) as a novel technique for neuroma prevention in the rat sciatic nerve model. A 20-mm segment of the right sciatic nerve was excised in 18 Lewis rats, and the animals were divided into 3 experimental groups (n = 6/group): group I-control, nerve stump without protection; group II-muscle burying group, nerve stump buried in the muscle; group III-ESJ group, nerve stump protected by ESJ. The ESJ was created from the excised sciatic nerve and applied as a "cap" over the proximal nerve stump. The presence of neuropathic pain was assessed weekly by pinprick test and Tinel sign, up to 24 weeks postsurgery. At 24 weeks, assessments, such as macroscopic evaluation, retrograde neuronal labeling analysis, histomorphometry, and neural/connective tissue ratio were performed. Epineural sheath jacket significantly reduced neuroma formation, which was associated with decreased Tinel sign (16.7%, P < 0.05) response compared with the nerve stump control. Moreover, ESJ reduced axonal sprouting, bulb-shaped nerve ending formation and perineural adhesions, as confirmed by macroscopic evaluation. Histological evaluation confirmed that nerve stumps protected with the ESJ showed less fibrosis and presented well-organized axonal structure. Neural/connective tissue ratio and retrograde neuronal labeling analysis revealed significantly improved results in the ESJ group compared to the control nerve stump group (P = 0.032 and P = 0.042, respectively). The protective effect of the ESJ against neuroma formation was confirmed by behavioral and histological analyses, showing outcomes comparable to the muscle

  10. Tamoxifen inhibits malignant peripheral nerve sheath tumor growth in an estrogen receptor–independent manner

    PubMed Central

    Byer, Stephanie J.; Eckert, Jenell M.; Brossier, Nicole M.; Clodfelder-Miller, Buffie J.; Turk, Amy N.; Carroll, Andrew J.; Kappes, John C.; Zinn, Kurt R.; Prasain, Jeevan K.; Carroll, Steven L.

    2011-01-01

    Few therapeutic options are available for malignant peripheral nerve sheath tumors (MPNSTs), the most common malignancy associated with neurofibromatosis type 1 (NF1). Guided by clinical observations suggesting that some NF1-associated nerve sheath tumors are hormonally responsive, we hypothesized that the selective estrogen receptor (ER) modulator tamoxifen would inhibit MPNST tumorigenesis in vitro and in vivo. To test this hypothesis, we examined tamoxifen effects on MPNST cell proliferation and survival, MPNST xenograft growth, and the mechanism by which tamoxifen impeded these processes. We found that 1–5 μM 4-hydroxy-tamoxifen induced MPNST cell death, whereas 0.01–0.1 μM 4-hydroxy-tamoxifen inhibited mitogenesis. Dermal and plexiform neurofibromas, MPNSTs, and MPNST cell lines expressed ERβ and G-protein-coupled ER-1 (GPER); MPNSTs also expressed estrogen biosynthetic enzymes. However, MPNST cells did not secrete 17β-estradiol, exogenous 17β-estradiol did not stimulate mitogenesis or rescue 4-hydroxy-tamoxifen effects on MPNST cells, and the steroidal antiestrogen ICI-182,780 did not mimic tamoxifen effects on MPNST cells. Further, ablation of ERβ and GPER had no effect on MPNST proliferation, survival, or tamoxifen sensitivity, indicating that tamoxifen acts via an ER-independent mechanism. Consistent with this hypothesis, inhibitors of calmodulin (trifluoperazine, W-7), another known tamoxifen target, recapitulated 4-hydroxy-tamoxifen effects on MPNST cells. Tamoxifen was also effective in vivo, demonstrating potent antitumor activity in mice orthotopically xenografted with human MPNST cells. We conclude that 4-hydroxy-tamoxifen inhibits MPNST cell proliferation and survival via an ER-independent mechanism. The in vivo effectiveness of tamoxifen provides a rationale for clinical trials in cases of MPNSTs. PMID:21075781

  11. Initial clinical experience with image-guided linear accelerator-based spinal radiosurgery for treatment of benign nerve sheath tumors.

    PubMed

    Selch, Michael T; Lin, Kevin; Agazaryan, Nzhde; Tenn, Steve; Gorgulho, Alessandra; DeMarco, John J; DeSalles, Antonio A F

    2009-12-01

    Stereotactic radiosurgery has proven a safe and effective treatment of cranial nerve sheath tumors. A similar approach should be successful for histologically identical spinal nerve sheath tumors. The preliminary results of linear accelerator-based spinal radiosurgery were retrospectively reviewed for a group of 25 nerve sheath tumors. Tumor location was cervical 11, lumbar 10, and thoracic 4. Thirteen tumors caused sensory disturbance, 12 pain, and 9 weakness. Tumor size varied from 0.9 to 4.1 cm (median, 2.1 cm). Radiosurgery was performed with a 60-MV linear accelerator equipped with a micro-multileaf collimator. Median peripheral dose and prescription isodose were 12 Gy and 90%, respectively. Image guidance involved optical tracking of infrared reflectors, fusion of amorphous silicon radiographs with dynamically reconstructed digital radiographs, and automatic patient positioning. Follow-up varied from 12 to 58 months (median, 18). There have been no local failures. Tumor size remained stable in 18 cases, and 7 (28%) demonstrated more than 2 mm reduction in tumor size. Of 34 neurologic symptoms, 4 improved. There has been no clinical or imaging evidence for spinal cord injury. One patient had transient increase in pain and one transient increase in numbness. Results of this limited experience indicate linear accelerator-based spinal radiosurgery is feasible for treatment of benign nerve sheath tumors. Further follow-up is necessary, but our results imply spinal radiosurgery may represent a therapeutic alternative to surgery for nerve sheath tumors. Symptom resolution may require a prescribed dose of more than 12 Gy. Copyright 2009 Elsevier Inc. All rights reserved.

  12. Effects of acute controlled changes in end-tidal carbon dioxide on the diameter of the optic nerve sheath: a transorbital ultrasonographic study in healthy volunteers.

    PubMed

    Dinsmore, M; Han, J S; Fisher, J A; Chan, V W S; Venkatraghavan, L

    2017-02-08

    Transorbital ultrasonographic measurement of the diameter of the optic nerve sheath is a non-invasive, bed-side examination for detecting raised intracranial pressure. However, the ability of the optic nerve sheath diameter to predict acute changes in intracranial pressures remains unknown. The aim of this study was to examine the dynamic changes of the optic nerve sheath diameter in response to mild fluctuations in cerebral blood volume induced by changes in end-tidal carbon dioxide. We studied 11 healthy volunteers. End-tidal carbon dioxide was controlled by a model-based prospective end-tidal targeting system (RespirAct™). The volunteers' end-tidal carbon dioxide was targeted and maintained for 10 min each at normocapnia (baseline); hypercapnia (6.5 kPa); normocapnia (baseline 1); hypocapnia (3.9 kPa) and on return to normocapnia (baseline 2). A single investigator repeatedly measured the optic nerve sheath diameter for 10 min at each level of carbon dioxide. With hypercapnia, there was a significant increase in optic nerve sheath diameter, with a mean (SD) increase from baseline 4.2 (0.7) mm to 4.8 (0.8) mm; p < 0.001. On return to normocapnia, the optic nerve sheath diameter rapidly reverted back to baseline values. This study confirms dynamic changes in the optic nerve sheath diameter with corresponding changes in carbon dioxide, and their reversibly with normocapnia.

  13. Malignant peripheral nerve sheath tumour (MPNST): the clinical implications of cellular signalling pathways.

    PubMed

    Katz, Daniela; Lazar, Alexander; Lev, Dina

    2009-10-19

    Malignant peripheral nerve sheath tumour (MPNST) is a rare malignancy accounting for 3-10% of all soft tissue sarcomas. Most MPNSTs arise in association with peripheral nerves or deep neurofibromas and may originate from neural crest cells, although the specific cell of origin is uncertain. Approximately half of MPNSTs occur in the setting of neurofibromatosis type 1 (NF1), an autosomal dominant disorder with an incidence of approximately one in 3500 persons; the remainder of MPNSTs develop sporadically. In addition to a variety of clinical manifestations, approximately 8-13% of NF1 patients develop MPNSTs, which are the leading cause of NF1-related mortality. Surgical resection is the mainstay of MPNST clinical management. However, because of invasive growth, propensity to metastasise, and limited sensitivity to chemotherapy and radiation, MPNST has a guarded to poor prognosis. Five-year survival rates of only 20-50% indicate an urgent need for improved therapeutic approaches. Recent work in this field has identified several altered intracellular signal transduction cascades and deregulated tyrosine kinase receptors, posing the possibility of personalised, targeted therapeutics. However, expanded knowledge of MPNST molecular pathobiology will be needed to meaningfully apply such approaches for the benefit of afflicted patients.

  14. Medullary metastasis of a malignant peripheral nerve sheath tumor: A case report

    PubMed Central

    Hagi, Tomohito; Nakamura, Tomoki; Yokoji, Ayumu; Matsumine, Akihiko; Sudo, Akihiro

    2016-01-01

    The present study reports a case of medullary metastasis without lung metastasis that occurred as a result of a malignant peripheral nerve sheath tumor (MPNST). An 81-year-old woman presented with a MPNST in the left brachial plexus, arising from the cervical nerve root. The patient underwent carbon ion radiotherapy; however, tumor recurrence was identified in the left shoulder. Subsequently, the patient underwent wide excision. Three weeks subsequent to surgery, imbalance and dysarthria developed suddenly. Dysphagia emerged and left upper limb pain disappeared on the day after symptom development. Magnetic resonance imaging (MRI) revealed that this was due to metastasis to the medulla. Five days subsequent to the onset of dysarthria, the patient succumbed due to respiratory failure. To the best of our knowledge, no previous cases of medullary metastasis arising from a MPNST in the absence of lung metastasis have been reported. MRI is a useful examination tool for the identification of brain metastases; however, the high cost of MRI as a routine examination must be considered due to the rarity of brain metastases. Therefore, methods to detect brain metastasis warrant further investigation. PMID:27588138

  15. Superficial or cutaneous malignant peripheral nerve sheath tumor--clinical experience at Taipei Veterans General Hospital.

    PubMed

    Feng, Chin-Jung; Ma, Hsu; Liao, Wen-Chieh

    2015-05-01

    Primary malignant peripheral nerve sheath tumors (MPNSTs) with a cutaneous or subcutaneous origin represent a small subset of MPNSTs thought to be derived from cutaneous neurofibromas or small peripheral nerves. Few cases of superficial MPNSTs originating from the skin have been reported in the literature. From October 1999 to February 2014, 13 patients were diagnosed with superficial or cutaneous MPNSTs and received treatment at Taipei Veterans General Hospital. Clinical data were collected via retrospective chart review. A retrospective study was performed to compare superficial and deep-seated lesions in terms of local recurrence, distal metastasis, and survival analysis. The relevant literature is also briefly reviewed. The most frequent initial symptoms were local swelling and pain. Ten tumors were found in the extremities, and 3 tumors were located on the trunk. All patients underwent surgery with curative intent. Four patients developed local recurrence, and 3 developed distant metastasis. Three of 13 patients died after a follow-up period of 11 to 180 months (mean, 53.4). Compared to deep-seated MPNSTs, superficial MPNSTs had a lower histopathological grading and better survival rate. Superficial MPNSTs are a rare variant of MPNST. The relatively frequent lack of associated neurofibromatosis and superficial location within the dermis and subcutis may result in this entity being overlooked. According to our clinical experience, superficial MPNSTs might have better prognosis, but similar recurrence and metastasis rates compared with deep-seated lesions. Hence, awareness of this entity should prompt its consideration in the differential diagnosis of cutaneous sarcomas.

  16. Optic nerve sheath distention in Leber's optic neuropathy and the significance of the "Wallace mutation".

    PubMed

    Smith, J L; Tse, D T; Byrne, S F; Johns, D R; Stone, E M

    1990-12-01

    We recently encountered a 27-year-old man who presented an atypical clinical picture of Leber's hereditary optic neuropathy: His family history was negative, visual loss continued steadily for over 8 months, circumpapillary microangiopathy was equivocal, the optic discs showed large physiologic cups, and both optic nerve sheaths were notably distended with increased subarachnoid fluid. The latter was confirmed by ultrasonography, computed tomography, and magnetic resonance imaging. The patient's asymptomatic brother also showed unimpressive circumpapillary microangiopathy in the fundi. The asymptomatic mother from France was then seen, and she showed classic circumpapillary microangiopathy in the fundi. Studies of mitochondrial DNA showed the classic point mutation at position 11778 as reported by Wallace in all three family members. Another patient previously seen with classic Leber's hereditary optic neuropathy recently had mitochondrial DNA studies along with three other affected family members and five unaffected family members in the maternal lineage. All nine of these individuals were completely normal at the Wallace locus. In fact, sequencing of the entire ND-4 gene from one affected individual revealed it to be perfectly normal at the amino acid level. The importance of obtaining quantitative ultrasonography and the 30 degrees test, and studying mitochondrial DNA in patients suspected of having Leber's optic nerve disease is emphasized.

  17. A distal ileum malignant peripheral nerve sheath tumor causing intussusception in a patient in China: a case report.

    PubMed

    Zhu, Lin-Bo; Li, Peng-Fei; Xiao, Wei-Hua; Zhang, Peng-Bin; Li, Jun-Qiang; Sun, Ming-Fei

    2017-01-19

    Malignant peripheral nerve sheath tumors (MPNSTs) arise from a peripheral nerve or display nerve sheath differentiation. Most MPNSTs typically originate on the trunk, extremities, head, neck, and paravertebral regions. Gastrointestinal MPNSTs are rare entities with only 10 cases reported worldwide in the literatures. Here, we report the first Chinese case of a malignant peripheral nerve sheath tumor of the distal ileum presenting as intussusception. A 53-year-old female patient without pathological antecedent for neurofibromatosis was admitted with pain in the right lower abdomen and multiple episodes of vomiting for 1 week. Preoperative diagnosis was intussusception with a contrast-enhanced computed tomography scan (CECT) of the abdomen showing characteristic target sign. Due to difficulty reducing the ileum-colon intussusception, right hemicolectomy with ileocolostomy was performed. Histopathology was suggestive of low-grade MPNST. The patient received postoperative care and was followed up for 9 months. There is no sign of tumor recurrence and metastatic disease. This case is unique in terms of a rare tumor presenting with unusual complication.

  18. Angiofollicular lymph node hyperplasia resembling a spinal nerve sheath tumor: a rare case of Castleman's disease.

    PubMed

    Stevens, E Andrew; Strowd, Roy E; Mott, Ryan T; Oaks, Timothy E; Wilson, John A

    2009-09-01

    Angiofollicular lymph node hyperplasia (Castleman's disease) is a lymphoproliferative disorder of unknown etiology. Although uncommon, the localized form of this disease can manifest in the central nervous system, typically as a meningeal-based intracranial lesion. Castleman's disease involving the spine is exceedingly rare. This represents only the second reported case of a patient with Castleman's disease whose presentation mimicked that of a spinal nerve sheath tumor. We report a rare case of angiofollicular lymph node hyperplasia that mimicked a spinal nerve sheath tumor and was treated with gross total resection. Case report. A 31-year-old female with angiofollicular lymph node hyperplasia presenting with a paraspinal mass. The patient's outcome was based on clinical history, physical and radiographic examinations. A 31-year-old woman with a mediastinal mass previously diagnosed as sarcoid via biopsy presented with the new onset of radicular pain and radiographic enlargement of her mass. Magnetic resonance imaging demonstrated a 4.3x5.7x6.0 cm homogenously enhancing soft-tissue lesion that emanated from and widened the T3/T4 neural foramen on the left. The differential diagnosis based on the location of the lesion and imaging characteristics included schwannoma, neurofibroma, paraganglioma, sarcoid, and lymphoma. Gross total resection was performed via thoracotomy. Histological examination identified angiofollicular lymph node hyperplasia (Castleman's disease) of the hyaline-vascular subtype. The patient did not receive adjuvant chemotherapy or radiation therapy. The patient had resolution of her symptoms without any clinical or radiographic evidence of recurrence at 1-year follow-up using magnetic resonance imaging with and without contrast. Castleman's disease is a rare pathologic entity that should be considered in the differential diagnosis of a patient with a paraspinal mass. Spine surgeons should be aware of this diagnosis as it has treatment and follow

  19. Malignant peripheral nerve sheath tumor of adrenal gland with heterologus osseous differentiation in a case of Von Recklinghausen's disease.

    PubMed

    Baisakh, Manas R; Mohapatra, Nachiketa; Adhikary, Samiran D; Routray, Debasis

    2014-01-01

    Malignant peripheral nerve sheath tumor (MPNST) of the adrenal gland is extremely rare. Most of them occur in association with neurofibromatosis, ganglioneuroma or as part of a composite tumor such as pheochromocytoma. Only seven cases of MPNST of the adrenal gland have been reported in the literature till date. Discriminating this entity from other soft tissue sarcomas and gastrointestinal stromal tumor of the adrenal gland has important diagnostic and therapeutic implications. Moreover, the tumor size and pattern of expression for certain immunohistochemical markers may serve as independent predictors of aggressiveness. Herein we present a 24-years-old male with features of Von Recklinghausen's disease who presented with large left adrenal gland malignant peripheral nerve sheath tumor.

  20. Ectopic orbital meningioma: report of two cases and literature review.

    PubMed

    Gündüz, Kaan; Kurt, Rengin Aslıhan; Erden, Esra

    2014-01-01

    Ectopic orbital meningioma is a rare tumor usually affecting the medial orbit. We present two cases that occurred in a 56-year-old woman and a 28-year-old man. The tumors in both patients were subtotally excised via orbitotomy surgery and were located in the superior quadrant in one of our patients and in the temporal quadrant in the other. Following histopathologic diagnosis, external beam radiotherapy (EBRT) was administered to one patient and intensity modulated radiotherapy to the other. We identified 12 other well-documented cases of ectopic orbital meningioma previously reported. Ectopic meningioma should be considered in the differential diagnosis of medial as well as lateral and superior orbital tumors. The tumor is usually well circumscribed but can be ill defined in imaging studies. There are intralesional calcifications and sclerosis of adjacent bone in some cases. Ectopic orbital meningioma can recur after incomplete excision. Based on the efficacy of EBRT in optic nerve sheath meningioma, we used this treatment to decrease the risk of recurrence in our two patients and found no tumor recurrence at follow-ups of 24 and 74 months, but one patient had severe vision loss from radiation retinopathy. Copyright © 2014 Elsevier Inc. All rights reserved.

  1. Schwannoma of the tibial nerve sheath as a cause of tarsal tunnel syndrome--a case study.

    PubMed

    Milnes, Helen L; Pavier, Julian C

    2012-09-01

    A schwannoma is a benign, encapsulated and non-invasive tumour whose origin is derived from Schwann cells. Its incidence in the foot and ankle is rare. The etiology, incidence, clinical presentation, histology, differential diagnoses and surgical treatment are discussed. The authors present a case of a schwannoma of the tibial nerve sheath causing tarsal tunnel syndrome of the foot. The diagnosis in this case was delayed for eight years, which unfortunately is not uncommon with pedal schwannomas.

  2. Trp53 Haploinsufficiency Modifies EGFR-Driven Peripheral Nerve Sheath Tumorigenesis

    PubMed Central

    Rahrmann, Eric P.; Moriarity, Branden S.; Otto, George M.; Watson, Adrienne L.; Choi, Kwangmin; Collins, Margaret H.; Wallace, Margaret; Webber, Beau R.; Forster, Colleen L.; Rizzardi, Anthony E.; Schmechel, Stephen C.; Ratner, Nancy; Largaespada, David A.

    2015-01-01

    Malignant peripheral nerve sheath tumors (MPNSTs) are genetically diverse, aggressive sarcomas that occur sporadically or in association with neurofibromatosis type 1 syndrome. Reduced TP53 gene expression and amplification/overexpression of the epidermal growth factor receptor (EGFR) gene occur in MPNST formation. We focused on determining the cooperativity between reduced TP53 expression and EGFR overexpression for Schwann cell transformation in vitro (immortalized human Schwann cells) and MPNST formation in vivo (transgenic mice). Human gene copy number alteration data, microarray expression data, and TMA analysis indicate that TP53 haploinsufficiency and increased EGFR expression co-occur in human MPNST samples. Concurrent modulation of EGFR and TP53 expression in HSC1λ cells significantly increased proliferation and anchorage-independent growth in vitro. Transgenic mice heterozygous for a Trp53-null allele and overexpressing EGFR in Schwann cells had a significant increase in neurofibroma and grade 3 PNST (MPNST) formation compared with single transgenic controls. Histological analysis of tumors identified a significant increase in pAkt expression in grade 3 PNSTs compared with neurofibromas. Array comparative genome hybridization analysis of grade 3 PNSTs identified recurrent focal regions of chromosomal gains with significant enrichment in genes involved in extracellular signal–regulated kinase 5 signaling. Collectively, altered p53 expression cooperates with overexpression of EGFR in Schwann cells to enhance in vitro oncogenic properties and tumorigenesis and progression in vivo. PMID:24832557

  3. Malignant peripheral nerve sheath tumor as a cause of chronic cardiac insufficiency in cattle

    PubMed Central

    2013-01-01

    Chronic cardiac insufficiency was associated with a malignant peripheral nerve sheath tumor in a cow. An eight-year-old cow developed a progressive condition (over a period of three months) characterized by an enhanced abdominal volume, reluctance to move, a positive jugular pulse, watery diarrhea and death. At necropsy, moderate subcutaneous edema and an enhanced hepatic lobular pattern were observed. A 23x20x11 cm firm, grayish-white mass adhered to and infiltrated the right atrium. Multiple firm, yellowish-white nodules of 0.5 to 12 cm in diameter were diffusely scattered in the epicardium and parietal pericardium. Histologically, the tumor was poorly circumscribed with foci of infiltration of the myocardium. The neoplastic cells had two major histologic patterns, Antoni types A and B. Within occasional foci, pleomorphic cells with an epithelioid appearance were present in addition to multinucleated cells with periodic acid Schiff (PAS)-positive cytoplasmic globules. Foci of cartilaginous and granular differentiations were interspersed among the neoplastic cells. Multiple vessels presented wall hyalinization and tumoral embolus. Large necrotic foci with mineralization and cholesterol clefts were also observed. Immunohistochemically, the tumor was positive for S100 protein, vimentin and neuron-specific enolase labeling. PMID:23369465

  4. Fatty acid synthase is a metabolic oncogene targetable in malignant peripheral nerve sheath tumors

    PubMed Central

    Patel, Ami V.; Johansson, Gunnar; Colbert, Melissa C.; Dasgupta, Biplab; Ratner, Nancy

    2015-01-01

    Background Malignant peripheral nerve sheath tumors (MPNSTs) are soft tissue sarcomas with minimal therapeutic opportunities. We observed that lipid droplets (LDs) accumulate in human MPNST cell lines and in primary human tumor samples. The goal of this study was to investigate the relevance of lipid metabolism to MPNST survival and as a possible therapeutic target. Methods Based on preliminary findings that MPNSTs accumulate LDs, we hypothesized that a deregulated lipid metabolism supports MPNST cell survival/proliferation rate. To test this, we examined respiration, role of fatty acid oxidation (FAO), and the enzyme fatty acid synthase involved in de novo fatty acid synthesis in MPNSTs using both genetic and pharmacological tools. Results We demonstrate that LDs accumulate in MPNST cell lines, primary human and mouse MPNST tumors, and neural crest cells. LDs from MPNST cells disappear on lipid deprivation, indicating that LDs can be oxidized as a source of energy. Inhibition of FAO decreased oxygen consumption and reduced MPNST survival, indicating that MPNST cells likely metabolize LDs through active FAO. FAO inhibition reduced oxygen consumption and survival even in the absence of exogenous lipids, indicating that lipids synthesized de novo can also be oxidized. Consequently, inhibition of de novo fatty acid synthesis, which is overexpressed in human MPNST cell lines, effectively reduced MPNST survival and delayed induction of tumor growth in vivo. Conclusion Our results show that MPNSTs depend on lipid metabolic pathways and suggest that disrupting lipid metabolism could be a potential new strategy for the development of MPNST therapeutics. PMID:26116612

  5. Malignant peripheral nerve sheath tumor with and without neurofibromatosis type 1.

    PubMed

    Vasconcelos, Roberto André Torres de; Coscarelli, Pedro Guimarães; Alvarenga, Regina Papais; Acioly, Marcus André

    2017-06-01

    In this study, we review the institution's experience in treating malignant peripheral nerve sheath tumors (MPNSTs). A secondary aim was to compare outcomes between MPNSTs with and without neurofibromatosis type 1 (NF1). Ninety-two patients with MPNSTs, over a period of 20 years, were reviewed. A retrospective chart review was performed. The median age was 43.5 years (range, 3-84 years) and 55.4% were female; 41 patients (44.6%) had NF1-associated tumors. Mean tumor sizes were 15.8 ± 8.2 cm and 10.8 ± 6.3 cm for patients with and without NF1, respectively. Combined two- and five-year overall survival was 48.5% and 29%. Multivariate analysis confirmed the association of tumor size greater than 10 cm (hazard ratio (HR) 2.99; 95% confidence interval (CI) 1.14-7.85; p = 0.0258) and presence of NF1 (HR 3.41; 95%CI 1.88-6.19; p < 0.001) with a decreased overall survival. Tumor size and NF1 status were the most important predictors of overall survival in our population.

  6. Survivin expression and prognostic significance in pediatric malignant peripheral nerve sheath tumors (MPNST).

    PubMed

    Alaggio, Rita; Turrini, Riccardo; Boldrin, Daniela; Merlo, Anna; Gambini, Claudio; Ferrari, Andrea; Dall'igna, Patrizia; Coffin, Cheryl M; Martines, Annalisa; Bonaldi, Laura; De Salvo, Gian Luca; Zanovello, Paola; Rosato, Antonio

    2013-01-01

    Malignant peripheral nerve sheath tumors (MPNST) are very aggressive malignancies comprising approximately 5-10% of all soft tissue sarcomas. In this study, we focused on pediatric MPNST arising in the first 2 decades of life, as they represent one the most frequent non-rhabdomyosarcomatous soft tissue sarcomas in children. In MPNST, several genetic alterations affect the chromosomal region 17q encompassing the BIRC5/SURVIVIN gene. As cancer-specific expression of survivin has been found to be an effective marker for cancer detection and outcome prediction, we analyzed survivin expression in 35 tumor samples derived from young patients affected by sporadic and neurofibromatosis type 1-associated MPNST. Survivin mRNA and protein expression were assessed by Real-Time PCR and immunohistochemical staining, respectively, while gene amplification was analyzed by FISH. Data were correlated with the clinicopathological characteristics of patients. Survivin mRNA was overexpressed in pediatric MPNST and associated to a copy number gain of BIRC5; furthermore, increased levels of transcripts correlated with a higher FNCLCC tumor grade (grade 1 and 2 vs. 3, p = 0.0067), and with a lower survival probability (Log-rank test, p = 0.0038). Overall, these data support the concept that survivin can be regarded as a useful prognostic marker for pediatric MPNST and a promising target for therapeutic interventions.

  7. Poly (ADP) ribose polymerase inhibition: A potential treatment of malignant peripheral nerve sheath tumor.

    PubMed

    Kivlin, Christine M; Watson, Kelsey L; Al Sannaa, Ghadah A; Belousov, Roman; Ingram, Davis R; Huang, Kai-Lieh; May, Caitlin D; Bolshakov, Svetlana; Landers, Sharon M; Kalam, Azad Abul; Slopis, John M; McCutcheon, Ian E; Pollock, Raphael E; Lev, Dina; Lazar, Alexander J; Torres, Keila E

    2016-01-01

    Poly (ADP) ribose polymerase (PARP) inhibitors, first evaluated nearly a decade ago, are primarily used in malignancies with known defects in DNA repair genes, such as alterations in breast cancer, early onset 1/2 (BRCA1/2). While no specific mutations in BRCA1/2 have been reported in malignant peripheral nerve sheath tumors (MPNSTs), MPNST cells could be effectively targeted with a PARP inhibitor to drive cells to synthetic lethality due to their complex karyotype and high level of inherent genomic instability. In this study, we assessed the expression levels of PARP1 and PARP2 in MPNST patient tumor samples and correlated these findings with overall survival. We also determined the level of PARP activity in MPNST cell lines. In addition, we evaluated the efficacy of the PARP inhibitor AZD2281 (Olaparib) in MPNST cell lines. We observed decreased MPNST cell proliferation and enhanced apoptosis in vitro at doses similar to, or less than, the doses used in cell lines with established defective DNA repair genes. Furthermore, AZD2281 significantly reduced local growth of MPNST xenografts, decreased the development of macroscopic lung metastases, and increased survival of mice with metastatic disease. Our results suggest that AZD2281 could be an effective therapeutic option in MPNST and should be further investigated for its potential clinical use in this malignancy.

  8. Appropriate modulation of autophagy sensitizes malignant peripheral nerve sheath tumor cells to treatment with imatinib mesylate.

    PubMed

    Okano, Munehiro; Sakata, Naoki; Ueda, Satoshi; Takemura, Tsukasa

    2014-04-01

    Malignant peripheral nerve sheath tumor (MPNST), very rare in childhood, is a highly aggressive soft-tissue tumor. We experienced a case of a 7-year-old boy with MPNST who was treated with imatinib mesylate (imatinib) after the identification of platelet-derived growth factor receptor expression in his tumor. We were unable to observe clinical benefits of imatinib in this patient. Therefore, cellular reactions of imatinib were investigated in vitro using 3 MPNST cell lines. Imatinib induced cytotoxicity in vitro with variable IC50 values (11.7 to >30 μM). Induction of apoptosis was not a pivotal mechanism in the inhibitory effects. We found that the treatment of MPNST cell lines with imatinib induced autophagy. Suppression of the initiation of autophagy by 3-methyladenine or small interfering RNA (siRNA) against beclin-1 attenuated the imatinib-mediated cytotoxicity. In contrast, blocking the formation of autophagosomes or the development of autolysosomes using siRNA against microtubule-associated protein light chain 3B, bafilomycin A1, chloroquine, or an MEK1/2 inhibitor (U0126) enhanced the imatinib-induced cytotoxicity in MPNST cells. Our data showed that the imatinib-mediated autophagy can function as a cytotoxic mechanism and that appropriate modulation of autophagy may sensitize MPNST cells to imatinib, which in turn may be a novel therapeutic strategy for MPNST.

  9. Transorbital sonographic evaluation of normal optic nerve sheath diameter in healthy volunteers in Bangladesh.

    PubMed

    Maude, Rapeephan R; Hossain, Md Amir; Hassan, Mahtab Uddin; Osbourne, Sophie; Sayeed, Katherine Langan Abu; Karim, Mohammed Rezaul; Samad, Rasheda; Borooah, Shyamanga; Dhillon, Bal; Day, Nicholas P J; Dondorp, Arjen M; Maude, Richard J

    2013-01-01

    Measurement of optic nerve sheath diameter (ONSD) by ultrasound is increasingly used as a marker to detect raised intracranial pressure (ICP). ONSD varies with age and there is no clear consensus between studies for an upper limit of normal. Knowledge of normal ONSD in a healthy population is essential to interpret this measurement. In a prospective observational study, ONSD was measured using a 15 MHz ultrasound probe in healthy volunteers in Chittagong, Bangladesh. The aims were to determine the normal range of ONSD in healthy Bangladeshi adults and children, compare measurements in males and females, horizontal and vertical beam orientations and left and right eyes in the same individual and to determine whether ONSD varies with head circumference independent of age. 136 subjects were enrolled, 12.5% of whom were age 16 or under. Median ONSD was 4.41 mm with 95% of subjects in the range 4.25-4.75 mm. ONSD was bimodally distributed. There was no relationship between ONSD and age (≥4 years), gender, head circumference, and no difference in left vs right eye or horizontal vs vertical beam. Ultrasonographic ONSD in Bangladeshi healthy volunteers has a narrow bimodal distribution independent of age (≥4 years), gender and head circumference. ONSD >4.75 mm in this population should be considered abnormal.

  10. Transorbital Sonographic Evaluation of Normal Optic Nerve Sheath Diameter in Healthy Volunteers in Bangladesh

    PubMed Central

    Maude, Rapeephan R.; Amir Hossain, Md; Hassan, Mahtab Uddin; Osbourne, Sophie; Sayeed, Katherine Langan Abu; Karim, Mohammed Rezaul; Samad, Rasheda; Borooah, Shyamanga; Dhillon, Bal; Day, Nicholas P. J.; Dondorp, Arjen M.; Maude, Richard J.

    2013-01-01

    Introduction Measurement of optic nerve sheath diameter (ONSD) by ultrasound is increasingly used as a marker to detect raised intracranial pressure (ICP). ONSD varies with age and there is no clear consensus between studies for an upper limit of normal. Knowledge of normal ONSD in a healthy population is essential to interpret this measurement. Methods In a prospective observational study, ONSD was measured using a 15 MHz ultrasound probe in healthy volunteers in Chittagong, Bangladesh. The aims were to determine the normal range of ONSD in healthy Bangladeshi adults and children, compare measurements in males and females, horizontal and vertical beam orientations and left and right eyes in the same individual and to determine whether ONSD varies with head circumference independent of age. Results 136 subjects were enrolled, 12.5% of whom were age 16 or under. Median ONSD was 4.41 mm with 95% of subjects in the range 4.25–4.75 mm. ONSD was bimodally distributed. There was no relationship between ONSD and age (≥4 years), gender, head circumference, and no difference in left vs right eye or horizontal vs vertical beam. Conclusions Ultrasonographic ONSD in Bangladeshi healthy volunteers has a narrow bimodal distribution independent of age (≥4 years), gender and head circumference. ONSD >4.75 mm in this population should be considered abnormal. PMID:24312515

  11. Survivin Expression and Prognostic Significance in Pediatric Malignant Peripheral Nerve Sheath Tumors (MPNST)

    PubMed Central

    Boldrin, Daniela; Merlo, Anna; Gambini, Claudio; Ferrari, Andrea; Dall'Igna, Patrizia; Coffin, Cheryl M.; Martines, Annalisa; Bonaldi, Laura; De Salvo, Gian Luca; Zanovello, Paola; Rosato, Antonio

    2013-01-01

    Malignant peripheral nerve sheath tumors (MPNST) are very aggressive malignancies comprising approximately 5–10% of all soft tissue sarcomas. In this study, we focused on pediatric MPNST arising in the first 2 decades of life, as they represent one the most frequent non-rhabdomyosarcomatous soft tissue sarcomas in children. In MPNST, several genetic alterations affect the chromosomal region 17q encompassing the BIRC5/SURVIVIN gene. As cancer-specific expression of survivin has been found to be an effective marker for cancer detection and outcome prediction, we analyzed survivin expression in 35 tumor samples derived from young patients affected by sporadic and neurofibromatosis type 1-associated MPNST. Survivin mRNA and protein expression were assessed by Real-Time PCR and immunohistochemical staining, respectively, while gene amplification was analyzed by FISH. Data were correlated with the clinicopathological characteristics of patients. Survivin mRNA was overexpressed in pediatric MPNST and associated to a copy number gain of BIRC5; furthermore, increased levels of transcripts correlated with a higher FNCLCC tumor grade (grade 1 and 2 vs. 3, p = 0.0067), and with a lower survival probability (Log-rank test, p = 0.0038). Overall, these data support the concept that survivin can be regarded as a useful prognostic marker for pediatric MPNST and a promising target for therapeutic interventions. PMID:24303016

  12. Poly (ADP) ribose polymerase inhibition: A potential treatment of malignant peripheral nerve sheath tumor

    PubMed Central

    Kivlin, Christine M.; Watson, Kelsey L.; Al Sannaa, Ghadah A.; Belousov, Roman; Ingram, Davis R.; Huang, Kai-Lieh; May, Caitlin D.; Bolshakov, Svetlana; Landers, Sharon M.; Kalam, Azad Abul; Slopis, John M.; McCutcheon, Ian E.; Pollock, Raphael E.; Lev, Dina; Lazar, Alexander J.; Torres, Keila E.

    2016-01-01

    ABSTRACT Poly (ADP) ribose polymerase (PARP) inhibitors, first evaluated nearly a decade ago, are primarily used in malignancies with known defects in DNA repair genes, such as alterations in breast cancer, early onset 1/2 (BRCA1/2). While no specific mutations in BRCA1/2 have been reported in malignant peripheral nerve sheath tumors (MPNSTs), MPNST cells could be effectively targeted with a PARP inhibitor to drive cells to synthetic lethality due to their complex karyotype and high level of inherent genomic instability. In this study, we assessed the expression levels of PARP1 and PARP2 in MPNST patient tumor samples and correlated these findings with overall survival. We also determined the level of PARP activity in MPNST cell lines. In addition, we evaluated the efficacy of the PARP inhibitor AZD2281 (Olaparib) in MPNST cell lines. We observed decreased MPNST cell proliferation and enhanced apoptosis in vitro at doses similar to, or less than, the doses used in cell lines with established defective DNA repair genes. Furthermore, AZD2281 significantly reduced local growth of MPNST xenografts, decreased the development of macroscopic lung metastases, and increased survival of mice with metastatic disease. Our results suggest that AZD2281 could be an effective therapeutic option in MPNST and should be further investigated for its potential clinical use in this malignancy. PMID:26650448

  13. Malignant peripheral nerve sheath tumor of the uterine corpus presenting as a huge abdominal neoplasm.

    PubMed

    Sengar Hajari, Anju Rani; Tilve, Arundhati G; Kulkarni, Jagdeesh N; Bharat, Rekhi

    2015-01-01

    A 45-year-old P3L3, referred to us with abdominopelvic mass for further management. Vaginal examination was suggestive of uterine mass. Magnetic resonance imaging. (MRI) of abdomen.pelvis disclosed a uterine mass with equivocal invasion of the fat plane with the sigmoid colon. Coelomic antigen. (CA) 125 was 120.2 U/ml. (normal range, 0-35 U/ml). On exploratory laparotomy entire pelvic cavity was filled with a mass that was seen arising from the uterus and involving the sigmoid colon. Hence, a total abdominal hysterectomy with bilateral salpingo-oopphorectomy. (TAH BSO) was performed, along with resection anastomosis of the rectosigmoid and excision of omental and pelvic peritoneal nodules. Histopathology and immunohistochemical analysis, including S100-P positivity confirmed diagnosis of a malignant peripheral nerve sheath tumor. (MPNST), with tumor deposits in the right parametrium, omentum, sigmoid colon, and pelvic peritoneum. This case is presented in view of its rarity and associated diagnostic and therapeutic implications.

  14. Histopathology and clinical outcome of NF1-associated vs. sporadic malignant peripheral nerve sheath tumors.

    PubMed

    Hagel, Christian; Zils, Ulrich; Peiper, Matthias; Kluwe, Lan; Gotthard, Stefan; Friedrich, Reinhard E; Zurakowski, David; von Deimling, Andreas; Mautner, Victor Felix

    2007-04-01

    The differences in the clinical course and histopathology of sporadic and neurofibromatosis type 1 (NF1)-associated malignant peripheral nerve sheath tumors (MPNST) were investigated retrospectively. The collective comprised 38 NF1 patients and 14 sporadic patients. NF1 patients were significantly younger at diagnosis (p<0.001) and had a significantly shorter survival time than sporadic patients (median survival 17 months vs. 42 months, Breslow p<0.05). The time interval to local recurrence and metastatic spread was also significantly shorter in NF1 patients (9.4 months vs. 30.0 months, p<0.01; 9.1 months vs. 33.2 months, p<0.001, respectively). In patients with the original histopathological data available (22 NF1 patients, 14 sporadic cases), NF1-associated MPNST showed a significantly higher cellularity compared to sporadic tumors (p<0.001) whereas sporadic MPNST featured a significantly higher pleomorphism (p<0.01). Most importantly, while histopathological variables correlated with French Fédération Nationale des Centres de Lutte Contre le Cancer grading in sporadic MPNST, this was not the case for NF1-associated tumors. The differences between NF1-associated and sporadic MPNST in regard to the clinical course and histopathology may reflect some fundamental differences in biology and pathomechanism of the two tumor groups. Our findings indicate the necessity for a separate grading scheme which takes into account the genetic background in NF1 patients.

  15. Primary uterine cervix melanoma resembling malignant peripheral nerve sheath tumor: a case report.

    PubMed

    Pusceddu, Sara; Bajetta, Emilio; Buzzoni, Roberto; Carcangiu, Maria Luisa; Platania, Marco; Del Vecchio, Michele; Ditto, Antonino

    2008-10-01

    A rare variant of malignant melanoma (MM) of the uterine cervix that mimics a malignant peripheral nerve sheath tumor (MPNST) is described. A 43-year-old white woman was admitted to the hospital complaining of genital discharge and vaginal bleeding. Neoadjuvant chemotherapy and total abdominal hysterectomy and bilateral salpingo-ovariectomy plus pelvic lymphadenectomy were performed, and the diagnosis was MPNST, FIGO IIB. Pathological examination showed a diffuse proliferation of amelanotic spindle cells and large, highly atypical, frequently multinucleated, bizarre, and S100-, HMB-45-, vimentin-positive cells. The patient remained disease-free for 43 months, when an abdominal computed tomographic scan showed local polypoid vaginal lesions, with histological features of typical MM. A pathological review was obtained in our institution by a gynecological pathologist, who defined the primary neoplasm in the cervix as an MM, with a pattern of growth histologically simulating an MPNST, metastatic to the vagina. To our knowledge, this is the first report in literature of MM of the uterine cervix resembling MPNST. Despite its rarity, this variant of MM should be considered when a diagnosis of cervix MPNST is made. The histological and immunohistochemical features of these different entities should be considered in the differential diagnosis.

  16. Molecular Heterogeneity of Canine Cutaneous Peripheral Nerve Sheath Tumors: A Drawback in the Diagnosis Refinement.

    PubMed

    Teixeira, Sílvia; Amorim, Irina; Rêma, Alexandra; Faria, Fátima; Gärtner, Fátima

    Peripheral nerve sheath tumors (PNSTs) belong to a very heterogeneous group of neoplasms occurring both in dogs and humans. The aim of the present study was to evaluate the histological and immunohistochemical features of canine cutaneous PNSTs contributing to further refine their diagnosis. The histopathological phenotype and biological behavior of 40 canine cutaneous PNSTs were evaluated and vimentin, S-100, glial fibrillary acidic protein (GFAP), neuron-specific enolase (NSE), desmin, alpha-smooth muscle actin (α-SMA) and Ki-67 immunoreactivity were assessed. Respectively, 17 and 23 lesions were classified as benign and malignant PNSTs. The malignant lesions were more often positive for S-100 and presented a proliferation index significantly higher when compared to the benign ones (p<0.05). The differential diagnosis of PNST on routine stained samples is difficult and the immunohistochemical examination may contribute to the final diagnosis. However, these lesions present a complex histogenesis and show very variable individual features; thus, an unequivocally immunohistochemical panel that could have supported the PNST diagnostic was not achieved. Nevertheless, we concluded that Ki-67 can be a useful marker helping to discriminate the biological behavior of canine PNST. Copyright © 2016 International Institute of Anticancer Research (Dr. John G. Delinassios), All rights reserved.

  17. [A rare case of nerve-sheath sarcoma with rhabdomyoblastic differentiation (malignant triton tumor)].

    PubMed

    Malerba, M; Garofalo, A

    2003-01-01

    Malignant peripheral nerve sheath tumors (MPNST) are spindle-cell sarcomas that appear in a setting of neurofibroma or schwannoma or are associated with peripheral nerves or demonstrate nerve sheath differentiation. Malignant triton tumor (MTT) is a subtype of MPNST that also contain tissue with skeletal muscle differentiation (embryonal, plemorphic and botryoid rhabdomyosarcoma). The estimated incidence of MPNSTs in patients with NF1 is 2-5% compared with 0.0001% in the general population and approximately 69% of the reported cases of MTT are associated with von Recklinghausen disease. In July 2002 a 37-year old man was readmitted to the Department of Oncologic Surgery of the S. Camillo-Forlanini Hospital in Rome for both a right-sided retroperitoneal paravertebral not palpable mass, incidentally detected at a follow-up MRI, and a left-sided popliteal mass, discovered at clinical evaluation. Seventeen months before, when the patient underwent surgery at the same Department for both a left-sided paravertebral inferior mediastinal neurofibroma and a right-sided axillary neurofibroma, diagnosis of von Recklinghausen disease (NF1) was made, according to the criteria established by the NIH Consensus Development. Conference on Neurofibromatosis of 1987. A xifopubic laparotomy was performed: the tumor appeared to be localized, well-capsulated and strictly associated to the lumbar and sacral nervous radicles (L4, L5, S1) without evidence of invasion. The tumor was completely resected with sparing of the psoas muscle and the lumbar plexus through a subperineural dissection technique. No intra-operative pathologic examination was performed. Postoperative pathologic findings showed evidence for a trition tumor. The popliteal mass was resected too and resulted to be a neurofibroma just like the tumors resected 17 months before when diagnosis of von Recklinghausen disease was made. The patient was disease free 6 months after initial surgery. Sarcoma arising in anatomic site

  18. Reproducibility and accuracy of optic nerve sheath diameter assessment using ultrasound compared to magnetic resonance imaging

    PubMed Central

    2013-01-01

    Background Quantification of the optic nerve sheath diameter (ONSD) by transbulbar sonography is a promising non-invasive technique for the detection of altered intracranial pressure. In order to establish this method as follow-up tool in diseases with intracranial hyper- or hypotension scan-rescan reproducibility and accuracy need to be systematically investigated. Methods The right ONSD of 15 healthy volunteers (mean age 24.5 ± 0.8 years) were measured by both transbulbar sonography (9 – 3 MHz) and 3 Tesla MRI (half-Fourier acquisition single-shot turbo spin-echo sequences, HASTE) 3 and 5 mm behind papilla. All volunteers underwent repeated ultrasound and MRI examinations in order to assess scan-rescan reproducibility and accuracy. Moreover, inter- and intra-observer variabilities were calculated for both techniques. Results Scan-rescan reproducibility was robust for ONSD quantification by sonography and MRI at both depths (r > 0.75, p ≤ 0.001, mean differences < 2%). Comparing ultrasound- and MRI-derived ONSD values, we found acceptable agreement between both methods for measurements at a depth of 3 mm (r = 0.72, p = 0.002, mean difference < 5%). Further analyses revealed good inter- and intra-observer reliability for sonographic measurements 3 mm behind the papilla and for MRI at 3 and 5 mm (r > 0.82, p < 0.001, mean differences < 5%). Conclusions Sonographic ONSD quantification 3 mm behind the papilla can be performed with good reproducibility, measurement accuracy and observer agreement. Thus, our findings emphasize the feasibility of this technique as a non-invasive bedside tool for longitudinal ONSD measurements. PMID:24289136

  19. Radiation-Induced Malignant Peripheral Nerve Sheath Tumors: A Systematic Review.

    PubMed

    Yamanaka, Ryuya; Hayano, Azusa

    2017-09-01

    Radiation-induced malignant peripheral nerve sheath tumors (MPNSTs) are an uncommon late risk of irradiation. We conducted the largest systematic review to date of individual patient data for patients with these tumors. We conducted a systematic search using the PubMed database, and compiled a systematic literature review. We used Kaplan-Meier analysis and a log-rank test to estimate survival. We analyzed 65 radiation-induced and 26 radiation-associated MPNSTs in patients with neurofibromatosis. The mean ages of onset for primary lesions of the 2 types were 31.7 ± 18.2 and 17.1 ± 12.4 years, respectively (P = 0.0008). The latency periods between radiotherapy and onset of the 2 types of MPNSTs were 13.5 ± 7.8 and 11.8 ± 7.5 years, respectively (P = 0.3101). The median overall survival and 5-year survival were 11 months (6.8%) and 23 months (5.8%), respectively (P = 0.2168). Negative surgical margin and patient sex were variables retained for the prognosis of radiation-induced and radiation-associated MPNSTs. The prognosis of radiation-induced and radiation-associated MPNST was worse than that reported for de novo MPNSTs. Surgical complete resection is the mainstay for treatment of radiation-induced and radiation-associated MPNSTs. The risk of incidence of secondary MPNSTs in patients treated with radiotherapy warrants longer follow-up periods. Copyright © 2017 Elsevier Inc. All rights reserved.

  20. Confirmation of mutation landscape of NF1-associated malignant peripheral nerve sheath tumors.

    PubMed

    Sohier, Pierre; Luscan, Armelle; Lloyd, Angharad; Ashelford, Kevin; Laurendeau, Ingrid; Briand-Suleau, Audrey; Vidaud, Dominique; Ortonne, Nicolas; Pasmant, Eric; Upadhyaya, Meena

    2017-05-01

    The commonest tumors associated with neurofibromatosis type 1 (NF1) are benign peripheral nerve sheath tumors, called neurofibromas. Malignant transformation of neurofibromas into aggressive MPNSTs may occur with a poor patient prognosis. A cooperative role of SUZ12 or EED inactivation, along with NF1, TP53, and CDKN2A loss-of-function, has been proposed to drive progression to MPNSTs. An exome sequencing analysis of eight MPNSTs, one plexiform neurofibroma, and seven cutaneous neurofibromas was undertaken. Biallelic inactivation of the NF1 gene was observed in the plexiform neurofibroma and the MPNSTs, underlining that somatic biallelic NF1 inactivation is likely to be the initiating event for plexiform neurofibroma genesis, although it is unlikely to be sufficient for the subsequent MPNST development. The majority (5/8) of MPNSTs in our analyses demonstrated homozygous or heterozygous deletions of CDKN2A, which may represent an early event following NF1 LOH in the malignant transformation of Schwann cells from plexiform neurofibroma to MPNST. Biallelic somatic alterations of SUZ12 was also found in 4/8 MPNSTs. EED biallelic alterations were detected in 2 of the other four MPNSTs, with one tumor having a homozygous EED deletion. A missense mutation in the chromatin regulator KDM2B was also identified in one MPNST. No TP53 point mutations were found in this study, confirming previous data that TP53 mutations may be relatively rare in NF1-associated MPNSTs. Our study confirms the frequent biallelic inactivation of PRC2 subunits SUZ12 and EED in MPNSTs, and suggests the implication of KDM2B.

  1. Dissecting the roles of Ephrin-A3 in malignant peripheral nerve sheath tumor by TALENs.

    PubMed

    Wang, Zhengguang; Liu, Zhendong; Liu, Bo; Liu, Gengyan; Wu, Song

    2015-07-01

    Malignant peripheral nerve sheath tumor (MPNST) is a rare and aggressive soft tissue sarcoma for which effective treatments have not yet been established due to poor understanding of its pathogenesis. Our previous study indicated that miR-210-mediated Ephrin-A3 (EFNA3) promotion of proliferation and invasion of MPNST cells plays an important role in MPNST tumorigenesis and progression. The purpose of the present study was to further investigate the roles of EFNA3 in MPNST. Constructed transcription activator-like effector nucleases (TALENs) and lentiviral vectors were transfected into MPNST ST88-14 (NF1 wild-type) and sNF96.2 (NF1 mutant type) cell lines to obtain gain- and loss-of-function cell lines for the EFNA3 function study. The results showed that the knockout of ENFA3 increased cellular viability and invasiveness of the MPNST cells. However, the adhesion ability of MPNST cells was enhanced or inhibited when EFNA3 was overexpressed or knocked out, respectively. It was also observed that knockout of EFNA3 significantly decreased the expression of phosphorylated FAK (p-FAK) and the tumor necrosis factor α (TNF-α) compared to that in the control cells, yet the expression of phosphatidylinositol 3-kinase (PI3K), GTPase, integrins, vascular endothelial growth factor (VEGF) and hypoxia-inducible factor 1α (HIF-α) increased significantly. Inversely, overexpression of EFNA3 significantly increased the expression of p-FAK and TNF-α compared to that in the control cells, yet the expression of PI3K, GTPase, integrins, VEGF and HIF-α decreased significantly. The results indicated that EFNA3 serves as a tumor suppressor in MPNST cells and it may play a critical role in the focal adhesion kinase (FAK) signaling and VEGF-associated tumor angiogenesis pathway. These findings may not only facilitate the better understanding of MPNST pathogenesis, but also suggest EFNA3 as a promising target for MPNST treatment.

  2. Neurofibromin specific antibody differentiates malignant peripheral nerve sheath tumors (MPNST) from other spindle cell neoplasms.

    PubMed

    Reuss, David E; Habel, Antje; Hagenlocher, Christian; Mucha, Jana; Ackermann, Ulrike; Tessmer, Claudia; Meyer, Jochen; Capper, David; Moldenhauer, Gerhard; Mautner, Victor; Frappart, Pierre-Olivier; Schittenhelm, Jens; Hartmann, Christian; Hagel, Christian; Katenkamp, Kathrin; Petersen, Iver; Mechtersheimer, Gunhild; von Deimling, Andreas

    2014-04-01

    Malignant peripheral nerve sheath tumors (MPNST) derive from the Schwann cell or perineurial cell lineage and occur either sporadically or in association with the tumor syndrome neurofibromatosis type 1 (NF1). MPNST often pose a diagnostic challenge due to their frequent lack of pathognomonic morphological or immunohistochemical features. Mutations in the NF1 tumor suppressor gene are found in all NF1-associated and many sporadic MPNST. The presence of NF1 mutation may have the potential to differentiate MPNST from several morphologically similar neoplasms; however, mutation detection is hampered by the size of the gene and the lack of mutational hot spots. Here we describe a newly developed monoclonal antibody binding to the C-terminus of neurofibromin (clone NFC) which was selected for optimal performance in routinely processed formalin-fixed and paraffin-embedded tissue. NFC immunohistochemistry revealed loss of neurofibromin in 22/25 (88 %) of NF1-associated and 26/61 (43 %) of sporadic MPNST. There was a strong association of neurofibromin loss with deletions affecting the NF1 gene (P < 0.01). In a series of 256 soft tissue tumors of different histotypes NFC staining showed loss of neurofibromin in 2/8 myxofibrosarcomas, 2/12 (16 %) pleomorphic liposarcomas, 1/16 (6 %) leiomyosarcomas, and 4/28 (14 %) unclassified undifferentiated pleomorphic sarcomas. However, loss of neurofibromin was not observed in 22 synovial sarcomas, 27 schwannomas, 23 solitary fibrous tumors, 14 low-grade fibromyxoid sarcomas, 50 dedifferentiated liposarcomas, 27 myxoid liposarcomas, 13 angiosarcomas, 9 extraskeletal myxoid chondrosarcomas, and 7 epitheloid sarcomas. Immunohistochemistry using antibody NFC may substantially facilitate sarcoma research and diagnostics.

  3. Morphological spectrum of peripheral nerve sheath tumors: An insight into World Health Organization 2013 classification

    PubMed Central

    Chikkannaiah, Panduranga; Boovalli, Mythri M.; Nathiyal, Velusamy; Venkataramappa, Srinivasamurthy

    2016-01-01

    Introduction: Peripheral nerve sheath tumors (PNSTs) are neuroectodermal in origin. Now these tumors are classified under World Health Organization (WHO) classification of tumors of soft tissue and bone 2013. Objective: To study the morphological spectrum of PNST and to study the secondary degenerative changes associated with it. Materials and Methods: This study was conducted from January 2010 to June 2015. The gross details of tumor and patient's demographic profiles were reviewed. The hematoxylin and eosin stained slides were reassessed and the lesions were categorized and classified as per the WHO 2013 classification. The tumors were also assessed for secondary degenerative changes. Results: Our study comprised 143 cases of PNST. Age of the patients ranged from 5 to 75 years. 21–30 years is the most common age of occurrence with head and neck being the most common site. The PNSTs observed in the present study were neurofibroma (NF) (61.5%), schwannoma (36%), malignant PNST (2%), and granular cell tumor (0.5%). Nearly 10% of NF fulfilled the criteria for neurofibromatosis 1 (NF1). Rare tumors such as plexiform schwannoma and granular cell tumor were also observed. Malignant tumors were larger in dimension than benign. Myxoid, cystic, and hyaline changes were commonly associated with benign tumors while necrosis, hemorrhage, and mitotic activity were seen with malignant tumors. Conclusion: This series highlights the pathological variants of PNST along with their morphological changes and NF1 association. It is essential to be familiar with all these variants of PNST for accurate diagnosis as they have varied biological behavior. PMID:27365950

  4. Intrathoracic peripheral nerve sheath tumors-a clinicopathological study of 75 cases.

    PubMed

    Boland, Jennifer M; Colby, Thomas V; Folpe, Andrew L

    2015-03-01

    Although peripheral nerve sheath tumors (PNSTs) are common in the posterior mediastinum, they are rare in other mediastinal compartments and in the pleuropulmonary parenchyma. We sought to characterize the clinicopathological features of PNSTs occurring in the lung, pleura, and mediastinum. Diagnoses were confirmed by slide review. Study cases include 21 benign pleuropulmonary PNSTs, 49 benign mediastinal PNSTs, and 5 malignant PNSTs. Benign pleuropulmonary tumors comprised 13 schwannomas, 6 neurofibromas, 1 perineurioma, and 1 ganglioneuroma. Six lesions were endobronchial (3 neurofibromas, 1 schwannoma, 1 perineurioma, 1 ganglioneuroma), whereas the remaining schwannomas and neurofibromas formed parenchymal masses (usually pleural based). Benign mediastinal PNSTs (46 posterior, 2 middle, and 1 anterior) were all schwannomas and showed a female predominance. None of the patients with benign PNSTs experienced recurrence. Of the 5 malignant PNSTs, 4 were pleuropulmonary (3 pleural based) and 1 occurred in the anterior mediastinum. Two of the 5 patients had a history of neurofibromatosis type 1 (aged 27 and 45 years). At last follow-up, 3 of the 5 patients had died of disease, 1 was alive with disease, and 1 was alive with no evidence of disease (41 months). Although rare, a wide histologic range of PNSTs occur in the lung. Although neurofibroma, perineurioma, and ganglioneuroma were observed as endobronchial lesions, most pulmonary schwannomas were pleural-based masses. Mediastinal PNSTs are dominated by posterior mediastinal schwannomas, although schwannomas rarely occur in the other mediastinal compartments as well. Malignant PNSTs are very rare in the thorax, where they show aggressive behavior. Copyright © 2015 Elsevier Inc. All rights reserved.

  5. Noninvasive and quantitative intracranial pressure estimation using ultrasonographic measurement of optic nerve sheath diameter

    PubMed Central

    Wang, Li-juan; Yao, Yan; Feng, Liang-shu; Wang, Yu-zhi; Zheng, Nan-nan; Feng, Jia-chun; Xing, Ying-qi

    2017-01-01

    We aimed to quantitatively assess intracranial pressure (ICP) using optic nerve sheath diameter (ONSD) measurements. We recruited 316 neurology patients in whom ultrasonographic ONSD was measured before lumbar puncture. They were randomly divided into a modeling and a test group at a ratio of 7:3. In the modeling group, we conducted univariate and multivariate analyses to assess associations between ICP and ONSD, age, sex, BMI, mean arterial blood pressure, diastolic blood pressure. We derived the mathematical function “Xing & Wang” from the modelling group to predict ICP and evaluated the function in the test group. In the modeling group, ICP was strongly correlated with ONSD (r = 0.758, p < 0.001), and this association was independent of other factors. The mathematical function was ICP = −111.92 + 77.36 × ONSD (Durbin-Watson value = 1.94). In the test group, a significant correlation was found between the observed and predicted ICP (r = 0.76, p < 0.001). Bland-Altman analysis yielded a mean difference between measurements of −0.07 ± 41.55 mmH2O. The intraclass correlation coefficient and its 95%CIs for noninvasive ICP assessments using our prediction model was 0.86 (0.79–0.90). Ultrasonographic ONSD measurements provide a potential noninvasive method to quantify ICP that can be conducted at the bedside. PMID:28169341

  6. Use of T2-weighted magnetic resonance imaging of the optic nerve sheath to detect raised intracranial pressure

    PubMed Central

    Geeraerts, Thomas; Newcombe, Virginia FJ; Coles, Jonathan P; Abate, Maria Giulia; Perkes, Iain E; Hutchinson, Peter JA; Outtrim, Jo G; Chatfield, Dot A; Menon, David K

    2008-01-01

    Introduction The dural sheath surrounding the optic nerve communicates with the subarachnoid space, and distends when intracranial pressure is elevated. Magnetic resonance imaging (MRI) is often performed in patients at risk for raised intracranial pressure (ICP) and can be used to measure precisely the diameter of optic nerve and its sheath. The objective of this study was to assess the relationship between optic nerve sheath diameter (ONSD), as measured using MRI, and ICP. Methods We conducted a retrospective blinded analysis of brain MRI images in a prospective cohort of 38 patients requiring ICP monitoring after severe traumatic brain injury (TBI), and in 36 healthy volunteers. ONSD was measured on T2-weighted turbo spin-echo fat-suppressed sequence obtained at 3 Tesla MRI. ICP was measured invasively during the MRI scan via a parenchymal sensor in the TBI patients. Results Measurement of ONSD was possible in 95% of cases. The ONSD was significantly greater in TBI patients with raised ICP (>20 mmHg; 6.31 ± 0.50 mm, 19 measures) than in those with ICP of 20 mmHg or less (5.29 ± 0.48 mm, 26 measures; P < 0.0001) or in healthy volunteers (5.08 ± 0.52 mm; P < 0.0001). There was a significant relationship between ONSD and ICP (r = 0.71, P < 0.0001). Enlarged ONSD was a robust predictor of raised ICP (area under the receiver operating characteristic curve = 0.94), with a best cut-off of 5.82 mm, corresponding to a negative predictive value of 92%, and to a value of 100% when ONSD was less than 5.30 mm. Conclusions When brain MRI is indicated, ONSD measurement on images obtained using routine sequences can provide a quantitative estimate of the likelihood of significant intracranial hypertension. PMID:18786243

  7. Giant malignant peripheral nerve sheath tumor of thigh in an adolescent with neurofibromatosis type 1: a case report

    PubMed Central

    Tosun, Hacı Bayram; Serbest, Sancar; Turk, Bilge Aydın; Gumustas, Seyit Ali; Uludag, Abuzer

    2015-01-01

    Malignant peripheral nerve sheath tumors (MPNSTs) are rare sarcomas of children and adolescents, and they are aggressive tumors with a high rate of local recurrence. We present a 15-year-old boy with neurofibromatosis type 1 (NF1), who had a giant MPNST on the right thigh taking into account the available literature. Diagnosis of MPNST may be delayed in NF1 patients due to confusion with a neurofibroma and/or a plexiform neurofibroma. Malignancy should be considered, especially in cases with big masses, with heterogeneous involvement, or in the presence of cysts or necrotic nodules. The aim of surgical treatment is complete surgical excision. PMID:26604833

  8. Ultrasonographic measurement of optic nerve sheath diameter: A point of care test helps in prognostication of Intensive Care Unit patients

    PubMed Central

    Banerjee, Arnab; Bala, Renu; Saini, Savita

    2017-01-01

    Early identification of elevated intracranial pressure (ICP) is critical to ensuring timely and appropriate management to improve patient outcome. Measurement of the optic nerve sheath diameter by ultrasound is a well studied modality for noninvasive assessment of ICP. Recent studies have shown it to correlate with invasively measured ICP. We utilized this technique in our ICU and found it to be of great help in guiding patient management and predicting the prognosis. A case series of four patients is reported illustrating its utility in ICU patients. PMID:28405042

  9. Rexin-G®, a tumor-targeted retrovector for malignant peripheral nerve sheath tumor: A case report

    PubMed Central

    Kim, Seth; Federman, Noah; Gordon, Erlinda M.; Hall, Frederick L.; Chawla, Sant P.

    2017-01-01

    Soft tissue sarcoma is a rare neoplasm of mesenchymal origin, accounting for only ~1% of all adult cancers and consisting of 75 histological subtypes. In the present report, the unique case of a 14 year-old female with metastatic malignant peripheral nerve sheath tumor (formerly, malignant melanotic schwannoma) of the parotid gland, who experienced a durable response and sustained tumor control with Rexin-G®, a tumor-targeted retroviral expression vector encoding an anti-cyclin G1 construct, is described. Post-parotidectomy, and prior to the administration of Rexin-G®, the patient received various chemotherapy regimens, including doxorubicin, ifosfamide, temozolomide, sorafenib, and an immunological therapy with interleukin-2, which only resulted in the further progression of lung metastases. The patient subsequently participated in a Phase 1/2 gene therapy study, during which she received intravenous Rexin-G® as monotherapy for two years with minimal drug-associated adverse events. Currently, the patient has no evidence of active disease 9 years after commencing the Rexin-G® treatment, and with no additional anti-cancer therapy. In conclusion, Rexin-G® may be a viable therapeutic option for malignant peripheral nerve sheath tumors, and should be further investigated in prospective histology-specific clinical trials for this type, and possibly other types, of chemotherapy-resistant sarcoma. PMID:28588778

  10. Epithelioid Malignant Peripheral Nerve Sheath Tumor Arising in a Schwannoma, in a Patient with “Neuroblastoma-like” Schwannomatosis and a Novel Germline SMARCB1 mutation

    PubMed Central

    Carter, Jodi M.; O'Hara, Carolyn; Dundas, George; Gilchrist, Dawna; Collins, Mark S.; Eaton, Katherine; Judkins, Alexander R.; Biegel, Jaclyn A.; Folpe, Andrew L.

    2011-01-01

    Epithelioid malignant peripheral nerve sheath tumors arising in pre-existing schwannomas are extremely rare. We report an unusual example occurring in a patient with multiple schwannomas (schwannomatosis), all but one of which showed “neuroblastoma-like” histology. By immunohistochemistry, both the epithelioid malignant peripheral nerve sheath tumor and the schwannomas showed a complete loss of the Smarcb1 protein. Subsequent genetic evaluation revealed the presence of a novel germline mutation in the SMARCB1/INI1 gene in the patient and three of her children, two of whom were diagnosed with atypical teratoid/rhabdoid tumors of the brain. PMID:22082606

  11. Verticillin A Inhibits Leiomyosarcoma and Malignant Peripheral Nerve Sheath Tumor Growth via Induction of Apoptosis

    PubMed Central

    Zewdu, A; Lopez, G; Braggio, D; Kenny, C; Constantino, D; Bid, HK; Batte, K; Iwenofu, OH; Oberlies, NH; Pearce, CJ; Strohecker, AM; Lev, D; Pollock, RE

    2017-01-01

    Objective The heterogeneity of soft tissue sarcoma (STS) represents a major challenge for the development of effective therapeutics. Comprised of over 50 different histology subtypes of various etiologies, STS subsets are further characterized as either karyotypically simple or complex. Due to the number of genetic anomalies associated with genetically complex STS, development of therapies demonstrating potency against this STS cluster is especially challenging and yet greatly needed. Verticillin A is a small molecule natural product with demonstrated anticancer activity; however, the efficacy of this agent has never been evaluated in STS. Therefore, the goal of this study was to explore verticillin A as a potential STS therapeutic. Methods We performed survival (MTS) and clonogenic analyses to measure the impact of this agent on the viability and colony formation capability of karyotypically complex STS cell lines: malignant peripheral nerve sheath tumor (MPNST) and leiomyosarcoma (LMS). The in vitro effects of verticillin A on apoptosis were investigated through annexin V/PI flow cytometry analysis and by measuring fluorescently-labeled cleaved caspase 3/7 activity. The impact on cell cycle progression was assessed via cytometric measurement of propidium iodide intercalation. In vivo studies were performed using MPNST xenograft models. Tumors were processed and analyzed using immunohistochemistry (IHC) for verticillin A effects on growth (Ki67) and apoptosis (cleaved caspase 3). Results Treatment with verticillin A resulted in decreased STS growth and an increase in apoptotic levels after 24 h. 100 nM verticillin A induced significant cellular growth abrogation after 24 h (96.7, 88.7, 72.7, 57, and 39.7% reduction in LMS1, S462, ST88, SKLMS1, and MPNST724, respectively). We observed no arrest in cell cycle, elevated annexin, and a nearly two-fold increase in cleaved caspase 3/7 activity in all MPNST and LMS cell lines. Control normal human Schwann (HSC) and

  12. Posttraumatic meningioma.

    PubMed

    Schiffer, J; Avidan, D; Rapp, A

    1985-07-01

    This report concerns three patients with intracranial meningioma developing at the site of an old head injury with skull fracture. These cases, along with literature reports, suggest a causal relationship between head trauma and the subsequent development of meningioma.

  13. Meningioma Causing Visual Impairment: Outcomes and Toxicity After Intensity Modulated Radiation Therapy

    SciTech Connect

    Maclean, Jillian; Fersht, Naomi; Bremner, Fion; Stacey, Chris; Sivabalasingham, Suganya; Short, Susan

    2013-03-15

    Purpose: To evaluate ophthalmologic outcomes and toxicity of intensity modulated radiation therapy (IMRT) in patients with meningiomas causing visual deficits. Methods and Materials: A prospective observational study with formal ophthalmologic and clinical assessment of 30 consecutive cases of meningioma affecting vision treated with IMRT from 2007 to 2011. Prescriptions were 50.4 Gy to mean target dose in 28 daily fractions. The median follow-up time was 28 months. Twenty-six meningiomas affected the anterior visual pathway (including 3 optic nerve sheath meningiomas); 4 were posterior to the chiasm. Results: Vision improved objectively in 12 patients (40%). Improvements were in visual field (5/16 patients), color vision (4/9 patients), acuity (1/15 patients), extraocular movements (3/11 patients), ptosis (1/5 patients), and proptosis (2/6 patients). No predictors of clinical response were found. Two patients had minor reductions in tumor dimensions on magnetic resonance imaging, 1 patient had radiological progression, and the other patients were stable. One patient experienced grade 2 keratitis, 1 patient had a minor visual field loss, and 5 patients had grade 1 dry eye. Conclusion: IMRT is an effective method for treating meningiomas causing ophthalmologic deficits, and toxicity is minimal. Thorough ophthalmologic assessment is important because clinical responses often occur in the absence of radiological change.

  14. Constructing a statistical atlas of the radii of the optic nerve and cerebrospinal fluid sheath in young healthy adults

    NASA Astrophysics Data System (ADS)

    Harrigan, Robert L.; Plassard, Andrew J.; Mawn, Louise A.; Galloway, Robert L.; Smith, Seth A.; Landman, Bennett A.

    2015-03-01

    Optic neuritis is a sudden inflammation of the optic nerve (ON) and is marked by pain on eye movement, and visual symptoms such as a decrease in visual acuity, color vision, contrast and visual field defects. The ON is closely linked with multiple sclerosis (MS) and patients have a 50% chance of developing MS within 15 years. Recent advances in multi-atlas segmentation methods have omitted volumetric assessment. In the past, measuring the size of the ON has been done by hand. We utilize a new method of automatically segmenting the ON to measure the radii of both the ON and surrounding cerebrospinal fluid (CSF) sheath to develop a normative distribution of healthy young adults. We examine this distribution for any trends and find that ON and CSF sheath radii do not vary between 20-35 years of age and between sexes. We evaluate how six patients suffering from optic neuropathy compare to this distribution of controls. We find that of these six patients, five of them qualitatively differ from the normative distribution which suggests this technique could be used in the future to distinguish between optic neuritis patients and healthy controls

  15. Optic Nerve Sheath Diameter: Translating a Terrestrial Focused Technique Into a Clinical Monitoring Tool for Space Flight

    NASA Technical Reports Server (NTRS)

    Mason, Sara S.; Foy, Millennia; Sargsyan, Ashot; Garcia, Kathleen; Wear, Mary L.; Bedi, Deepak; Ernst, Randy; Van Baalen, Mary

    2014-01-01

    Emergency medicine physicians recently adopted the use of ultrasonography to quickly measure optic nerve sheath diameter (ONSD) as concomitant with increased intracranial pressure. NASA Space and Clinical Operations Division has been using ground and on-orbit ultrasound capabilities since 2009 to consider this anatomical measure as a proxy for intracranial pressure in the microgravity environment. In the terrestrial emergency room population, an ONSD greater than 0.59 cm is considered highly predictive of elevated intracranial pressure. However, this cut-off limit is not applicable to the spaceflight setting since over 50% of US Operating Segment (USOS) astronauts have an ONSD greater than 0.60 cm even before missions. Crew Surgeon clinical decision-making is complicated by the fact that many astronauts have history of previous spaceflights. Data will be presented characterizing the distribution of baseline ONSD in the astronaut corps, longitudinal trends in-flight, and the predictive power of this measure related to increased intracranial pressure outcomes.

  16. Intracranial pressure-induced optic nerve sheath response as a predictive biomarker for optic disc edema in astronauts.

    PubMed

    Wostyn, Peter; De Deyn, Peter Paul

    2017-09-04

    A significant proportion of the astronauts who spend extended periods in microgravity develop ophthalmic abnormalities. Understanding this syndrome, called visual impairment and intracranial pressure (VIIP), has become a high priority for National Aeronautics and Space Administration, especially in view of future long-duration missions (e.g., Mars missions). Moreover, to ensure selection of astronaut candidates who will be able to complete long-duration missions with low risk of the VIIP syndrome, it is imperative to identify biomarkers for VIIP risk prediction. Here, we hypothesize that the optic nerve sheath response to alterations in intracranial pressure may be a potential predictive biomarker for optic disc edema in astronauts. If confirmed, this biomarker could be used for preflight identification of astronauts at risk for developing VIIP-associated optic disc edema.

  17. Primary hepatic malignant peripheral nerve sheath tumor successfully treated with combination therapy: a case report and literature review

    PubMed Central

    Jung, Hae Il; Lee, Hyoung Uk; Ahn, Tae Sung; Lee, Jong Eun; Lee, Hyun Yong; Cho, Hyon Doek; Lee, Sang Cheol

    2016-01-01

    Primary malignant peripheral nerve sheath tumor (MPNST) in a young female patient, not associated with neurofibromatosis type-I is extremely rare in the liver. A 33-year-old female was admitted with a right flank pain for a weak. The CT scan showed 12.5-cm-sized mass located at the right hepatic lobe. At laparotomy, about 20.0-cm-sized mass was on the right hepatic lobe with attachment to right diaphragmatic pleura. Right hepatic lobe and adherent part of diaphragmatic pleura were resected. On histology and immunohistochemistry, it was diagnosed MPNST. Adjuvant radiotherapy for the right diaphragmatic pleura and adjuvant chemotherapy with adriamycin, ifosfamide and cisplatin were sequentially performed. The prognosis of MPNST is generally poor and it is associated with a highly aggressive course of recurrence, metastases, and death. Our case is probably a first report about combination therapy. PMID:27904856

  18. [Malignant peripheric nerve sheath tumor of the orbit: first description of orbital location in a patient with NF1].

    PubMed

    Romero-Rojas, A E; Díaz-Pérez, J A; Lozano-Castillo, A

    2010-02-01

    The malignant peripheric nerve sheath tumor (MPNST), is a malignant neoplastic lesion originated in Schwann cells of the lining sheath of peripheral nerves. This neoplasia may appear with benign or malignant heterologous components, with divergent differentiation, as the glandular one. To describe for the first time in the literature, a case of a glandular MPNST, located at the orbit and to revise the literature on this tumoral lesion. Nine year old male, with a base diagnosis of NF1, who had exophthalmos, retro-ocular pain, headache, facial asymmetry and descent of the right eyeball, that started 1 year earlier. This patient showed in the Computed Tomography an Magnetic Resonance, a well delimited, lobulated, solid mass at the eyeball, which extended to the fontal and temporal brain parenchyma. A right Fronto-temporal craniotomy was made with fronto -orbital- zygomatic resection of the tumoral lesion. Later, a dural plasty and reconstruction with titanium mesh was made at the skull base. At present, the patient is asymptomatic after 4 months of follow up. A malignant biphasic neoplastic lesion was observed, reactive in the mesenchymal elements S100, PGP 9.5, neurofilaments and vimentin. The glandular component was positive for AE1/AE3, EMA, CEA and focally for CD57. There was also reactivity to cromogranin, synaptophysin, serotonin and somatostatin. The diagnosis of Glandular MPNST was made. For the first time in the literature a case of Glandular MPNST located at the orbit, which occurred in child with NF1, is described. This extremely uncommon neoplasia must be taken into account, in the study of biphasic malignant lesions, as its diagnosis is of great importance because of the bad prognosis of the affected patients.

  19. Narrowing of Meckel's cave and cavernous sinus and enlargement of the optic nerve sheath in Pseudotumor Cerebri.

    PubMed

    Degnan, Andrew Joseph; Levy, Lucien M

    2011-01-01

    Pseudotumor cerebri (PTC) is a clinical entity of uncertain etiology associated with several subtle findings on magnetic resonance imaging (MRI) including posterior flattening of the globes, enlargement of the optic nerve sheath (ONS), empty sella sign. We aimed to characterize the incidence of and significance of 2 novel MRI findings: narrowing of Meckel's cave and of cavernous sinus. Forty-six patients with a condition diagnosed as PTC based on clinical history were retrospectively reviewed, and their MRI studies were assessed for previously reported imaging findings associated with PTC. The maximal diameters of the cavernous sinuses, Meckel's caves, and ONSs were measured along with those of age-matched controls on axial T2-weighted images. The Meckel's caves and cavernous sinuses are significantly (P < 0.01) narrowed in patients (mean diameters: 0.41 and 0.25 cm) versus controls (0.54 and 0.36 cm), respectively. The ONS was enlarged in patients with a mean diameter of 0.65 cm versus 0.54 cm (P < 0.01). Meckel's cave narrowing and ONS enlargement seem to be better indicators of PTC than cavernous sinus narrowing, with sensitivities of 78.3% and 86.9% and specificities of 84.8% and 76.1% versus 60.9% and 76.1%, respectively. This finding of narrowed Meckel's caves in PTC may be clinically useful as a novel imaging finding seen on routine MRI studies. Optic nerve sheath enlargement is also confirmed as an important finding in PTC.

  20. Meningiomas of Meckel's cave.

    PubMed

    Nijensohn, D E; Araujo, J C; MacCarty, C S

    1975-08-01

    A retrospective review of 12 cases of meningioma of Meckel's cave involving the Gasserian ganglion or the trigeminal posterior root (or both) seen at the Mayo Clinic during a 20-year period suggested three clearly defined clinical groups. One group (the largest) had typical trigeminal neuralgia and an excellent prognosis after the removal of the easily detachable mass that was impinging on the ganglion. A second group, with meningiomas en plaque embedded in the ganglion, had a history of atypical trigeminal face pain without neurological deficit, but the prognosis for pain relief was not as good as in the previous group. A third group had a history of face dysesthesias and pain, objective trigeminal sensory loss, and multiple cranial nerve deficit; these patients had meningiomas with histological signs of mitotic activity and a poor prognosis, with return of intractable pain and recurrence of the tumor.

  1. Electrophoresis of polar fluorescent tracers through the nerve sheath labels neuronal populations for anatomical and functional imaging

    PubMed Central

    Isaacson, Matthew D.; Hedwig, Berthold

    2017-01-01

    The delivery of tracers into populations of neurons is essential to visualize their anatomy and analyze their function. In some model systems genetically-targeted expression of fluorescent proteins is the method of choice; however, these genetic tools are not available for most organisms and alternative labeling methods are very limited. Here we describe a new method for neuronal labelling by electrophoretic dye delivery from a suction electrode directly through the neuronal sheath of nerves and ganglia in insects. Polar tracer molecules were delivered into the locust auditory nerve without destroying its function, simultaneously staining peripheral sensory structures and central axonal projections. Local neuron populations could be labelled directly through the surface of the brain, and in-vivo optical imaging of sound-evoked activity was achieved through the electrophoretic delivery of calcium indicators. The method provides a new tool for studying how stimuli are processed in peripheral and central sensory pathways and is a significant advance for the study of nervous systems in non-model organisms. PMID:28084413

  2. Cytohistologic correlations of 24 malignant peripheral nerve sheath tumor (MPNST) in 17 patients: the Institut Curie experience.

    PubMed

    Klijanienko, Jerzy; Caillaud, Jean-Michel; Lagacé, Réal; Vielh, Philippe

    2002-08-01

    Cytomorphological patterns of malignant peripheral nerve sheath tumor (MPNST) are insufficiently documented in the literature. Cytological and histological specimens in 24 tumors in 17 patients were correlated. The review of the original cytology reports showed that four (16.6%) tumors were correctly diagnosed, eight (33.3%) were diagnosed as sarcoma not otherwise specified, four (16.7%) as fibrosarcoma, three (12.5%) as synovial sarcoma, three (12.5%) as leiomyosarcoma, and one (4.2%) case each as malignant fibrous histiocytoma and rhabdomyosarcoma. At the review tumors were histologically reclassified as well-differentiated MPNST in 11 (45.9%) cases, anaplastic MPNST in 11 (45.9%) cases, and epithelioid MPNST and malignant Triton tumor in one (4.2%) case each. Cytologically, well-differentiated MPNST were composed of polymorphous oval to round cells, small spindle-shaped cells with wavy and comma-like naked nuclei, and a fibrillary, delicate stroma. Anaplastic MPNST, moreover, were composed of anaplastic giant and polymorphous cells. The malignant Triton tumor was composed of oval to round rhabdomyoblastic cells with eccentric nuclei and the epithelioid MPNST of polymorphous and round, epithelial-like cells. The cytological diagnosis of MPNST may be difficult, especially in anaplastic tumors. The correlation between the cytological features and the clinical information--origin of the tumor from a nerve trunk, a preexisting neurofibroma, patients with known history of neurofibromatosis 1--could be indicative of an MPNST diagnosis.

  3. Cerebrospinal fluid dissemination of anaplastic intraventricular meningioma: report of a case presenting with progressive brainstem dysfunction and multiple cranial nerve palsies.

    PubMed

    Fujimaki, Motoki; Takanashi, Masashi; Kobayashi, Manami; Wada, Kei-ichiro; Machida, Yutaka; Kondo, Akihide; Hattori, Nobutaka; Miwa, Hideto

    2016-05-31

    It is extremely rare to see cerebrospinal fluid dissemination of intraventricular meningioma, particularly with the development of acute, progressive brainstem/cerebellar dysfunction with an absence of mass formation in the corresponding anatomical sites. An 81-year-old man was admitted because of double vision, right facial nerve palsy and truncal ataxia. Brain magnetic resonance imaging showed normal findings except for a tumor mass in the left lateral ventricle, which had been noted over 6 months previously. The patient developed hiccups, hyperventilation, and drowsiness, which worsened progressively, and did not respond to corticosteroid or intraventricular immunoglobulin therapy. Cerebrospinal fluid study revealed a mild elevation of protein, and cytology was negative. The patient died and an autopsy was performed. Postmortem investigation disclosed a malignant transformation of benign fibroid meningioma with cerebrospinal fluid dissemination of the malignant cells, diversely involving the surface of brainstem, cerebellum, and spinal cords, secondarily resulting in extensive ischemia in the brain parenchyma by vessel occlusion. If a patient with an intraventricular tumor develops acute, progressive neurological symptoms, the possibility that it is be caused by cerebrospinal fluid dissemination of tumor cells, after malignant transformation, should be considered.

  4. Association of tumor location, extent of resection, and neurofibromatosis status with clinical outcomes for 221 spinal nerve sheath tumors.

    PubMed

    Safaee, Michael; Parsa, Andrew T; Barbaro, Nicholas M; Chou, Dean; Mummaneni, Praveen V; Weinstein, Philip R; Tihan, Tarik; Ames, Christopher P

    2015-08-01

    OBJECT Intradural extramedullary spine tumors represent two-thirds of all primary spine neoplasms. Approximately half of these are peripheral nerve sheath tumors, mainly neurofibromas and schwannomas. Given the rarity of this disease and, thus, the limited analyses of clinical outcomes, the authors examined the association of tumor location, extent of resection, and neurofibromatosis (NF) status with clinical outcomes. METHODS Patients were identified through a search of the University of California, San Francisco, neuropathology database and a separate review of current procedural terminology codes. Data recorded included patient age, patient sex, clinical presentation, presence of NF, tumor type, tumor location, extent of resection (gross-total resection [GTR] or subtotal resection [STR]), and clinical follow-up. RESULTS Of 221 tumors in 199 patients (mean age 45 years), 53 were neurofibromas, 163 were schwannomas, and 5 were malignant peripheral nerve sheath tumors. The most common presenting symptom was spinal pain (76%), followed by weakness (36%) and sensory abnormalities (34%). Mean symptom duration was 16 months. In terms of spinal location, neurofibromas were more common in the cervical spine (74% vs 27%, p < 0.001), and schwannomas were more common in the thoracic and lumbosacral spine (73% vs 26%, p < 0.001). Rates of GTR were lower for neurofibromas than schwannomas (51% vs 83%, p < 0.001), regardless of location. Rates of GTR were lower for cervical (54%) than thoracic (90%) and lumbosacral (86%) lesions (p < 0.001). NF was associated with lower rates of GTR among all tumors (43% vs 86%, p < 0.001). The mean follow-up time was 32 months. Recurrence/progression was more common for neurofibromas than schwannomas (17% vs 7%, p = 0.03), although the mean time to recurrence/progression did not differ according to tumor type (45 vs 53 months, p = 0.63). As expected, GTR was associated with lower recurrence rates (4% vs 22%, p < 0.001). According to

  5. Optic nerve sheath diameter measurement: a means of detecting raised ICP in adult traumatic and non-traumatic neurosurgical patients.

    PubMed

    Raffiz, Mohd; Abdullah, Jafri M

    2017-01-01

    Bedside ultrasound measurement of optic nerve sheath diameter (ONSD) is emerging as a non-invasive technique to evaluate and predict raised intracranial pressure (ICP). It has been shown in previous literature that ONSD measurement has good correlation with surrogate findings of raised ICP such as clinical and radiological findings suggestive of raised ICP. The objective of the study is to find a correlation between sonographic measurements of ONSD value with ICP value measured via the gold standard invasive intracranial ICP catheter, and to find the cut-off value of ONSD measurement in predicting raised ICP, along with its sensitivity and specificity value. A prospective observational study was performed using convenience sample of 41 adult neurosurgical patients treated in neurosurgical intensive care unit with invasive intracranial pressure monitoring placed in-situ as part of their clinical care. Portable SonoSite ultrasound machine with 7 MHz linear probe were used to measure optic nerve sheath diameter using the standard technique. Simultaneous ICP readings were obtained directly from the invasive monitoring. Seventy-five measurements were performed on 41 patients. The non-parametric Spearman correlation test revealed a significant correlation at the 0.01 level between the ICP and ONSD value, with correlation coefficient of 0.820. The receiver operating characteristic curve generated an area under the curve with the value of 0.964, and with standard error of 0.22. From the receiver operating characteristic curve, we found that the ONSD value of 5.205 mm is 95.8% sensitive and 80.4% specific in detecting raised ICP. ONSD value of 5.205 is sensitive and specific in detecting raised ICP. Bedside ultrasound measurement of ONSD is readily learned, and is reproducible and reliable in predicting raised ICP. This non-invasive technique can be a useful adjunct to the current invasive intracranial catheter monitoring, and has wide potential clinical applications in

  6. Influence of Breaching the Connective Sheaths of the Donor Nerve on Its Myelinated Sensory Axons and on Their Sprouting into the End-to-Side Coapted Nerve in the Rat

    PubMed Central

    Žele, Tilen; Tomšič, Martin; Sketelj, Janez; Bajrović, Fajko F.

    2012-01-01

    Abstract The influence of breaching the connective sheaths of the donor sural nerve on axonal sprouting into the end-to-side coapted peroneal nerve was examined in the rat. In parallel, the effect of these procedures on the donor nerve was assessed. The sheaths of the donor nerve at the coaptation site were either left completely intact (group A) or they were breached by epineurial sutures (group B), an epineurial window (group C), or a perineurial window (group D). In group A, the compound action potential (CAP) of sensory axons was detected in ∼10% and 40% of the recipient nerves at 4 and 8 weeks, respectively, which was significantly less frequently than in group D at both recovery periods. In addition, the number of myelinated axons in the recipient nerve was significantly larger in group D than in other groups at 4 weeks. At 8 weeks, the number of axons in group A was only ∼15% of the axon numbers in other groups (p<0.05). Focal subepineurial degenerative changes in the donor nerves were only seen after 4 weeks, but not later. The average CAP area and the total number of myelinated axons in the donor nerves were not different among the experimental groups. In conclusion, myelinated sensory axons are able to penetrate the epiperineurium of donor nerves after end-to-side nerve coaption; however, their ingrowth into recipient nerves is significantly enhanced by breaching the epiperineurial sheets at the coaptation site. Breaching does not cause permanent injury to the donor nerve. PMID:22873667

  7. Comprehensive establishment and characterization of orthoxenograft mouse models of malignant peripheral nerve sheath tumors for personalized medicine.

    PubMed

    Castellsagué, Joan; Gel, Bernat; Fernández-Rodríguez, Juana; Llatjós, Roger; Blanco, Ignacio; Benavente, Yolanda; Pérez-Sidelnikova, Diana; García-Del Muro, Javier; Viñals, Joan Maria; Vidal, August; Valdés-Mas, Rafael; Terribas, Ernest; López-Doriga, Adriana; Pujana, Miguel Angel; Capellá, Gabriel; Puente, Xose S; Serra, Eduard; Villanueva, Alberto; Lázaro, Conxi

    2015-05-01

    Malignant peripheral nerve sheath tumors (MPNSTs) are soft-tissue sarcomas that can arise either sporadically or in association with neurofibromatosis type 1 (NF1). These aggressive malignancies confer poor survival, with no effective therapy available. We present the generation and characterization of five distinct MPNST orthoxenograft models for preclinical testing and personalized medicine. Four of the models are patient-derived tumor xenografts (PDTX), two independent MPNSTs from the same NF1 patient and two from different sporadic patients. The fifth model is an orthoxenograft derived from an NF1-related MPNST cell line. All MPNST orthoxenografts were generated by tumor implantation, or cell line injection, next to the sciatic nerve of nude mice, and were perpetuated by 7-10 mouse-to-mouse passages. The models reliably recapitulate the histopathological properties of their parental primary tumors. They also mimic distal dissemination properties in mice. Human stroma was rapidly lost after MPNST engraftment and replaced by murine stroma, which facilitated genomic tumor characterization. Compatible with an origin in a catastrophic event and subsequent genome stabilization, MPNST contained highly altered genomes that remained remarkably stable in orthoxenograft establishment and along passages. Mutational frequency and type of somatic point mutations were highly variable among the different MPNSTs modeled, but very consistent when comparing primary tumors with matched orthoxenografts generated. Unsupervised cluster analysis and principal component analysis (PCA) using an MPNST expression signature of ~1,000 genes grouped together all primary tumor-orthoxenograft pairs. Our work points to differences in the engraftment process of primary tumors compared with the engraftment of established cell lines. Following standardization and extensive characterization and validation, the orthoxenograft models were used for initial preclinical drug testing. Sorafenib (a BRAF

  8. Combinatorial therapeutic targeting of BMP2 and MEK-ERK pathways in NF1-associated malignant peripheral nerve sheath tumors

    PubMed Central

    Ahsan, Sidra; Ge, Yubin; Tainsky, Michael A.

    2016-01-01

    The clinical management of malignant peripheral nerve sheath tumors (MPNSTs) is challenging not only due to its aggressive and invasive nature, but also limited therapeutic options. Using gene expression profiling, our lab identified BMP2-SMAD1/5/8 pathway as a potential therapeutic target for treating MPNSTs. In this study, we explored the therapeutic impact of targeting BMP2-SMAD1/5/8 pathway in conjunction with RAS-MEK-ERK signaling, which is constitutively activated in MPNSTs. Our results indicated that single agent treatment with LDN-193189, a BMP2 Type I receptor inhibitor, did not affect the growth and survival of MPNST cells at biochemically relevant inhibitory concentrations. However, addition of a MEK1/2 inhibitor, selumetinib, to LDN-193189-treated cells resulted in significant inhibition of cell growth and induction of cell death. LDN-193189 at biochemically effective concentrations significantly inhibited motility and invasiveness of MPNST cells, and these effects were enhanced by the addition of selumetinib. Overall, our results advocate for a combinatorial therapeutic approach for MPNSTs that not only targets the growth and survival via inhibition of MEK1/2, but also its malignant spread by suppressing the activation of BMP2-SMAD1/5/8 pathway. Importantly, these studies were conducted in low-passage patient-derived MPNST cells, allowing for an investigation of the effects of the proposed drug treatments in a biologically-relevant context. PMID:27494873

  9. Multicentric fibromyxoid peripheral nerve sheath tumor (multicentric schwannoma) in a dromedary camel (Camelus dromedarius): morphopathological, immunohistochemical, and electron microscopic studies.

    PubMed

    Khodakaram-Tafti, A; Khordadmehr, M

    2011-11-01

    During postslaughter inspection of a 4-year-old male dromedary camel (Camelus dromedarius), numerous small nodules to large masses up to 4 cm in diameter were found on the serosal surfaces of forestomachs, large intestines, mesentery, liver, and spleen. Grossly, the masses were discrete, round, smooth, and white to gray that bulged from the serosal layer. Cut surfaces of the masses were discrete, round, white, and relatively homogeneous without any necrotic foci. Histopathologically, the masses were encapsulated and composed of a mixture of round and spindle-shaped cells in loose whorls of neoplastic cells with small elongated hyperchromatic wavy nuclei and a small amount of pale eosinophilic, poorly defined cytoplasm. Masson's trichrome staining showed mild amounts of collagen fibers forming an irregular, loose stroma. In immunohistochemistry, immunoreactivity for the Schwann cell marker (S100) was diffusely positive in the neoplastic cells. The immunoreactivity for CK, c-kit, and CD34 were negative. Ultrastructural examination confirmed the tumor was entirely formed of neoplastic Schwann cells. On the basis of the histopathological, immunohistochemical, and ultrastructural findings, the tumors were diagnosed as multicentric fibromyxoid peripheral nerve sheath tumor (multicentric schwannoma). This tumor has not been previously recorded in camel worldwide.

  10. Photothermal therapy improves the efficacy of a MEK inhibitor in neurofibromatosis type 1-associated malignant peripheral nerve sheath tumors

    PubMed Central

    Sweeney, Elizabeth E.; Burga, Rachel A.; Li, Chaoyang; Zhu, Yuan; Fernandes, Rohan

    2016-01-01

    Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive tumors with low survival rates and the leading cause of death in neurofibromatosis type 1 (NF1) patients under 40 years old. Surgical resection is the standard of care for MPNSTs, but is often incomplete and can generate loss of function, necessitating the development of novel treatment methods for this patient population. Here, we describe a novel combination therapy comprising MEK inhibition and nanoparticle-based photothermal therapy (PTT) for MPNSTs. MEK inhibitors block activity driven by Ras, an oncogene constitutively activated in NF1-associated MPNSTs, while PTT serves as a minimally invasive method to ablate cancer cells. Our rationale for combining these seemingly disparate techniques for MPNSTs is based on several reports demonstrating the efficacy of systemic chemotherapy with local PTT. We combine the MEK inhibitor, PD-0325901 (PD901), with Prussian blue nanoparticles (PBNPs) as PTT agents, to block MEK activity and simultaneously ablate MPNSTs. Our data demonstrate the synergistic effect of combining PD901 with PBNP-based PTT, which converge through the Ras pathway to generate apoptosis, necrosis, and decreased proliferation, thereby mitigating tumor growth and increasing survival of MPNST-bearing animals. Our results suggest the potential of this novel local-systemic combination “nanochemotherapy” for treating patients with MPNSTs. PMID:27833160

  11. Chemotherapy for the treatment of malignant peripheral nerve sheath tumors in neurofibromatosis 1: a 10-year institutional review

    PubMed Central

    2013-01-01

    Background Neurofibromatosis 1 (NF1) is the most common autosomal dominant disorder, with an incidence of 1 in 2,500-3,300 live births. NF1 is associated with significant morbidity and mortality because of complications, especially malignant peripheral nerve sheath tumors (MPNSTs), which mainly develop during adulthood. We evaluated our experience with management of NF1 with MPNSTs by standard chemotherapy with anthracycline and/or ifosfamide in terms of time to treatment failure and overall survival. Methods We performed a retrospective review of consecutive patients with NF1 and a diagnosis of MPNSTs between 1993 and 2003 in our referral center for NF1. Prognostic factors were evaluated by univariate analysis. Results We evaluated data for 21 patients with grade 1 (n=1), grade 2 (n=8) and grade 3 (n=12) MPNST; 16 presented localized disease and underwent surgery: margins for 6 were tumor-free (including 3 patients with amputation), 2 showed microscopic residual disease and 8 showed macroscopic residual disease. All patients received chemotherapy and 9 radiotherapy. Median time to treatment failure and overall survival were 7.8 and 17 months, respectively. Two patients were still alive at 138 and 167 months. We found no significant relationship between type of chemotherapy and time to treatment failure or overall survival. Conclusions MPNSTs are highly aggressive in NF1. Conventional chemotherapy does not seem to reduce mortality, and its role must be questioned. Recent advances in the molecular biology of MPNSTs may provide new prognostic factors and targeted therapies. PMID:23972085

  12. Combinatorial therapy with tamoxifen and trifluoperazine effectively inhibits malignant peripheral nerve sheath tumor growth by targeting complementary signaling cascades.

    PubMed

    Brosius, Stephanie N; Turk, Amy N; Byer, Stephanie J; Longo, Jody Fromm; Kappes, John C; Roth, Kevin A; Carroll, Steven L

    2014-11-01

    Chemotherapeutic agents effective against malignant peripheral nerve sheath tumors (MPNSTs) are urgently needed. We recently found that tamoxifen potently impedes xenograft growth. In vitro, tamoxifen inhibits MPNST proliferation and survival in an estrogen receptor-independent manner; these effects are phenocopied by the calmodulin inhibitor trifluoperazine. The present study was performed to establish the mechanism of action of tamoxifen in vivo and optimize its therapeutic effectiveness. To determine if tamoxifen has estrogen receptor-dependent effects in vivo, we grafted MPNST cells in castrated and ovariectomized mice; xenograft growth was unaffected by reductions in sex hormones. To establish whether tamoxifen and trifluoperazine additively or synergistically impede MPNST growth, mice xenografted with neurofibromatosis type 1-associated or sporadic MPNST cells were treated with tamoxifen, trifluoperazine, or both drugs for 30 days. Both monotherapies inhibited graft growth by 50%, whereas combinatorial treatment maximally reduced graft mass by 90% and enhanced decreases in proliferation and survival. Kinomic analyses showed that tamoxifen and trifluoperazine have both shared and distinct targets in MPNSTs. In addition, trifluoperazine prevented tamoxifen-induced increases in serum/glucocorticoid regulated kinase 1, a protein linked to tamoxifen resistance. These findings suggest that combinatorial therapy with tamoxifen and trifluoperazine is effective against MPNSTs because these agents target complementary pathways that are essential for MPNST pathogenesis.

  13. Malignant peripheral nerve sheath tumor (MPNST) in the spine: a retrospective analysis of clinical and molecular prognostic factors.

    PubMed

    Wang, Ting; Yin, Huabin; Han, Shuai; Yang, Xinhai; Wang, Jing; Huang, Quan; Yan, Wangjun; Zhou, Wang; Xiao, Jianru

    2015-04-01

    Spinal malignant peripheral nerve sheath tumors (MPNSTs) are relatively rare. There is little information published in the literature regarding this subject. The aim of this retrospective study was to evaluate factors that may affect the outcomes of patients with spinal MPNSTs by reviewing 43 patients with spinal MPNST who were treated in our hospital between 2001 and 2012. Univariate and multivariate analyses were performed to identify prognostic variables relative to patient and tumor characteristics, treatment modality and molecules. All 43 MPNST patients (25 men and 18 women; median age 49 years) underwent surgical resection, of whom 15 patients also underwent postoperative radiotherapy. Local recurrence was found in 21 (48.8 %) patients. Twenty-two (51.2 %) patients died during the follow-up periods with a median survival time of 49 months. The 5-year recurrence and survival rate was 53 and 44 % respectively. The statistical analyses suggested that high-grade malignancy and osteolytic destruction were closely associated with recurrence and death. A total of 38 cases accepted postoperative immunohistochemisty examine. S-100 was identified as an independent factor related to both recurrence and survival, adjusting for clinical factors. In conclusion, we confirmed that malignant grade and osteolytic destruction were the two independent factors for both recurrence and survival, while patients with S-100 protein negative had a higher recurrence rate and a lower survival rate.

  14. [Case of abdominal wall malignant peripheral nerve sheath tumor which is difficult to distinguish from a urachal disease].

    PubMed

    Tatenuma, Tomoyuki; Sakata, Ryoko; Sugiura, Shinpei; Tajiri, Takehiro; Gondo, Toshikazu; Kitami, Kazuo

    2013-09-01

    Malignant peripheral nerve sheath tumors (MPNST) are highly malignant soft tissue sarcomas. It is very rare for MPNST to arise in the abdominal wall. We report a case of abdominal wall MPNST that was difficult to distinguish from a urachal disease. A 72-year-old woman found a mass of the umbilicus in October 2011. She visited a digestive surgery department in November because it gradually enlarged. Diagnostic imaging suggested a urachal tumor. She was then referred to our clinic. Contrast enhanced CT showed that the 5-cm cystic tumor extended from the umbilicus to abdominal wall. The tumor showed low uptake value in PET-CT. We diagnosed her with a urachal cyst, but could not deny urachal carcinoma. Therefore, we performed surgical resection in January 2012. The pathological diagnosis was MPNST. She has not experienced recurrence for 9 months. MPNST mostly occur in the retroperitoneum close to the spine, extremities, head, and neck. It is very rare for them to occur in the abdominal wall. This is the sixth case including overseas reports. In addition, this is the first case in which it was difficult to distinguish from a urachal disease.

  15. Pediatric and adult malignant peripheral nerve sheath tumors: an analysis of data from the surveillance, epidemiology, and end results program.

    PubMed

    Amirian, E Susan; Goodman, J Clay; New, Pamela; Scheurer, Michael E

    2014-02-01

    Malignant peripheral nerve sheath tumors (MPNSTs) are rare soft tissue sarcomas that arise predominantly from Schwann cells. Despite the fact that MPNSTs have high local recurrence rates and are generally associated with poor prognosis, little is known about prognostic factors or effective clinical management for this tumor type. The purpose of this study was to describe the distributions of patient and tumor characteristics and to identify predictors of cause-specific survival among MPNST cases reported to SEER between 1973 and 2008. Patient and tumor characteristics were compared between pediatric and adult MPNST cases. Cox regression and tree-based survival analysis were used to examine factors associated with MPNST-related mortality separately among adults and children. A total of 1,315 MPNST cases were isolated from the 1973-2008 SEER dataset. Among pediatric cases, sex, race, and radiation therapy predicted MPNST survival, whereas among adults, tumor site, tumor grade, number of primary tumors, and tumor size were significant predictors. As tumor size at diagnosis/resection may be the only somewhat "modifiable" prognostic factor, future studies should aim to identify biological and social attributes associated with tumor size at diagnosis, separately among individuals with and without NF-1, in order to help identify earlier opportunities for clinical intervention.

  16. Involvement of PACAP/ADNP signaling in the resistance to cell death in malignant peripheral nerve sheath tumor (MPNST) cells.

    PubMed

    Castorina, Alessandro; Giunta, Salvatore; Scuderi, Soraya; D'Agata, Velia

    2012-11-01

    Malignant peripheral nerve sheath tumors (MPNSTs) are sarcomas able to grow under conditions of metabolic stress caused by insufficient nutrients or oxygen. Both pituitary adenylate cyclase-activating polypeptide (PACAP) and activity-dependent neuroprotective protein (ADNP) have glioprotective potential. However, whether PACAP/ADNP signaling is involved in the resistance to cell death in MPNST cells remains to be clarified. Here, we investigated the involvement of this signaling system in the survival response of MPNST cells against hydrogen peroxide (H(2)O(2))-evoked death both in the presence of normal serum (NS) and in serum-starved (SS) cells. Results showed that ADNP levels increased time-dependently (6-48 h) in SS cells. Treatment with PACAP38 (10(-9) to 10(-5) M) dose-dependently increased ADNP levels in NS but not in SS cells. PAC(1)/VPAC receptor antagonists completely suppressed PACAP-stimulated ADNP increase and partially reduced ADNP expression in SS cells. NS-cultured cells exposed to H(2)O(2) showed significantly reduced cell viability (~50 %), increased p53 and caspase-3, and DNA fragmentation, without affecting ADNP expression. Serum starvation significantly reduced H(2)O(2)-induced detrimental effects in MPNST cells, which were not further ameliorated by PACAP38. Altogether, these finding provide evidence for the involvement of an endogenous PACAP-mediated ADNP signaling system that increases MPNST cell resistance to H(2)O(2)-induced death upon serum starvation.

  17. Preclinical evaluation of the combination of mTOR and proteasome inhibitors with radiotherapy in malignant peripheral nerve sheath tumors.

    PubMed

    Yamashita, A S; Baia, G S; Ho, J S Y; Velarde, E; Wong, J; Gallia, G L; Belzberg, A J; Kimura, E T; Riggins, G J

    2014-05-01

    About one half of malignant peripheral nerve sheath tumors (MPNST) have Neurofibromin 1 (NF1) mutations. NF1 is a tumor suppressor gene essential for negative regulation of RAS signaling. Survival for MPNST patients is poor and we sought to identify an effective combination therapy. Starting with the mTOR inhibitors rapamycin and everolimus, we screened for synergy in 542 FDA approved compounds using MPNST cells with a native NF1 loss in both alleles. We further analyzed the cell cycle and signal transduction. In vivo growth effects of the drug combination with local radiation therapy (RT) were assessed in MPNST xenografts. The synergistic combination of mTOR inhibitors with bortezomib yielded a reduction in MPNST cell proliferation. The combination of mTOR inhibitors and bortezomib also enhanced the anti-proliferative effect of radiation in vitro. In vivo, the combination of mTOR inhibitor (everolimus) and bortezomib with RT decreased tumor growth and proliferation, and augmented apoptosis. The combination of approved mTOR and proteasome inhibitors with radiation showed a significant reduction of tumor growth in an animal model and should be investigated and optimized further for MPNST therapy.

  18. Malignant Triton Tumor (Malignant Peripheral Nerve Sheath Tumor With Rhabdomyoblastic Differentiation) Occurring in a Vascularized Free Flap Reconstruction Graft.

    PubMed

    Ram, Roopa; Gardner, Jerad; Alapati, Sindhura; Jambhekar, Kedar; Pandey, Tarun; Montgomery, Corey; Nicholas, Richard

    2017-08-01

    Malignant peripheral nerve sheath tumor (MPNST) is a rare form of sarcoma arising from Schwann cells or pluripotent cells of the neural crest. Malignant triton tumor (MTT) is a subtype of MPNST with a component of malignant rhabdomyoblasts in addition to malignant Schwann cells. MPNST and MTT are both aggressive malignancies that most commonly arise from large deep neurofibromas in patients with neurofibromatosis type 1 (NF-1). However, sporadic non-NF-1 cases of MTT have also been reported in the literature. We present a case of a 50-year-old African American male with no stigmata of NF-1 who developed a large mass in a free flap on the right calf. The free flap had been placed by plastic surgery 15 years previously following open right tibial and fibular fractures associated with a large soft tissue defect. Biopsy of the enlarging mass, followed by wide surgical excision, confirmed the pathology to be MTT. Although the development of a high-grade soft tissue sarcoma has been previously reported as a late complication of radiation therapy following free flap reconstructions, we believe this is the first reported case of sarcomatous transformation arising in a vascularized, free muscle transfer.

  19. KANK1 inhibits cell growth by inducing apoptosis though regulating CXXC5 in human malignant peripheral nerve sheath tumors

    PubMed Central

    Cui, Zhibin; Shen, Yingjia; Chen, Kenny H.; Mittal, Suresh K.; Yang, Jer-Yen; Zhang, GuangJun

    2017-01-01

    Malignant peripheral nerve sheath tumors (MPNSTs) are a type of rare sarcomas with a poor prognosis due to its highly invasive nature and limited treatment options. Currently there is no targeted-cancer therapy for this type of malignancy. Thus, it is important to identify more cancer driver genes that may serve as targets of cancer therapy. Through comparative oncogenomics, we have found that KANK1 was a candidate tumor suppressor gene (TSG) for human MPNSTs. Although KANK1 is known as a cytoskeleton regulator, its tumorigenic function in MPNSTs remains largely unknown. In this study, we report that restoration of KANK1 in human MPNST cells inhibits cell growth both in human cell culture and xenograft mice by increasing apoptosis. Consistently, knockdown of KANK1 in neurofibroma cells promoted cell growth. Using RNA-seq analysis, we identified CXXC5 and other apoptosis-related genes, and demonstrated that CXXC5 is regulated by KANK1. Knockdown of CXXC5 was found to diminish KANK1-induced apoptosis in MPNST cells. Thus, KANK1 inhibits MPNST cell growth though CXXC5 mediated apoptosis. Our results suggest that KANK1 may function as a tumor suppressor in human MPNSTs, and thus it may be useful for targeted therapy. PMID:28067315

  20. Photothermal therapy improves the efficacy of a MEK inhibitor in neurofibromatosis type 1-associated malignant peripheral nerve sheath tumors

    NASA Astrophysics Data System (ADS)

    Sweeney, Elizabeth E.; Burga, Rachel A.; Li, Chaoyang; Zhu, Yuan; Fernandes, Rohan

    2016-11-01

    Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive tumors with low survival rates and the leading cause of death in neurofibromatosis type 1 (NF1) patients under 40 years old. Surgical resection is the standard of care for MPNSTs, but is often incomplete and can generate loss of function, necessitating the development of novel treatment methods for this patient population. Here, we describe a novel combination therapy comprising MEK inhibition and nanoparticle-based photothermal therapy (PTT) for MPNSTs. MEK inhibitors block activity driven by Ras, an oncogene constitutively activated in NF1-associated MPNSTs, while PTT serves as a minimally invasive method to ablate cancer cells. Our rationale for combining these seemingly disparate techniques for MPNSTs is based on several reports demonstrating the efficacy of systemic chemotherapy with local PTT. We combine the MEK inhibitor, PD-0325901 (PD901), with Prussian blue nanoparticles (PBNPs) as PTT agents, to block MEK activity and simultaneously ablate MPNSTs. Our data demonstrate the synergistic effect of combining PD901 with PBNP-based PTT, which converge through the Ras pathway to generate apoptosis, necrosis, and decreased proliferation, thereby mitigating tumor growth and increasing survival of MPNST-bearing animals. Our results suggest the potential of this novel local-systemic combination “nanochemotherapy” for treating patients with MPNSTs.

  1. Primary Intraosseous Malignant Peripheral Nerve Sheath Tumor of the Medial Cuneiform: A Case Report and Review of the Literature.

    PubMed

    Muthusamy, Saravanaraja; Conway, Sheila A; Pitcher, J David; Temple, H Thomas

    Peripheral nerve sheath tumors (benign and malignant) usually arise in the soft tissues and are unusual in bone. Intraosseous peripheral nerve sheath tumors are usually benign and constitute approximately 0.2% of all bone tumors. Intraosseous malignant peripheral nerve sheath tumors (MPNSTs) are uncommon and usually result from secondary invasion. Only a few cases of primary intraosseous MPNSTs have been reported in published studies, and these were localized mostly in the mandible (approximately 50%) or maxilla, spine, and, occasionally, in the appendicular skeleton. To the best of our knowledge, we report the first case of primary intraosseous MPNST involving a midtarsal bone (medial cuneiform). The patient was a 62-year-old female who presented with pain and tenderness but without swelling. Imaging revealed nonspecific findings, and the preoperative computed tomography-guided biopsy findings were consistent with MPNST. The patient was treated with neoadjuvant radiotherapy, followed by wide local excision and allograft reconstruction. At the final follow-up examination (24 months), the graft had been incorporated without evidence of local recurrence or distant disease. The patient with primary intraosseous MPNST of the medial cuneiform described in the present report presented with nonspecific clinical and radiologic findings. Thus, a high index of suspicion and histopathologic examination, including immunohistochemistry, are necessary for an accurate diagnosis.

  2. Order-disorder phenomena in myelinated nerve sheaths: V. Effects of temperature on rat sciatic and optic nerves, and structural differences between the two types of nerve.

    PubMed

    Mateu, L; Luzzati, V; Vonasek, E; Mateu, E; Villegas, G M; Vargas, R

    1995-01-13

    We describe in this work X-ray scattering and electron microscope studies of rat sciatic and optic nerves as a function of temperature. The scattering experiments were analyzed as described in the previous papers of this series: a variety of parameters were determined, some of which characterize the lattice disorder, others the structure of the motif. The main results are the following. All the parameters determined by the X-ray scattering study vary with temperature and the temperature-dependence is specific for the type of nerve (sciatic or optic). Most of the disorder-related parameters display a minimum or a maximum in the vicinity of physiological temperature (38 degrees C in rat); this observation, strongly supported by the electron microscope study, shows that the degree of organization of myelin is highest near physiological temperature. The structure of the motif, as revealed by the electron density profile, is fairly different in the two types of nerves (in contrast with the assumption made by previous workers); the structure also varies with temperature and the temperature-induced alterations are nerve-type specific. In the two types of nerve the thickness of the lipid bilayer varies with temperature as expected for a lipid-containing system with hydrocarbon chains in the disordered conformation. In sciatic nerve the thickness of the (thinner) cytoplasmic polar layer, which is also the layer most affected by lattice disorder in this type of nerve, decreases dramatically with increasing temperature. In optic nerve, in which lattice disorder predominantly affects the extracellular layer, the thickness of both the cytoplasmic and the extracellular layer is barely affected by temperature.

  3. [Diagnostic difficulties in the laryngeal malignant peripheral nerve sheath tumor (MPNST)].

    PubMed

    Pabiszczak, Maciej; Woźniak, Aldona; Wierzbicka, Małgorzata; Leszczyńska, Małgorzata; Szyfter, Witold

    2004-01-01

    The malignant tumor deriving from the peripheral nerve sheet, previously described as malignant Schwannoma or neurosarcoma is extremely rare as malignancy localized in the larynx. The half of cases has been developing on the basis of neurofibromatosis in von Recklinghausen disease type I or seldom, type II. The high grade of malignancy end tendency to reccurences and distant metastases is typical for this tumors. The case of 64 year old man with larynx neurosarcoma was presented. The diagnostic difficulties were caused by clinical presentation of the smooth tumor covered by unchanged mucosa and typical histological features of the tumor. The final histological assessment was complemented by positive immunohistochemical reaction (antigens against protein S-100, NSE and PG 9.5).

  4. [Multiple meningiomas].

    PubMed

    Terrier, L-M; François, P

    2016-06-01

    Multiple meningiomas (MMs) or meningiomatosis are defined by the presence of at least 2 lesions that appear simultaneously or not, at different intracranial locations, without the association of neurofibromatosis. They present 1-9 % of meningiomas with a female predominance. The occurrence of multiple meningiomas is not clear. There are 2 main hypotheses for their development, one that supports the independent evolution of these tumors and the other, completely opposite, that suggests the propagation of tumor cells of a unique clone transformation, through cerebrospinal fluid. NF2 gene mutation is an important intrinsic risk factor in the etiology of multiple meningiomas and some exogenous risk factors have been suspected but only ionizing radiation exposure has been proven. These tumors can grow anywhere in the skull but they are more frequently observed in supratentorial locations. Their histologic types are similar to unique meningiomas of psammomatous, fibroblastic, meningothelial or transitional type and in most cases are benign tumors. The prognosis of these tumors is eventually good and does not differ from the unique tumors except for the cases of radiation-induced multiple meningiomas, in the context of NF2 or when diagnosed in children where the outcome is less favorable. Each meningioma lesion should be dealt with individually and their multiple character should not justify their resection at all costs.

  5. Modelling the effects of electric fields on nerve fibres: influence of the myelin sheath.

    PubMed

    Richardson, A G; McIntyre, C C; Grill, W M

    2000-07-01

    The excitation and conduction properties of computer-based cable models of mammalian motor nerve fibres, incorporating three different myelin representations, are compared. The three myelin representations are a perfectly insulating single cable (model A), a finite impedance single cable (model B) and a finite impedance double cable (model C). Extracellular stimulation of the three models is used to study their strength-duration and current-distance (I-X) relationships, conduction velocity (CV) and action potential shape. All three models have a chronaxie time that is within the experimental range. Models B and C have increased threshold currents compared with model A, but each model has slope to the I-X relationship that matches experimental results. Model B has a CV that matches experimental data, whereas the CV of models A and C are above and below the experimental range, respectively. Model C is able to produce a depolarising afterpotential (DAP), whereas models A and B exhibit hyperpolarising afterpotentials. Models A and B are determined to be the preferred models when low-frequency stimulation (< approximately 25 Hz) is used, owing to their efficiency and accurate excitation and conduction properties. For high frequency stimulation (approximately 25 Hz and greater), model C, with its ability to produce a DAP, is necessary accurately to simulate excitation behaviour.

  6. Optimal optic nerve sheath diameter threshold for the identification of elevated opening pressure on lumbar puncture in a Chinese population.

    PubMed

    Wang, Lijuan; Feng, Liangshu; Yao, Yan; Wang, Yuzhi; Chen, Ying; Feng, Jiachun; Xing, Yingqi

    2015-01-01

    Ultrasonography of the optic nerve sheath diameter (ONSD) is a non-invasive and rapid method that might be helpful in the identification of increased intracranial pressure (ICP). The use of an ONSD greater than 5 mm on ultrasound as an indicator of increased ICP in a Caucasian population has been studied. However, the cut-off point of this predictor in Chinese patients has not been established. Thus, we conducted this study to identify the ONSD criterion for the detection of elevated opening pressure on lumbar puncture (LP) in a Chinese population and to investigate the influencing factors. This study was a blind cross-sectional study. Patients who presented with suspected increased ICP were included. The opening pressure on LP of each participant was confirmed. We analyzed the clinical differences between the groups of patients with abnormal and normal opening pressures on LP. A receiver operating characteristic curve was constructed to determine the ONSD cut-off point for the identification of abnormal opening pressure on LP. In total, 279 patients were recruited, and 101 patients presented with elevated opening pressure on LP. ONSD was a significant independent predictor of elevated opening pressure on LP (p<0.001). However, no statistical significance was observed regarding the factors that might have affected this relationship including gender, age, body mass index, waistline, head circumference, hypertension and pathological subtype. The ONSD cut-off point for the identification of elevated opening pressure on LP was 4.1 mm; this cut-off yielded a sensitivity of 95% and a specificity of 92%. ONSD is a strong and accurate predictor of elevated opening pressure on LP. The cut-off point of this predictor in a Chinese population was remarkably lower than that found in a Caucasian population. Thus, ethnic differences should be noted when using the ONSD as an indicator of increased ICP.

  7. Doxycycline potentiates antitumor effect of 5-aminolevulinic acid-mediated photodynamic therapy in malignant peripheral nerve sheath tumor cells

    PubMed Central

    Lee, Ming-Jen; Hung, Shih-Hsuan; Huang, Mu-Ching; Tsai, Tsuimin

    2017-01-01

    Neurofibromatosis type 1 (NF1) is one of the most common neurocutaneous disorders. Some NF1 patients develop benign large plexiform neurofibroma(s) at birth, which can then transform into a malignant peripheral nerve sheath tumor (MPNST). There is no curative treatment for this rapidly progressive and easily metastatic neurofibrosarcoma. Photodynamic therapy (PDT) has been developed as an anti-cancer treatment, and 5-aminolevulinic (ALA) mediated PDT (ALA-PDT) has been used to treat cutaneous skin and oral neoplasms. Doxycycline, a tetracycline derivative, can substantially reduce the tumor burden in human and animal models, in addition to its antimicrobial effects. The purpose of this study was to evaluate the effect and to investigate the mechanism of action of combined doxycycline and ALA-PDT treatment of MPNST cells. An 3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide (MTT) assay showed that the combination of ALA-PDT and doxycycline significantly reduce MPNST survival rate, compared to cells treated with each therapy alone. Isobologram analysis showed that the combined treatment had a synergistic effect. The increased cytotoxic activity could be seen by an increase in cellular protoporphyrin IX (PpIX) accumulation. Furthermore, we found that the higher retention of PpIX was mainly due to increasing ALA uptake, rather than activity changes of the enzymes porphobilinogen deaminase and ferrochelatase. The combined treatment inhibited tumor growth in different tumor cell lines, but not in normal human Schwann cells or fibroblasts. Similarly, a synergistic interaction was also found in cells treated with ALA-PDT combined with minocycline, but not tetracycline. In summary, doxycycline can potentiate the effect of ALA-PDT to kill tumor cells. This increased potency allows for a dose reduction of doxycycline and photodynamic radiation, reducing the occurrence of toxic side effects in vivo. PMID:28558025

  8. Malignant peripheral nerve sheath tumor (MPNST) arising in diffuse-type neurofibroma: clinicopathologic characterization in a series of 9 cases.

    PubMed

    Schaefer, Inga-Marie; Fletcher, Christopher D M

    2015-09-01

    Diffuse-type neurofibroma, an uncommon variant of neurofibroma, is associated with neurofibromatosis type 1 in ∼60% of cases. Typically presenting in young adults as ill-defined plaque-like dermal/subcutaneous thickening, most cases are located on the trunk or the head and neck region. Malignant transformation is extremely rare. Nine cases of malignant peripheral nerve sheath tumor (MPNST) arising in diffuse-type neurofibroma (identified in consult files) are described, including clinicopathologic features and follow-up. Five patients were male and 4 female, aged 31 to 59 years (median 49 y). All diffuse-type neurofibromas contained Meissner corpuscles, with tumor sizes ranging between 3.6 and 45 cm (median, 7.4 cm). Five patients had a clinical history of neurofibromatosis type 1, and 1 had Klippel-Trénaunay-Weber syndrome. Six tumors arose on the trunk and 1 each on the leg, arm, and scalp. Increased cellularity, nuclear atypia, and mitoses (range, 1 to 63/50 high-power fields) indicated transition to MPNST, classified as low grade in 5, intermediate to high grade in 1, and high grade in 3 cases, 1 of which exhibited heterologous angiosarcomatous differentiation. S-100 expression was quite strong and diffuse in the neurofibroma components and less extensive or weaker in MPNST. Follow-up, available for all patients (median, 80.5 mo, except 1 recent case), revealed that 1 patient developed local recurrence after 9 months; 1 with metastases at the time of initial diagnosis died 1 month after tumor resection. All other patients were alive without evidence of disease at 15 to 145 months (median, 83 mo). Diffuse-type neurofibroma may show transformation to MPNST in very rare instances. It is important to be aware of possible malignant change, requiring thorough sampling of resection specimens and long-term clinical follow-up of patients with unexcised lesions.

  9. Extradural en-plaque spinal meningioma with intraneural invasion.

    PubMed

    Tuli, Jayshree; Drzymalski, Dan Michael; Lidov, Hart; Tuli, Sagun

    2012-01-01

    Extradural spinal meningiomas are rare. Our understanding of purely extradural spinal meningiomas is incomplete because most reports rarely differentiate purely extradural meningiomas from extradural meningiomas with an intradural component. Occasionally, reports have described involvement of the adjacent nerve root, but there has never been a description of an extradural meningioma that actually infiltrates the nerve root. A 42-year-old woman presented with progressive lower extremity weakness and numbness below T3 during the span of 4 months with imaging evidence of an extradural lesion compressing the cord from T4 through T6. Surgical resection revealed an extradural mass extending through the foramen at T5-6 and encompassing the cord and T5 root on the left. Pathologically, the lesion was a World Health Organization grade I meningioma with nerve root invasion and a concerning elevated mindbomb homolog 1 (MIB-1) of 9.4%. Purely extradural meningiomas are rare, and our case is one of the first to describe a patient with an extradural meningioma that actually infiltrates the nerve root. Extradural spinal meningiomas are usually not adherent to the dura, but only appear to be adherent or invade (as in our patient) the adjacent nerve root. They are easily mistaken preoperatively and grossly intraoperatively for malignant metastatic tumors and can change the proposed surgical treatment. The long-term prognosis remains uncertain, but our patient's last follow-up suggests a favorable prognosis. Copyright © 2012 Elsevier Inc. All rights reserved.

  10. [Ptosis secondary to cavernous sinus meningioma].

    PubMed

    Louis, M; Goga, D; François, P; Laure, B

    2013-12-01

    Meningiomas of the cavernous sinus are often the cause of neuro-ophthalmologic manifestations. Fifty percent of affected patients present with ptosis. We report a case of ptosis acquired during the first year of life due to oculomotor nerve palsy secondary to a cavernous sinus meningioma. We then discuss the causes of third cranial nerve palsy and treatment options for ptosis associated with CN III palsy. A fifteen-year-old female patient presented with ptosis due to a third cranial nerve palsy appearing within the first year of life. Magnetic resonance imaging (MRI) revealed a cavernous sinus meningioma. The ptosis was treated by frontalis suspension using autologous temporalis fascia. The meningioma required regular follow-up. Ptosis due to third cranial nerve palsy is rare in children. The most common etiologies are congenital and represent 33 to 40% of cases in various studies. Other etiologies are traumatic, tumoral, vascular and infectious. The cause needs to be found by imaging over the entire course of the nerve. Cavernous sinus meningioma is one cause of third cranial nerve palsy. The surgical treatment of ptosis due to third cranial nerve palsy is levator resection or frontalis suspension with a strip of fascia lata or temporalis fascia. Copyright © 2013. Published by Elsevier Masson SAS.

  11. Dual mTORC1/2 inhibition induces anti-proliferative effect in NF1-associated plexiform neurofibroma and malignant peripheral nerve sheath tumor cells.

    PubMed

    Varin, Jennifer; Poulain, Laury; Hivelin, Mikael; Nusbaum, Patrick; Hubas, Arnaud; Laurendeau, Ingrid; Lantieri, Laurent; Wolkenstein, Pierre; Vidaud, Michel; Pasmant, Eric; Chapuis, Nicolas; Parfait, Béatrice

    2016-06-14

    Approximately 30-50% of individuals with Neurofibromatosis type 1 develop benign peripheral nerve sheath tumors, called plexiform neurofibromas (PNFs). PNFs can undergo malignant transformation to highly metastatic malignant peripheral nerve sheath tumors (MPNSTs) in 5-10% of NF1 patients, with poor prognosis. No effective systemic therapy is currently available for unresectable tumors. In tumors, the NF1 gene deficiency leads to Ras hyperactivation causing the subsequent activation of the AKT/mTOR and Raf/MEK/ERK pathways and inducing multiple cellular responses including cell proliferation. In this study, three NF1-null MPNST-derived cell lines (90-8, 88-14 and 96-2), STS26T sporadic MPNST cell line and PNF-derived primary Schwann cells were used to test responses to AZD8055, an ATP-competitive "active-site" mTOR inhibitor. In contrast to rapamycin treatment which only partially affected mTORC1 signaling, AZD8055 induced a strong inhibition of mTORC1 and mTORC2 signaling in MPNST-derived cell lines and PNF-derived Schwann cells. AZD8055 induced full blockade of mTORC1 leading to an efficient decrease of global protein synthesis. A higher cytotoxic effect was observed with AZD8055 compared to rapamycin in the NF1-null MPNST-derived cell lines with IC50 ranging from 70 to 140 nM and antiproliferative effect was confirmed in PNF-derived Schwann cells. Cell migration was impaired by AZD8055 treatment and cell cycle analysis showed a G0/G1 arrest. Combined effects of AZD8055 and PD0325901 MEK inhibitor as well as BRD4 (BromoDomain-containing protein 4) inhibitors showed a synergistic antiproliferative effect. These data suggest that NF1-associated peripheral nerve sheath tumors are an ideal target for AZD8055 as a single molecule or in combined therapies.

  12. Dual mTORC1/2 inhibition induces anti-proliferative effect in NF1-associated plexiform neurofibroma and malignant peripheral nerve sheath tumor cells

    PubMed Central

    Hivelin, Mikael; Nusbaum, Patrick; Hubas, Arnaud; Laurendeau, Ingrid; Lantieri, Laurent; Wolkenstein, Pierre; Vidaud, Michel; Pasmant, Eric; Chapuis, Nicolas; Parfait, Béatrice

    2016-01-01

    Approximately 30-50% of individuals with Neurofibromatosis type 1 develop benign peripheral nerve sheath tumors, called plexiform neurofibromas (PNFs). PNFs can undergo malignant transformation to highly metastatic malignant peripheral nerve sheath tumors (MPNSTs) in 5-10% of NF1 patients, with poor prognosis. No effective systemic therapy is currently available for unresectable tumors. In tumors, the NF1 gene deficiency leads to Ras hyperactivation causing the subsequent activation of the AKT/mTOR and Raf/MEK/ERK pathways and inducing multiple cellular responses including cell proliferation. In this study, three NF1-null MPNST-derived cell lines (90-8, 88-14 and 96-2), STS26T sporadic MPNST cell line and PNF-derived primary Schwann cells were used to test responses to AZD8055, an ATP-competitive “active-site” mTOR inhibitor. In contrast to rapamycin treatment which only partially affected mTORC1 signaling, AZD8055 induced a strong inhibition of mTORC1 and mTORC2 signaling in MPNST-derived cell lines and PNF-derived Schwann cells. AZD8055 induced full blockade of mTORC1 leading to an efficient decrease of global protein synthesis. A higher cytotoxic effect was observed with AZD8055 compared to rapamycin in the NF1-null MPNST-derived cell lines with IC50 ranging from 70 to 140 nM and antiproliferative effect was confirmed in PNF-derived Schwann cells. Cell migration was impaired by AZD8055 treatment and cell cycle analysis showed a G0/G1 arrest. Combined effects of AZD8055 and PD0325901 MEK inhibitor as well as BRD4 (BromoDomain-containing protein 4) inhibitors showed a synergistic antiproliferative effect. These data suggest that NF1-associated peripheral nerve sheath tumors are an ideal target for AZD8055 as a single molecule or in combined therapies. PMID:26840085

  13. Optic nerve sheath diameter sonography for the diagnosis of increased intracranial pressure: a systematic review and meta-analysis protocol.

    PubMed

    Koziarz, Alex; Sne, Niv; Kegel, Fraser; Alhazzani, Waleed; Nath, Siddharth; Badhiwala, Jetan H; Rice, Timothy; Engels, Paul; Samir, Faidi; Healey, Andrew; Kahnamoui, Kamyar; Banfield, Laura; Sharma, Sunjay; Reddy, Kesava; Hawryluk, Gregory W J; Kirkpatrick, Andrew W; Almenawer, Saleh A

    2017-08-11

    Increased intracranial pressure (ICP) is a significant neurological issue that may lead to permanent neurological sequelae. When evaluating patients with traumatic brain injury, it is crucial to identify those with high ICP in order to expedite ICP lowering measures and maintain adequate cerebral perfusion. Several measures are used to recognise patients with increased ICP including CT scan, MRI, ICP monitor, and lumbar puncture (LP). However, these tests can be invasive, associated with radiation exposure, contraindicated, or not readily available. Ultrasonography measurement of the optic nerve sheath diameter (ONSD) is proposed as a non-invasive and quick measure to identify high ICP. The aim of this systematic review and meta-analysis will be to examine the accuracy of ONSD sonography for increased ICP diagnosis. We will include published and unpublished randomised controlled trials, observational studies, and abstracts, with no publication type or language restrictions. Search strategies will be designed to peruse the MEDLINE, Embase, Web of Science, WHO Clinical Trials, ClinicalTrials.gov, CINAHL, and the Cochrane Library databases. We will also implement strategies to search grey literature. Two reviewers will independently complete data abstraction and conduct quality assessment. Included studies will be assessed using the Quality Assessment of Diagnostic Accuracy Studies-2 (QUADAS-2) tool. We will construct the hierarchical summary receiver operating characteristic curve for included studies and pool sensitivity and specificity using the bivariate model. We also plan to conduct prespecified subgroup analyses to explore heterogeneity. The overall quality of evidence will be rated using Grading of Recommendations, Assessment, Development and Evaluations (GRADE). Research ethics board approval is not required for this study as it draws from published data and raises no concerns related to patient privacy. This review will provide a comprehensive assessment of

  14. Grading Meningioma

    PubMed Central

    Okuchi, Sachi; Okada, Tomohisa; Yamamoto, Akira; Kanagaki, Mitsunori; Fushimi, Yasutaka; Okada, Tsutomu; Yamauchi, Moritaka; Kataoka, Masako; Arakawa, Yoshiki; Takahashi, Jun C.; Minamiguchi, Sachiko; Miyamoto, Susumu; Togashi, Kaori

    2015-01-01

    Abstract The purpose was to compare capability of fluorine-18 fluorodeoxyglucose (FDG)-PET and thallium-201 (Tl)-SPECT for grading meningioma. This retrospective study was conducted as a case-control study under approval by the institutional review board. In the hospital information system, 67 patients (22 men and 45 women) who had both FDG-PET and Tl-SPECT preoperative examinations were found with histopathologic diagnosis of meningioma. The maximum FDG uptake values of the tumors were measured, and they were standardized to the whole body (SUVmax) and normalized as gray matter ratio (SUVRmax). Mean and maximum Tl uptake ratios (TURmean and TURmax, respectively) of the tumors were measured and normalized as ratios to those of the contralateral normal brain. Receiver-operating characteristic curve analyses of the 4 indexes were conducted for differentiation between low- and high-grade meningiomas, and areas under the curves (AUCs) were compared. Correlation coefficients were calculated between these indexes and Ki-67. Fifty-six meningiomas were classified as grade I (low grade), and 11 were grade II or III (high grade). In all 4 indexes, a significant difference was observed between low- and high-grade meningiomas (P < 0.05). AUCs were 0.817 (SUVmax), 0.781 (SUVRmax), 0.810 (TURmean), and 0.831 (TURmax), and no significant difference was observed among the indexes. Their sensitivity and specificity were 72.7% to 90.9% and 71.4% to 87.5%, respectively. Correlation of the 4 indexes to Ki-67 was statistically significant, but coefficients were relatively low (0.273–0.355). Tl-SPECT, which can be used at hospitals without a cyclotron or an FDG distribution network, has high diagnostic capability of meningioma grades comparable to FDG-PET. PMID:25674763

  15. Classic Ras Proteins Promote Proliferation and Survival Via Distinct Phosphoproteome Alterations in Neurofibromin-Null Malignant Peripheral Nerve Sheath Tumor Cells

    PubMed Central

    Brossier, Nicole M.; Prechtl, Amanda M.; Longo, Jody Fromm; Barnes, Stephen; Wilson, Landon S.; Byer, Stephanie J.; Brosius, Stephanie N.; Carroll, Steven L.

    2015-01-01

    Neurofibromin, the tumor suppressor encoded by the neurofibromatosis type 1 (NF1) gene, potentially suppresses the activation of H-Ras, N-Ras and K-Ras. However, it is not known whether these classic Ras proteins are hyperactivated in NF1-null nerve sheath tumors, how they contribute to tumorigenesis and what signaling pathways mediate their effects. Here we show that H-Ras, N-Ras and K-Ras are coexpressed with their activators, (guanine nucleotide exchange factors), in neurofibromin-null malignant peripheral nerve sheath tumor (MPNST) cells and that all 3 Ras proteins are activated. Dominant negative (DN) H-Ras, a pan-inhibitor of the classic Ras family, inhibited MPNST proliferation and survival, but not migration. However, NF1-null MPNST cells were variably dependent on individual Ras proteins. In some lines, ablation of H-Ras, N-Ras and/or K-Ras inhibited mitogenesis. In others, ablation of a single Ras protein had no effect on proliferation; in these lines, ablation of a single Ras protein resulted in compensatory increases in the activation and/or expression of other Ras proteins. Using mass spectrometry-based phosphoproteomics, we identified 7 signaling networks affecting morphology, proliferation and survival that are regulated by DN H-Ras. Thus, neurofibromin loss activates multiple classic Ras proteins that promote proliferation and survival by regulating several distinct signaling cascades. PMID:25946318

  16. Effect of Positive End-Expiratory Pressure on the Sonographic Optic Nerve Sheath Diameter as a Surrogate for Intracranial Pressure during Robot-Assisted Laparoscopic Prostatectomy: A Randomized Controlled Trial

    PubMed Central

    Chin, Ji-Hyun; Kim, Wook-Jong; Lee, Joonho; Han, Yun A.; Lim, Jinwook; Hwang, Jai-Hyun; Cho, Seong-Sik

    2017-01-01

    Background Positive end-expiratory pressure (PEEP) can increase intracranial pressure. Pneumoperitoneum and the Trendelenburg position are associated with an increased intracranial pressure. We investigated whether PEEP ventilation could additionally influence the sonographic optic nerve sheath diameter as a surrogate for intracranial pressure during pneumoperitoneum combined with the Trendelenburg position in patients undergoing robot-assisted laparoscopic prostatectomy. Methods After anesthetic induction, 38 patients were randomly allocated to a low tidal volume ventilation (8 ml/kg) without PEEP group (zero end-expiratory pressure [ZEEP] group, n = 19) or low tidal volume ventilation with 8 cmH2O PEEP group (PEEP group, n = 19). The sonographic optic nerve sheath diameter was measured prior to skin incision, 5 min and 30 min after pneumoperitoneum and the Trendelenburg position, and at the end of surgery. The study endpoint was the difference in the sonographic optic nerve sheath diameter 5 min after pneumoperitoneum and the Trendelenburg position between the ZEEP and PEEP groups. Results Optic nerve sheath diameters 5 min after pneumoperitoneum and the Trendelenburg position did not significantly differ between the groups [least square mean (95% confidence interval); 4.8 (4.6–4.9) mm vs 4.8 (4.7–5.0) mm, P = 0.618]. Optic nerve sheath diameters 30 min after pneumoperitoneum and the Trendelenburg position also did not differ between the groups [least square mean (95% confidence interval); 4.5 (4.3–4.6) mm vs 4.5 (4.4–4.6) mm, P = 0.733]. Conclusions An 8 cmH2O PEEP application under low tidal volume ventilation does not induce an increase in the optic nerve sheath diameter during pneumoperitoneum combined with the steep Trendelenburg position, suggesting that there might be no detrimental effects of PEEP on the intracranial pressure during robot-assisted laparoscopic prostatectomy. Trial Registration ClinicalTrial.gov NCT02516566 PMID:28107408

  17. Monocular nasal hemianopia from atypical sphenoid wing meningioma.

    PubMed

    Stacy, Rebecca C; Jakobiec, Frederick A; Lessell, Simmons; Cestari, Dean M

    2010-06-01

    Neurogenic monocular nasal field defects respecting the vertical midline are quite uncommon. We report a case of a unilateral nasal hemianopia that was caused by compression of the left optic nerve by a sphenoid wing meningioma. Histological examination revealed that the pathology of the meningioma was consistent with that of an atypical meningioma, which carries a guarded prognosis with increased chance of recurrence. The tumor was debulked surgically, and the patient's visual field defect improved.

  18. Nerve Injuries of the Upper Extremity

    MedlinePlus

    ... of individual nerve fibers and surrounding outer sheath (“insulation”) Figure 2: Nerve repair with realignment of bundles © ... of individual nerve fibers and surrounding outer sheath insulation Figure 2 - Nerve repair with realignment of bundles ...

  19. Convection-Enhanced Delivery (CED) in an Animal Model of Malignant Peripheral Nerve Sheath Tumors and Plexiform Neurofibromas

    DTIC Science & Technology

    2013-02-01

    sheath tumors or plexiform neurofibromas. References: Perrin GQ, Fishbein L, Thomson SA, et al., Plexiform-like neurofibromas develop in the...mouse by intraneural xenograft of an NF1 tumor-derived Schwann cell line. J Neurosci Res, 2007. 85(6): p. 1347-1357. 3 Perrin GQ, Li H, Fishbein L

  20. Clinical and imaging findings, treatments, and outcomes in 27 dogs with imaging diagnosed trigeminal nerve sheath tumors: A multi-center study.

    PubMed

    Swift, Katie E; McGrath, Stephanie; Nolan, Michael W; Young, Martin; Reese, Michael; Rao, Sangeeta; Randall, Elissa; Leary, Del; LaRue, Susan

    2017-07-31

    The clinical behavior of canine trigeminal nerve sheath tumors and benefits of previously reported treatments are incompletely defined. Aims of this retrospective, multicenter, observational study were to describe clinical signs, tumor localization characteristics, treatments, and clinical outcomes in a group of dogs with this neoplasm. Databases at four hospitals were reviewed for dogs with a trigeminal nerve sheath tumor diagnosis, magnetic resonance imaging (MRI) studies, and presentation between 2004 and 2014. A single observer recorded medical record findings and two observers recorded MRI characteristics by consensus. A total of 27 dogs met inclusion criteria (15 treated with stereotactic radiation therapy and 12 unirradiated). Two unirradiated dogs were excluded from outcome analyses. The most common presenting signs were masticatory muscle atrophy (26 dogs), neurologic signs referable to intracranial disease (13), and ocular disease (12). Based on MRI findings, all dogs had disease extending centrally at the level of the brainstem. The most commonly affected trigeminal nerve branches were the mandibular (26 dogs), maxillary (22), and ophthalmic (10). Of 15 dogs treated with stereotactic radiation therapy, one had improved muscle atrophy, and six had poor ocular health after treatment. Neurologic signs improved in 4/5 dogs with intracranial signs. Overall median survival time for the 10 unirradiated dogs with available follow-up was 12 days and 441 days for the 15 stereotactic radiation therapy dogs. Mean survival times between these groups were not significantly different (mean 95% CI for unirradiated dogs was 44-424 days and mean 95% CI for stereotactic radiation therapy dogs was 260-518 days). © 2017 American College of Veterinary Radiology.

  1. Comprehensive adipocytic and neurogenic tissue microarray analysis of NY-ESO-1 expression - a promising immunotherapy target in malignant peripheral nerve sheath tumor and liposarcoma.

    PubMed

    Shurell, Elizabeth; Vergara-Lluri, Maria E; Li, Yunfeng; Crompton, Joseph G; Singh, Arun; Bernthal, Nicholas; Wu, Hong; Eilber, Fritz C; Dry, Sarah M

    2016-11-08

    Immunotherapy targeting cancer-testis antigen NY-ESO-1 shows promise for tumors with poor response to chemoradiation. Malignant peripheral nerve sheath tumors (MPNSTs) and liposarcomas (LPS) are chemoresistant and have few effective treatment options. Materials Methods: Using a comprehensive tissue microarray (TMA) of both benign and malignant tumors in primary, recurrent, and metastatic samples, we examined NY-ESO-1 expression in peripheral nerve sheath tumor (PNST) and adipocytic tumors. The PNST TMA included 42 MPNSTs (spontaneous n = 26, NF1-associated n = 16), 35 neurofibromas (spontaneous n = 22, NF-1 associated n = 13), 11 schwannomas, and 18 normal nerves. The LPS TMA included 48 well-differentiated/dedifferentiated (WD/DD) LPS, 13 myxoid/round cell LPS, 3 pleomorphic LPS, 8 lipomas, 1 myelolipoma, and 3 normal adipocytic tissue samples. Stained in triplicate, NY-ESO-1 intensity and density were scored. NY-ESO-1 expression was exclusive to malignant tumors. 100% of myxoid/round cell LPS demonstrated NY-ESO-1 expression, while only 6% of WD/DD LPS showed protein expression, one of which was WD LPS. Of MPNST, 4/26 (15%) spontaneous and 2/16 (12%) NF1-associated MPNSTs demonstrated NY-ESO-1 expression. Strong NY-ESO-1 expression was observed in myxoid/round cell and dedifferentiated LPS, and MPNST in primary, neoadjuvant, and metastatic settings. We found higher prevalence of NY-ESO-1 expression in MPNSTs than previously reported, highlighting a subset of MPNST patients who may benefit from immunotherapy. This study expands our understanding of NY-ESO-1 in WD/DD LPS and is the first demonstration of staining in a WD LPS and metastatic/recurrent myxoid/round cell LPS. These results suggest immunotherapy targeting NY-ESO-1 may benefit patients with aggressive tumors resistant to conventional therapy.

  2. Comprehensive adipocytic and neurogenic tissue microarray analysis of NY-ESO-1 expression - a promising immunotherapy target in malignant peripheral nerve sheath tumor and liposarcoma

    PubMed Central

    Shurell, Elizabeth; Vergara-Lluri, Maria E.; Li, Yunfeng; Crompton, Joseph G.; Singh, Arun; Bernthal, Nicholas; Wu, Hong; Eilber, Fritz C.; Dry, Sarah M.

    2016-01-01

    Background Immunotherapy targeting cancer-testis antigen NY-ESO-1 shows promise for tumors with poor response to chemoradiation. Malignant peripheral nerve sheath tumors (MPNSTs) and liposarcomas (LPS) are chemoresistant and have few effective treatment options. Materials Methods Using a comprehensive tissue microarray (TMA) of both benign and malignant tumors in primary, recurrent, and metastatic samples, we examined NY-ESO-1 expression in peripheral nerve sheath tumor (PNST) and adipocytic tumors. The PNST TMA included 42 MPNSTs (spontaneous n = 26, NF1-associated n = 16), 35 neurofibromas (spontaneous n = 22, NF-1 associated n = 13), 11 schwannomas, and 18 normal nerves. The LPS TMA included 48 well-differentiated/dedifferentiated (WD/DD) LPS, 13 myxoid/round cell LPS, 3 pleomorphic LPS, 8 lipomas, 1 myelolipoma, and 3 normal adipocytic tissue samples. Stained in triplicate, NY-ESO-1 intensity and density were scored. Results NY-ESO-1 expression was exclusive to malignant tumors. 100% of myxoid/round cell LPS demonstrated NY-ESO-1 expression, while only 6% of WD/DD LPS showed protein expression, one of which was WD LPS. Of MPNST, 4/26 (15%) spontaneous and 2/16 (12%) NF1-associated MPNSTs demonstrated NY-ESO-1 expression. Strong NY-ESO-1 expression was observed in myxoid/round cell and dedifferentiated LPS, and MPNST in primary, neoadjuvant, and metastatic settings. Conclusions We found higher prevalence of NY-ESO-1 expression in MPNSTs than previously reported, highlighting a subset of MPNST patients who may benefit from immunotherapy. This study expands our understanding of NY-ESO-1 in WD/DD LPS and is the first demonstration of staining in a WD LPS and metastatic/recurrent myxoid/round cell LPS. These results suggest immunotherapy targeting NY-ESO-1 may benefit patients with aggressive tumors resistant to conventional therapy. PMID:27655679

  3. Characterizing the immune microenvironment of malignant peripheral nerve sheath tumor by PD-L1 expression and presence of CD8+ tumor infiltrating lymphocytes

    PubMed Central

    Shurell, Elizabeth; Singh, Arun S.; Crompton, Joseph G.; Jensen, Sarah; Li, Yunfeng; Dry, Sarah; Nelson, Scott; Chmielowski, Bartosz; Bernthal, Nicholas; Federman, Noah; Tumeh, Paul; Eilber, Fritz C.

    2016-01-01

    Background Malignant peripheral nerve sheath tumor (MPNST) is an aggressive sarcoma with few treatment options. Tumor immune state has not been characterized in MPNST, and is important in determining response to immune checkpoint blockade. Our aim was to evaluate the expression of programmed death-ligand 1 (PD-L1), programmed cell death protein 1 (PD-1), and presence of CD8+ tumor infiltrating lymphocytes (TILs) in MPNST, and correlate these findings with clinical behavior and outcome. Results PD-L1 staining of at least 1% was seen in 0/20 nerves, 2/68 benign lesions and 9/53 MPNST. Two of 68 benign lesions and 7/53 (13%) MPNST had at least 5% PD-L1 staining. CD8 staining of at least 5% was seen in 1/20 (5%) nerves, 45/68 (66%) benign lesions and 30/53 (57%) MPNST. PD-L1 was statistically more prevalent in MPNST than both nerves and benign lesions (p=0.049 and p=0.008, respectively). Expression of PD-1 was absent in all tissue specimens. There was no correlation of PD-L1 or CD8 expression with disease state (primary versus metastatic) or patient survival. Methods A comprehensive PNST tissue microarray was created from 141 surgical specimens including primary, recurrent, and metastatic MPNST (n=53), neurofibromas (n=57), schwannoma (n=11), and normal nerve (n=20). Cores were stained in triplicate for PD-L1, PD-1, and CD8, and expression compared between tumor types. These data were then examined for survival correlates in 35 patients with primary MPNST. Conclusions MPNST is characterized by low PD-L1 and absent PD-1 expression with significant CD8+ TIL presence. MPNST immune microenvironment does not correlate with patient outcome. PMID:27588404

  4. Primary Intraosseous Meningioma.

    PubMed

    Chen, Thomas C

    2016-04-01

    Primary intraosseous meningiomas are a subtype of primary extradural meningiomas. They represent approximately two-thirds of extradural meningiomas and fewer than 2% of meningiomas overall. These tumors originate within the bones of the skull and can have a clinical presentation and radiographic differential diagnosis different from those for intradural meningiomas. Primary intraosseous meningiomas are classified based on location and histopathologic characteristics. Treatment is primarily surgical resection with wide margins if possible. Sparse literature exists regarding the use of adjuvant therapies. The literature regarding primary intraosseous meningiomas consists primarily of clinical case reports and case series. This literature is reviewed and summarized in this article.

  5. Triterpenoid saponin flaccidoside II from Anemone flaccida triggers apoptosis of NF1-associated malignant peripheral nerve sheath tumors via the MAPK-HO-1 pathway

    PubMed Central

    Han, Lin-tao; Fang, Yin; Cao, Yan; Wu, Feng-hua; Liu, E; Mo, Guo-yan; Huang, Fang

    2016-01-01

    Malignant peripheral nerve sheath tumors (MPNSTs) are highly aggressive soft tissue neoplasms that are extremely rare and are frequently associated with neurofibromatosis type 1 patients. MPNSTs are typically fatal, and there is no effective treatment so far. In our previous study, we showed that flaccidoside II, one of the triterpenoid saponins isolated from Anemone flaccida Fr. Schmidt, has antitumor potential by inducing apoptosis. In the present study, we found that flaccidoside II inhibits proliferation and facilitates apoptosis in MPNST cell lines ST88-14 and S462. Furthermore, this study provides a mechanism by which the downregulation of heme oxygenase-1 via extracellular signal-regulated kinase-1/2 and p38 mitogen-activated protein kinase pathways is involved in the apoptotic role of flaccidoside II. This study suggested the potential of flaccidoside II as a novel pharmacotherapeutic approach for MPNSTs. PMID:27103823

  6. Successful treatment with doxorubicin and ifosfamide for mediastinal malignant peripheral nerve sheath tumor with loss of H3K27me3 expression.

    PubMed

    Seno, Noriko; Fukushima, Toshirou; Gomi, Daisuke; Kobayashi, Takashi; Sekiguchi, Nodoka; Matsushita, Hidehiro; Ozawa, Takesumi; Tsukahara, Yoshiko; Mamiya, Keiko; Koizumi, Tomonobu; Sano, Kenji

    2017-09-06

    Malignant peripheral nerve sheath tumor (MPNST) in the thorax is an extremely rare disease, and half of all cases of MPNST are associated with neurofibromatosis type I. Sporadic intrathoracic MPNST is difficult to diagnose and treat. Because of the rarity of intrathoracic MPNST, the optimal method of diagnosis and the efficacy of chemotherapy are unknown. Herein, we present a case of inoperable mediastinal MPNST, in which the diagnosis was immunohistochemically made by the loss of H3K27me3 expression in a transbronchial needle biopsy specimen. The patient showed a good response to doxorubicin plus ifosfamide chemotherapy. The present case highlights that MPNST should be included in the differential diagnosis of non-posterior mediastinum thoracic lesions, and that appropriate diagnosis and treatment for intrathoracic MPNST should be considered in patients with a thoracic mass. © 2017 The Authors. Thoracic Cancer published by China Lung Oncology Group and John Wiley & Sons Australia, Ltd.

  7. Jugular fossa meningioma: presentation and treatment options.

    PubMed

    Rutt, Amy L; Chen, Xiaoli; Sataloff, Robert T

    2009-10-01

    Primary jugular fossa meningiomas are among the rarest subtypes of meningioma. They are intimately related to the lower cranial nerves, the jugular bulb and vein, and the temporal bone, and they have a tendency to extend both intra- and extracranially. The most common morbidity associated with jugular fossa lesions is lower cranial nerve deficits. In these cases, the differential diagnosis and preoperative radiographic diagnosis are very important because preoperative management and operative planning for the jugular fossa subtype differ considerably from those of other types of meningioma. Because of the rarity of this condition, our understanding of its treatment, long-term follow-up, and recurrence is limited. As experience with radiosurgical treatment of all meningiomas is accumulating, we may find that radiosurgery of jugular fossa meningiomas is appropriate. In the meantime, cure is still possible with complete surgical resection, and surgical morbidity can be minimized through meticulous planning and surgical technique. We describe a case of primary jugular fossa meningioma in a 45-year-old man who presented with complaints of chronic left aural fullness, hearing loss, and difficulty understanding voices. Imaging revealed the presence of a destructive jugular fossa mass. The patient underwent surgical resection without complication, and he was free of recurrence at 1 year of follow-up.

  8. Overexpression of PDGFRA cooperates with loss of NF1 and p53 to accelerate the molecular pathogenesis of malignant peripheral nerve sheath tumors.

    PubMed

    Ki, D H; He, S; Rodig, S; Look, A T

    2017-02-23

    Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive, frequently metastatic sarcomas that are associated with neurofibromatosis type 1 (NF1), a prominent inherited genetic disease in humans. Although loss of the NF1 gene predisposes to MPNST induction, relatively long tumor latency in NF1 patients suggests that additional genetic or epigenetic abnormalities are needed for the development of these nerve sheath malignancies. To study the molecular pathways contributing to the formation of MPNSTs in NF1 patients, we used a zebrafish tumor model defined by nf1 loss in a p53-deficient background together with the overexpression of either wild-type or constitutively activated PDGFRA (platelet-derived growth factor receptor-α) under control of the sox10 neural crest-specific promoter. Here we demonstrate the accelerated onset and increased penetrance of MPNST formation in fish overexpressing both the wild-type and the mutant PDGFRA transgenes in cells of neural crest origin. Interestingly, overexpression of the wild-type PDGFRA was even more potent in promoting transformation than the mutant PDGFRA, which is important because ~78% of human MPNSTs have expression of wild-type PDGFRA, whereas only 5% harbor activating mutations of the gene encoding this receptor. Further analysis revealed the induction of cellular senescence in zebrafish embryos overexpressing mutant, but not wild-type, PDGFRA, suggesting a mechanism through which the oncogenic activity of the mutant receptor is tempered by the activation of premature cellular senescence in an NF1-deficient background. Taken together, our study suggests a model in which overexpression of wild-type PDGFRA associated with NF1 deficiency leads to aberrant activation of downstream RAS signaling and thus contributes importantly to MPNST development-a prediction supported by the ability of the kinase inhibitor sunitinib alone and in combination with the MEK inhibitor trametinib to retard MPNST progression in

  9. Overexpression of PDGFRA cooperates with loss of NF1 and p53 to accelerate the molecular pathogenesis of malignant peripheral nerve sheath tumors

    PubMed Central

    Ki, D H; He, S; Rodig, S; Look, A T

    2017-01-01

    Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive, frequently metastatic sarcomas that are associated with neurofibromatosis type 1 (NF1), a prominent inherited genetic disease in humans. Although loss of the NF1 gene predisposes to MPNST induction, relatively long tumor latency in NF1 patients suggests that additional genetic or epigenetic abnormalities are needed for the development of these nerve sheath malignancies. To study the molecular pathways contributing to the formation of MPNSTs in NF1 patients, we used a zebrafish tumor model defined by nf1 loss in a p53-deficient background together with the overexpression of either wild-type or constitutively activated PDGFRA (platelet-derived growth factor receptor-α) under control of the sox10 neural crest-specific promoter. Here we demonstrate the accelerated onset and increased penetrance of MPNST formation in fish overexpressing both the wild-type and the mutant PDGFRA transgenes in cells of neural crest origin. Interestingly, overexpression of the wild-type PDGFRA was even more potent in promoting transformation than the mutant PDGFRA, which is important because ~78% of human MPNSTs have expression of wild-type PDGFRA, whereas only 5% harbor activating mutations of the gene encoding this receptor. Further analysis revealed the induction of cellular senescence in zebrafish embryos overexpressing mutant, but not wild-type, PDGFRA, suggesting a mechanism through which the oncogenic activity of the mutant receptor is tempered by the activation of premature cellular senescence in an NF1-deficient background. Taken together, our study suggests a model in which overexpression of wild-type PDGFRA associated with NF1 deficiency leads to aberrant activation of downstream RAS signaling and thus contributes importantly to MPNST development—a prediction supported by the ability of the kinase inhibitor sunitinib alone and in combination with the MEK inhibitor trametinib to retard MPNST progression in

  10. Primary extracranial meningioma of the mandible.

    PubMed

    Mosqueda-Taylor, Adalberto; Domínguez-Malagon, Hugo; Cano-Valdez, Ana-Maria; Montiel-Hernandez, Ana-Maria

    2009-04-01

    Meningiomas are benign tumors of mesodermal origin that arise from arachnoid cell clusters that penetrate the dura to form arachnoid villi. These neoplasms represent one of the most common neoplasms developing within the central nervous system and are usually located at points of entry of vessels and nerves through the dura. Extracranial meningiomas (EM) comprise only 2% of all meningiomas, and only six cases of primary EM of the jawbones have been described to date. They may arise as an extension of intracranial meningiomas or as primary tumors and may be clinically indistinguishable from other benign tumours of the jaws, as they usually present as a well-delineated unencapsulated tumors. In this article a case of primary intramandibular primary EM that appeared as a well-defined osteolytic radiolucent lesion of the jaw is reported. The salient clinico-pathological features of this case is compared to those previously reported in the literature and differential diagnosis and therapeutic considerations are discussed.

  11. Meningioma involving Meckel's cave: transpetrosal surgical anatomy and clinical considerations.

    PubMed

    Choo, Daniel I; Steward, David L; Pensak, Myles L

    2002-09-01

    Meningiomas originating in Meckel's cave (MC) are uncommon lesions that represent 1% of all intracranial meningiomas. Innovations in skull base surgery have enabled resection of these lesions with less morbidity, but require an intimate knowledge of both lesional pathology and regional microneuroanatomy. To review the surgical and clinical considerations involved in the management of MC meningiomas, we retrospectively reviewed data from patients who underwent transpetrosal resection of primary MC meningiomas between 1984 and 1998. Of 146 patients who underwent transpetrosal removal of meningiomas, 7 were believed to have tumors originating in MC. All 7 patients presented with trigeminal dysfunction, facial pain, and/or headache. Complete tumor removal was achieved in 5 of the 7 patients. Facial hypoesthesia or anesthesia, paralysis of cranial nerve VI, and ophthalmoplegia were among the postoperative complications encountered. Meningiomas of MC represent treatable lesions whose diagnosis requires prompt imaging of patients with trigeminal dysfunction and symptoms of facial pain and headache.

  12. CT, MRI, and (18)F-FDG PET/CT findings of malignant peripheral nerve sheath tumor of the head and neck.

    PubMed

    Kim, Ha Youn; Hwang, Ji Young; Kim, Hyung-Jin; Kim, Yi Kyung; Cha, Jihoon; Park, Gyeong Min; Kim, Sung Tae

    2017-10-01

    Background Malignant peripheral nerve sheath tumor (MPNST) is a highly malignant tumor and rarely occurs in the head and neck. Purpose To describe the imaging features of MPNST of the head and neck. Material and Methods We retrospectively analyzed computed tomography (CT; n = 14), magnetic resonance imaging (MRI; n = 16), and (18)F-FDG PET/CT (n = 5) imaging features of 18 MPNSTs of the head and neck in 17 patients. Special attention was paid to determine the nerve of origin from which the tumor might have arisen. Results All lesions were well-defined (n = 3) or ill-defined (n = 15) masses (mean, 6.1 cm). Lesions were at various locations but most commonly the neck (n = 8), followed by the intracranial cavity (n = 3), paranasal sinus (n = 2), and orbit (n = 2). The nerve of origin was inferred for 11 lesions: seven in the neck, two in the orbit, one in the cerebellopontine angle, and one on the parietal scalp. Attenuation, signal intensity, and enhancement pattern of the lesions on CT and MRI were non-specific. Necrosis/hemorrhage/cystic change within the lesion was considered to be present on images in 13 and bone change in nine. On (18)F-FDG PET/CT images, all five lesions demonstrated various hypermetabolic foci with maximum standard uptake value (SUVmax) from 3.2 to 14.6 (mean, 7.16 ± 4.57). Conclusion MPNSTs can arise from various locations in the head and neck. Though non-specific, a mass with an ill-defined margin along the presumed course of the cranial nerves may aid the diagnosis of MPSNT in the head and neck.

  13. Posterior Fossa Meningioma

    PubMed Central

    Saleh, Essam A.; Taibah, Abdel Kader; Achilli, Vittorio; Aristegui, Miguel; Mazzoni, Antonio; Sanna, Mario

    1994-01-01

    Posterior fossa meningioma is the second most common tumor in the cerebellopontine angle. It has a higher rate of postoperative morbidity and mortality compared to acoustic neuroma. Forty posterior fossa meningioma patients managed in our centers were reviewed. Thirty-nine patients were managed surgically with 42 surgical procedures. The approaches used were the translabyrinthine approach in 18 patients (43%), the modified transcochlear in 11 cases (26%), the petro-occipital transsigmoid in 5 cases (12%), the suboccipital in 4 cases (10%), the petro-occipital trassigmoid transcervical in 2 cases (5%), the petro-occipital transsigmoid transtentorial in 1 case (2%), and a subtemporal transtentorial for another case (2%). Facial nerve anatomical integrity was preserved in 87% of procedures but was interrupted in 5 cases, with 4 of the latter subsequently repaired. Total tumor removal was accomplished in 38 cases. A second-stage total tumor removal is planned for the remaining case. There was only one case of perioperative death and no cases of radiological recurrence so far. ImagesFigure 1Figure 2Figure 3Figure 4p206-bFigure 5p207-bFigure 5 PMID:17171173

  14. Spatially- and temporally-controlled postnatal p53 knockdown cooperates with embryonic Schwann cell precursor Nf1 gene loss to promote malignant peripheral nerve sheath tumor formation.

    PubMed

    Hirbe, Angela C; Dahiya, Sonika; Friedmann-Morvinski, Dinorah; Verma, Inder M; Clapp, D Wade; Gutmann, David H

    2016-02-16

    Malignant peripheral nerve sheath tumors (MPNSTs) are highly aggressive sarcomas that arise sporadically or in association with the Neurofibromatosis type 1 (NF1) cancer predisposition syndrome. In individuals with NF1, MPNSTs are hypothesized to arise from Nf1-deficient Schwann cell precursor cells following the somatic acquisition of secondary cooperating genetic mutations (e.g., p53 loss). To model this sequential genetic cooperativity, we coupled somatic lentivirus-mediated p53 knockdown in the adult right sciatic nerve with embryonic Schwann cell precursor Nf1 gene inactivation in two different Nf1 conditional knockout mouse strains. Using this approach, ~60% of mice with Periostin-Cre-mediated Nf1 gene inactivation (Periostin-Cre; Nf1(flox/flox) mice) developed tumors classified as low-grade MPNSTs following p53 knockdown (mean, 6 months). Similarly, ~70% of Nf1+/- mice with GFAP-Cre-mediated Nf1 gene inactivation (GFAP-Cre; Nf1(flox/null) mice) developed low-grade MPNSTs following p53 knockdown (mean, 3 months). In addition, wild-type and Nf1+/- mice with GFAP-Cre-mediated Nf1 loss develop MPNSTs following somatic p53 knockout with different latencies, suggesting potential influences of Nf1+/- stromal cells in MPNST pathogenesis. Collectively, this new MPNST model system permits the analysis of somatically-acquired events as well as tumor microenvironment signals that potentially cooperate with Nf1 loss in the development and progression of this deadly malignancy.

  15. Meningioma recurrence

    PubMed Central

    Bencze, János; Varkoly, Gréta; Kouhsari, Mahan C; Klekner, Álmos

    2016-01-01

    Abstract Meningioma accounts for more than 30% of all intracranial tumours. It affects mainly the elderly above the age of 60, at a female:male ratio of 3:2. The prognosis is variable: it is usually favourable with no progression in tumour grade and no recurrence in WHO grade 1 tumours. However, a minority of tumours represent atypical (grade 2) or anaplastic (grade 3) meningiomas; this heterogeneity is also reflected in histopathological appearances. Irrespective of the grade, the size of the tumour and the localisation may have severe, sometimes lethal consequences. Following neurosurgical interventions to remove the tumour, recurrence and progression in WHO grade may occur. Our knowledge on predisposing histomorphological and molecular factors of recurrence is rather limited. These can be classified as I) demographic II) environmental, III) genetic and epigenetic IV) imaging, V) neuropathological, and VI) neurosurgical. In view of the complex background of tumour recurrence, the recognition of often subtle signs of increased risk of recurrence requires close collaboration of experts from several medical specialties. This multidisciplinary approach results in better therapy and fewer complications related to tumour recurrence. PMID:28352788

  16. Convection-Enhanced Delivery (CED) in an Animal Model of Malignant Peripheral Nerve Sheath Tumors and Plexiform Neurofibromas

    DTIC Science & Technology

    2011-09-01

    povidone-iodine swabs or gauzes. 70% alcohol swab or gauze is used to remove the remaining povidone-iodine from the skin. ~100μL 1% lidocaine is...site. A few drops of lidocaine are dropped on the nerve. A 34 Gauge fused silica (WPI) cannula is inserted at a sharp angle (~10°) along the nerve...remove the remaining povidone-iodine from the skin. ~100μL 1% lidocaine is injected along the incision line to ensure adequate analgesia. A 15mm dorsal

  17. Meningiomas of Meckel's cave.

    PubMed

    Delfini, R; Innocenzi, G; Ciappetta, P; Domenicucci, M; Cantore, G

    1992-12-01

    A series of 16 patients with meningiomas of Meckel's cave is reported. Trigeminal neuralgia, typical or atypical, was the initial symptom in 10 patients (62.5%). At admission, trigeminal signs and symptoms were present in 15 patients (93.7%); in 7 patients (43.7%), trigeminal dysfunction was combined with the impairment of other cranial nerves. On retrospective analysis, these patients fall into two clinical groups that differ also in prognosis. Group 1 comprises eight patients with trigeminal signs and symptoms only. These patients had small meningiomas strictly affecting Meckel's cave. Total removal of the tumor was achieved in seven of eight patients, without adjunctive postoperative neurological deficits. In this group, there were no tumor recurrences. Group 2 comprises the other eight patients in whom trigeminal dysfunction was combined with impairment of other cranial nerves. These patients had large tumors arising from Meckel's cave and secondarily invading the cavernous sinus (five patients) or extending into the posterior fossa (two patients) or largely growing into the middle fossa (one patient). Total removal was achieved in only one patient, and a worsening of the preoperative neurological status was observed in four patients; there were three cases of tumor progression. A subtemporal intradural approach (used in the past in every case) is still used for the small tumors of Group 1 with good results. Since 1985, for tumors involving the cavernous sinus, we have employed a frontotemporal craniotomy with extradural clinoidectomy and superior and lateral approach to the cavernous sinus. When the tumor extends toward the posterior fossa, we use a combined temporosuboccipital-transpetrosal approach.(ABSTRACT TRUNCATED AT 250 WORDS)

  18. [Operative neurosurgery: personal view and historical backgrounds. (5) Meningioma].

    PubMed

    Yonekawa, Yasuhiro

    2009-01-01

    long time. In case of growth, stereotactic radiosurgery is the first choice and at last would come surgical intervention at the cost of quality of life QOL. Appropriate approaches for meningiomas arising from the incisura tentorii would be either the amygdalohippocampectomy AHE approach namely transSylvian transsulcus circularis approach for their anterior localization or the supracerebellar transtentorial SCTT approach for the posterior localization in the sitting position. In the latter following structures are to be preserved with great care: A. parietooccipitalis, trochlear nerve, Vena Rosenthal and the superior cerebellar artery which could have considerable supply to the tumor. Meningiomas of the falcotentorial junction are managed also with this approach but may necessitate combination of the suboccipital transtentorial approach large upper clivus meningiomas can be removed more effectively by paramedian or lateral suboccipital craniotomy via SCTT approach in the sitting position rather than the subtemporal transpetrosal approach. Clean and wider operative fields in the former approach are emphasized. Special mention is made to transvertebralis (dural) ring approach TVRA for the foramen magnum or lower clivus meningiomas, in which the vertebral artery can be mobilized without performing more extensive far lateral approach. Difficulties of management of recurrent parasagittal meningiomas with the location corresponding to the gyrus paracentralis plus supplementary motor area are to be emphasized. Role of the venous sinus reconstruction is discussed. Difficulties of management of recurrent meningiomas represented by atypical or anaplastic meningiomas WHO grade II or III which can not be managed only by surgical removal is discussed by presenting some example cases. Biological activity of meningiomas in different location can be quite different in multiple recurrent meningiomas. Meningiomas intractable to irradiation and/or chemotherapy are another challenging

  19. EZH2-miR-30d-KPNB1 pathway regulates malignant peripheral nerve sheath tumour cell survival and tumourigenesis.

    PubMed

    Zhang, Pingyu; Garnett, Jeannine; Creighton, Chad J; Al Sannaa, Ghadah Abbas; Igram, Davis R; Lazar, Alexander; Liu, Xiuping; Liu, Changgong; Pollock, Raphael E

    2014-02-01

    Malignant peripheral nerve sheath tumours (MPNSTs), which develop sporadically or from neurofibromatosis, recur frequently with high metastatic potential and poor outcome. The polycomb group protein enhancer of zeste homologue 2 (EZH2) is an important regulator for various human malignancies. However, the function of EZH2 in MPNSTs is unknown. Here we report that the EZH2-miR-30d-KPNB1 signalling pathway is critical for MPNST tumour cell survival in vitro and tumourigenicity in vivo. Up-regulated EZH2 in MPNST inhibits miR-30d transcription via promoter binding activity, leading to enhanced expression of the nuclear transport receptor KPNB1 that is inhibited by miR-30d targeting of KPNB1 3' UTR region. Furthermore, inhibition of EZH2 or KPNB1, or miR-30d over-expression, induces MPNST cell apoptosis in vitro and suppresses tumourigenesis in vivo. More importantly, forced over-expression of KPNB1 rescues MPNST cell apoptosis induced by EZH2 knockdown. Immunohistochemical analyses show that EZH2 and KPNB1 over-expression is observed in human MPNST specimens and is negatively associated with miR-30d expression. Our findings identify a novel signalling pathway involved in MPNST tumourigenesis, and also suggest that EZH2-miR-30d-KPNB1 signalling represents multiple potential therapeutic targetable nodes for MPNST.

  20. TERT promoter mutations and BRAF mutations are rare in sporadic, and TERT promoter mutations are absent in NF1-related malignant peripheral nerve sheath tumors.

    PubMed

    Dubbink, Hendrikus J; Bakels, Hannah; Post, Edward; Zwarthoff, Ellen C; Verdijk, Robert M

    2014-11-01

    Hot spot mutations in the promoter region of telomerase reverse transcriptase (TERT promoter mutations) occur frequently in tumors of neuroectodermal origin such as melanoma and glioma. Many of these tumors are of neuroectodermal or ectomesenchymal origin which is suggestive of TERT promoter mutations playing a role in the development of malignant peripheral nerve sheath tumors (MPNSTs). In melanoma a correlation has been suggested between the occurrence of TERT promoter mutations and v-RAF murine sarcoma viral oncogene homolog B1 (BRAF) mutations. We investigated TERT promoter and BRAF mutation frequency in respectively 94 and 86 consecutive MPNST cases from our institute. TERT promoter mutation analysis on DNA from formalin-fixed, paraffin-embedded specimens was performed by SNaPshot analysis. Sequence analysis of BRAF was performed by bidirectional DNA sequencing. We identified TERT C228T or C250T promoter mutations in 10 % (9/94) and BRAF V600E mutations in 3 % (3/86) of MPNSTs. All TERT promoter- and BRAF mutations occurred in NF1 unrelated tumors. One co-occurrence of a TERT promoter- and a BRAF mutation was observed. In comparison with other neuroectodermal derived malignant neoplasms, TERT promoter mutations occur at relatively low frequency in MPNSTs. The observation of TERT promotor and BRAF mutations in sporadic MPNSTs and the absence of TERT promotor and rarity of BRAF mutations in NF1 related tumors may imply an alternative genetic route of tumor progression in both patient groups.

  1. Peripheral nerve sheath tumors of the gastrointestinal tract: a multicenter study of 58 patients including NF1-associated gastric schwannoma and unusual morphologic variants.

    PubMed

    Agaimy, Abbas; Märkl, Bruno; Kitz, Julia; Wünsch, Peter H; Arnholdt, Hans; Füzesi, Laszlo; Hartmann, Arndt; Chetty, Runjan

    2010-04-01

    The frequency and morphological spectrum of gastrointestinal peripheral nerve sheath tumors (PNSTs) from consecutive case material has not been studied in the c-KIT era. We reviewed all mesenchymal gastrointestinal (GI) lesions at our departments according to current diagnostic criteria. PNSTs formed the third commonest group of mesenchymal GI tumors with a lower frequency (< or =5%) compared to gastrointestinal stromal tumors (GISTs; approximately 50%) and smooth muscle neoplasms ( approximately 30%). Granular cell tumors (GCTs; n = 31) and schwannomas (n = 22) were the most common types of PNSTs encountered. Rare tumors included neurofibromatosis 1 (NF1)-associated PNSTs (n = 5) and gastric perineurioma (n = 1). Thirteen schwannomas (including also some recent cases) were initially diagnosed as GIST, leiomyoma, or neurofibroma. Unusual histological variants included sigmoid GCT with prominent lipomatous component (n = 1), reticular-microcystic schwannoma of small (n = 1) and large (n = 1) bowel, NF1-associated gastric schwannoma (the first case to date), and psammomatous melanotic colonic schwannoma unrelated to Carney complex (n = 1). PNSTs coexisted with GIST in four patients (three had definite NF1). In conclusion, PNSTs of the GI tract are rare uniformly benign neoplasms that may show schwannian, perineurial, fibroblastic, or mixed differentiation. Most of them (92%) occurred sporadically unassociated with NF1 or NF2. Gastrointestinal PNSTs are still underrecognized by general pathologists. Awareness of their diverse morphology will help to avoid confusing them with smooth muscle neoplasms and GIST that they may closely mimic.

  2. Discovery of a small molecule targeting IRA2 deletion in budding yeast and neurofibromin loss in malignant peripheral nerve sheath tumor cells

    PubMed Central

    Wood, Matthew; Rawe, Melissa; Johansson, Gunnar; Pang, Shu; Soderquist, Ryan S.; Patel, Ami V.; Nelson, Sandra; Seibel, William; Ratner, Nancy; Sanchez, Yolanda

    2011-01-01

    Malignant peripheral nerve sheath tumor (MPNST) is a life-threatening complication of neurofibromatosis type 1 (NF1). NF1 is caused by mutation in the gene encoding neurofibromin, a negative regulator of Ras signaling. There are no effective pharmacologic therapies for MPNST. To identify new therapeutic approaches targeting this dangerous malignancy, we developed assays in NF1+/+ and NF1−/ − MPNST cell lines and in budding yeast lacking the NF1 homologue IRA2 (ira2Δ). Here we describe UC1, a small molecule that targets NF1−/− cell lines and ira2Δ budding yeast. Using yeast genetics we identified NAB3 as a high-copy suppressor of UC1 sensitivity. NAB3 encodes an RNA binding protein that associates with the C-terminal domain of RNA Pol II and plays a role in the termination of non-polyadenylated RNA transcripts. Strains with deletion of IRA2 are sensitive to genetic inactivation of NAB3, suggesting an interaction between Ras signaling and Nab3-dependent transcript termination. This work identifies a lead compound and a possible target pathway for NF1-associated MPNST, and demonstrates a novel model system approach to identify and validate target pathways for cancer cells in which NF1 loss drives tumor formation. PMID:21697395

  3. The Challenge of Cancer Genomics in Rare Nervous System Neoplasms: Malignant Peripheral Nerve Sheath Tumors as a Paradigm for Cross-Species Comparative Oncogenomics.

    PubMed

    Carroll, Steven L

    2016-03-01

    Comprehensive genomic analyses of common nervous system cancers provide new insights into their pathogenesis, diagnosis, and treatment. Although analogous studies of rare nervous system tumors are needed, there are major barriers to performing such studies. Cross-species comparative oncogenomics, identifying driver mutations in mouse cancer models and validating them in human tumors, is a promising alternative. Although still in its infancy, this approach is being applied to malignant peripheral nerve sheath tumors (MPNSTs), rare Schwann cell-derived malignancies that occur sporadically, after radiotherapy, and in neurofibromatosis type 1. Studies of human neurofibromatosis type 1-associated tumors suggest that NF1 tumor suppressor loss in Schwann cells triggers cell-autonomous and intercellular changes, resulting in development of benign neurofibromas; subsequent neurofibroma-MPNST progression is caused by aberrant growth factor signaling and mutations affecting the p16(INK4A)-cyclin D1-CDK4-Rb and p19(ARF)-Mdm2-p53 cell cycle pathways. Mice with Nf1, Trp53, and/or Cdkn2a mutations that overexpress the Schwann cell mitogen neuregulin-1 or overexpress the epidermal growth factor receptor validate observations in human tumors and, to various degrees, model human tumorigenesis. Genomic analyses of MPNSTs arising in neuregulin-1 and epidermal growth factor receptor-overexpressing mice and forward genetic screens with Sleeping Beauty transposons implicate additional signaling cascades in MPNST pathogenesis. These studies confirm the utility of mouse models for MPNST driver gene discovery and provide new insights into the complexity of MPNST pathogenesis.

  4. A novel NF1 mutation in a Chinese patient with giant café-au-lait macule in neurofibromatosis type 1 associated with a malignant peripheral nerve sheath tumor and bone abnormality.

    PubMed

    Tong, H-X; Li, M; Zhang, Y; Zhu, J; Lu, W-Q

    2012-08-29

    Neurofibromatosis type 1 (NF1; OMIM#162200) is a common neurocutaneous disorder that is characterized by multiple café-au-lait, skinfold freckling, Lisch nodules, and neurofibromas. Mutations in the NF1 gene, which encodes the neurofibromin protein, have been identified as the pathogenic gene of NF1. In this study, we present a clinical and molecular study of a Chinese patient with giant café-au-lait in NF1. The patient showed >6 café-au-lait spots on the body, axillary freckling, and multiple subcutaneous neurofibromas. He also had a malignant peripheral nerve sheath tumor and bone abnormalities. The germline mutational analysis of the NF1 gene revealed a novel missense mutation in exon 13. It is a novel heterozygous nucleotide G>A transition at position 2241 of the NF1 gene. We found no mutation in malignant peripheral nerve sheath tumor DNA from this patient. This expands the database for NF1 gene mutations in NF1. Its absence in the normal chromosomes suggests that it is responsible for the NF1 phenotype. To our knowledge, this is the first case of giant café-au-lait macule in NF1 associated with a malignant peripheral nerve sheath tumor and bone abnormality.

  5. The ROAM/EORTC-1308 trial: Radiation versus Observation following surgical resection of Atypical Meningioma: study protocol for a randomised controlled trial.

    PubMed

    Jenkinson, Michael D; Javadpour, Mohsen; Haylock, Brian J; Young, Bridget; Gillard, Helen; Vinten, Jacqui; Bulbeck, Helen; Das, Kumar; Farrell, Michael; Looby, Seamus; Hickey, Helen; Preusser, Mattheus; Mallucci, Conor L; Hughes, Dyfrig; Gamble, Carrol; Weber, Damien C

    2015-11-14

    Atypical meningiomas are an intermediate grade brain tumour with a recurrence rate of 39-58 %. It is not known whether early adjuvant radiotherapy reduces the risk of tumour recurrence and whether the potential side-effects are justified. An alternative management strategy is to perform active monitoring with magnetic resonance imaging (MRI) and to treat at recurrence. There are no randomised controlled trials comparing these two approaches. A total of 190 patients will be recruited from neurosurgical/neuro-oncology centres across the United Kingdom, Ireland and mainland Europe. Adult patients undergoing gross total resection of intracranial atypical meningioma are eligible. Patients with multiple meningioma, optic nerve sheath meningioma, previous intracranial tumour, previous cranial radiotherapy and neurofibromatosis will be excluded. Informed consent will be obtained from patients. This is a two-stage trial (both stages will run in parallel): Stage 1 (qualitative study) is designed to maximise patient and clinician acceptability, thereby optimising recruitment and retention. Patients wishing to continue will proceed to randomisation. Stage 2 (randomisation) patients will be randomised to receive either early adjuvant radiotherapy for 6 weeks (60 Gy in 30 fractions) or active monitoring. The primary outcome measure is time to MRI evidence of tumour recurrence (progression-free survival (PFS)). Secondary outcome measures include assessing the toxicity of the radiotherapy, the quality of life, neurocognitive function, time to second line treatment, time to death (overall survival (OS)) and incremental cost per quality-adjusted life year (QALY) gained. ROAM/EORTC-1308 is the first multi-centre randomised controlled trial designed to determine whether early adjuvant radiotherapy reduces the risk of tumour recurrence following complete surgical resection of atypical meningioma. The results of this study will be used to inform current neurosurgery and neuro

  6. Meningiomas of the cerebellopontine angle.

    PubMed

    Matthies, C; Carvalho, G; Tatagiba, M; Lima, M; Samii, M

    1996-01-01

    Meningiomas of the cerebellopontine angle (CPA) represent a clinically and surgically interesting entity. The opportunity of complete surgical excision and the incidence of impairment of nerval structures largely depend on the tumour biology that either leads to displacement of surrounding structures by an expansive type of growth or to an enveloping of nerval and vascular structures by an en plaque type of growth. As the origin and the direction of growth are very variable, the exact tumour extension in relation to the nerval structures and the tumour origin can be identified sometimes only at the time of surgery. Out of a series of 230 meningiomas of the posterior skull base operated between 1978 and 1993, data of 134 meningiomas involving the cerebellopontine angle are presented. There were 20% male and 80% female patients, age at the time of surgery ranging from 18 to 76 years, on the average 51 years. The clinical presentation was characterized by a predominant disturbance of the cranial nerves V (19%), VII (11%), VIII (67%) and the caudal cranial nerves (6%) and signs of ataxia (28%). 80% of the meningiomas were larger than 30 mm in diameter, 53% led to evident brainstem compression or dislocation and 85% extended anteriorly to the internal auditory canal. Using the lateral suboccipital approach in the majority of cases and a combined presigmoidal or combined suboccipital and subtemporal approaches in either sequence in 5%, complete tumour removal (Simpson I and II) was accomplished in 95% and subtotal tumour removal in 5%. Histologically the meningiotheliomatous type was most common (49%) followed by the mixed type (19%), fibroblastic (16%), psammomatous (7%), hemangioblastic (7%) and anaplastic (2%) types. Major post-operative complications were CSF leakage (8%) requiring surgical revision in 2% and hemorrhage (3%) requiring revision in 2%. While the majority of neurological disturbances showed signs of recovery, facial nerve paresis or paralysis was

  7. Jugular foramen meningiomas: review of the major surgical series.

    PubMed

    Bakar, Bulent

    2010-01-01

    Primary jugular foramen meningiomas are uncommon, with 96 previous cases published between 1992 and 2007. Exact location and extent of tumor were determined on the basis of radiologic and operative findings and used to develop a staging system. The mean age of patients was 39.4 years. The lesion was located on the right in 14 patients and on the left in 11 patients. The series identified 23 males and 58 females. The most common presenting clinical symptoms were hearing loss and tinnitus. Most clinical findings were middle ear mass and neck mass. Most meningiomas were World Health Organization grade I. The most common postoperative complications were lower cranial nerve paresis and facial nerve paresis. Surgical planning should consider that meningiomas usually invade the dura mater, cranial nerves, and surrounding bone. The surgeon should carefully collect detailed data about the tumor, and consult an otolaryngologist preoperatively for lower cranial nerve functions and hearing levels.

  8. TAGLN expression is upregulated in NF1-associated malignant peripheral nerve sheath tumors by hypomethylation in its promoter and subpromoter regions.

    PubMed

    Park, Gun-Hoo; Lee, Su-Jin; Yim, Hyunee; Han, Jae-Ho; Kim, Hyon J; Sohn, Young-Bae; Ko, Jung Min; Jeong, Seon-Yong

    2014-10-01

    Neurofibromatosis type 1 (NF1) caused by NF1 gene mutation is a commonly inherited autosomal dominant disorder. Malignant peripheral nerve sheath tumors (MPNSTs), a type of aggressive sarcoma, are a major cause of mortality in NF1 patients. The malignant transformation of benign plexiform neurofibromas (PNs) to MPNSTs is a marked peculiarity in NF1 patients, yet the pathogenesis remains poorly understood. We found that an actin-associated protein transgelin (SM22) was highly expressed in NF1-deficient MPNST tissues compared to NF1-deficient PN tissues using immunohistological staining and primary cultured MPNST cells in western blot analysis. We further found that this transgelin upregulation was caused by increased transcriptional expression of the TAGLN gene encoding transgelin. Comparison of DNA methylation values in the promoter and subpromoter regions of the TAGLN gene in three types of NF1-deficient primary-cultured cells, derived from an NF1 patient's normal phenotype, a benign PN and MPNST tissues, revealed that the TAGLN gene was hypomethylated in the MPNST cells. Next, to determine the functional role of transgelin in MPNST pathogenesis, we manipulated the TAGLN gene expression and investigated the alteration of the RAS-mitogen-activated protein kinase (MAPK) signaling pathway in the normal-phenotypic and malignant tumor cells. The downregulation of TAGLN expression in NF1-deficient MPNST tumor cells through the treatment of the small interfering RNA resulted in a decrease in the RAS activation (GTP-RAS) and the downstream ERK1/2 activation (phosphorylated ERK1/2), while the overexpression of TAGLN in normal-phenotypic NF1-deficient cells caused an increase in RAS and ERK1/2 activation. These results indicate that upregulation of transgelin caused by hypomethylation of the TAGLN gene is closely involved in tumor progression in NF1.

  9. Protein expression of BIRC5, TK1, and TOP2A in malignant peripheral nerve sheath tumours--A prognostic test after surgical resection.

    PubMed

    Kolberg, Matthias; Høland, Maren; Lind, Guro E; Ågesen, Trude H; Skotheim, Rolf I; Hall, Kirsten Sundby; Mandahl, Nils; Smeland, Sigbjørn; Mertens, Fredrik; Davidson, Ben; Lothe, Ragnhild A

    2015-06-01

    No consensus treatment regime exists beyond surgery for malignant peripheral nerve sheath tumours (MPNST), and the purpose of the present study was to find new approaches to stratify patients with good and poor prognosis and to better guide therapeutic intervention for this aggressive soft tissue cancer. From a total of 67 MPNSTs from Scandinavian patients with and without neurofibromatosis type 1, 30 MPNSTs were investigated by genome-wide RNA expression profiling and 63 MPNSTs by immunohistochemical (IHC) analysis, and selected genes were submitted to analyses of disease-specific survival. The potential drug target genes survivin (BIRC5), thymidine kinase 1 (TK1), and topoisomerase 2-alpha (TOP2A), all encoded on chromosome arm 17q, were up-regulated in MPNST as compared to benign neurofibromas. Each of them was found to be independent prognostic markers on the gene expression level, as well as on the protein level. A prognostic profile was identified by combining the nuclear expression scores of the three proteins. For patients with completely resected tumours only 15% in the high risk group were alive after two years, as compared to 78% in the low risk group. In conclusion, we found a novel protein expression profile which identifies MPNST patients with inferior prognosis even after assumed curative surgery. The tested proteins are drug targets; therefore the expression profile may provide predictive information guiding the design of future clinical trials. Importantly, as the effect is seen on the protein level using IHC, the biomarker panel can be readily implemented in routine clinical testing.

  10. Individual patient data systematic review and meta-analysis of optic nerve sheath diameter ultrasonography for detecting raised intracranial pressure: protocol of the ONSD research group.

    PubMed

    Dubourg, Julie; Messerer, Mahmoud; Karakitsos, Dimitrios; Rajajee, Venkatakrishna; Antonsen, Erik; Javouhey, Etienne; Cammarata, Alessandro; Cotton, Michael; Daniel, Roy Thomas; Denaro, Carmelo; Douzinas, Emmanuel; Dubost, Clément; Berhouma, Moncef; Kassai, Behrouz; Rabilloud, Muriel; Gullo, Antonino; Hamlat, Abderrhammane; Kouraklis, Gregorios; Mannanici, Giuseppe; Marill, Keith; Merceron, Sybille; Poularas, John; Ristagno, Giuseppe; Noble, Vicki; Shah, Sachita; Kimberly, Heidi; Cammarata, Gianluca; Moretti, Riccardo; Geeraerts, Thomas

    2013-08-06

    The purpose of the optic nerve sheath diameter (ONSD) research group project is to establish an individual patient-level database from high quality studies of ONSD ultrasonography for the detection of raised intracranial pressure (ICP), and to perform a systematic review and an individual patient data meta-analysis (IPDMA), which will provide a cutoff value to help physicians making decisions and encourage further research. Previous meta-analyses were able to assess the diagnostic accuracy of ONSD ultrasonography in detecting raised ICP but failed to determine a precise cutoff value. Thus, the ONSD research group was founded to synthesize data from several recent studies on the subject and to provide evidence on the diagnostic accuracy of ONSD ultrasonography in detecting raised ICP. This IPDMA will be conducted in different phases. First, we will systematically search for eligible studies. To be eligible, studies must have compared ONSD ultrasonography to invasive intracranial devices, the current reference standard for diagnosing raised ICP. Subsequently, we will assess the quality of studies included based on the QUADAS-2 tool, and then collect and validate individual patient data. The objectives of the primary analyses will be to assess the diagnostic accuracy of ONSD ultrasonography and to determine a precise cutoff value for detecting raised ICP. Secondly, we will construct a logistic regression model to assess whether patient and study characteristics influence diagnostic accuracy. We believe that this IPD MA will provide the most reliable basis for the assessment of diagnostic accuracy of ONSD ultrasonography for detecting raised ICP and to provide a cutoff value. We also hope that the creation of the ONSD research group will encourage further study. PROSPERO registration number: CRD42012003072.

  11. Individual patient data systematic review and meta-analysis of optic nerve sheath diameter ultrasonography for detecting raised intracranial pressure: protocol of the ONSD research group

    PubMed Central

    2013-01-01

    Background The purpose of the optic nerve sheath diameter (ONSD) research group project is to establish an individual patient-level database from high quality studies of ONSD ultrasonography for the detection of raised intracranial pressure (ICP), and to perform a systematic review and an individual patient data meta-analysis (IPDMA), which will provide a cutoff value to help physicians making decisions and encourage further research. Previous meta-analyses were able to assess the diagnostic accuracy of ONSD ultrasonography in detecting raised ICP but failed to determine a precise cutoff value. Thus, the ONSD research group was founded to synthesize data from several recent studies on the subject and to provide evidence on the diagnostic accuracy of ONSD ultrasonography in detecting raised ICP. Methods This IPDMA will be conducted in different phases. First, we will systematically search for eligible studies. To be eligible, studies must have compared ONSD ultrasonography to invasive intracranial devices, the current reference standard for diagnosing raised ICP. Subsequently, we will assess the quality of studies included based on the QUADAS-2 tool, and then collect and validate individual patient data. The objectives of the primary analyses will be to assess the diagnostic accuracy of ONSD ultrasonography and to determine a precise cutoff value for detecting raised ICP. Secondly, we will construct a logistic regression model to assess whether patient and study characteristics influence diagnostic accuracy. Discussion We believe that this IPD MA will provide the most reliable basis for the assessment of diagnostic accuracy of ONSD ultrasonography for detecting raised ICP and to provide a cutoff value. We also hope that the creation of the ONSD research group will encourage further study. Trial registration PROSPERO registration number: CRD42012003072 PMID:23919384

  12. Genomic and Molecular Characterization of Malignant Peripheral Nerve Sheath Tumor Identifies the IGF1R Pathway as a Primary Target for Treatment

    PubMed Central

    Yang, Jilong; Ylipää, Antti; Sun, Yan; Zheng, Hong; Chen, Kexin; Nykter, Matti; Trent, Jonathan; Ratner, Nancy; Lev, Dina C.; Zhang, Wei

    2011-01-01

    Purpose Malignant peripheral nerve sheath tumor (MPNST) is a rare sarcoma that lacks effective therapeutic strategies. We gain insight into the most recurrent genetically altered pathways with the purpose of scanning possible therapeutic targets. Experimental design We performed a microarray based-comparative genomic hybridization (aCGH) profiling of two cohorts of primary MPNST tissue samples including 25 patients treated at The University of Texas MD Anderson Cancer Center and 26 patients from Tianjin Cancer Hospital. IHC and cell biology detection and validation were performed on human MPNST tissues and cell lines. Results Genomic characterization of 51 MPNST tissue samples identified several frequently amplified regions harboring 2,599 genes and regions of deletion including 4,901 genes. At the pathway level, we identified a significant enrichment of copy number–altering events in the insulin-like growth factor 1 receptor (IGF1R) pathway, including frequent amplifications of the IGF1R gene itself. To validate the IGF1R pathway as a potential target in MPNSTs, we first confirmed that high IGF1R protein correlated with worse tumor-free survival in an independent set of samples using immunohistochemistry. Two MPNST cell lines (ST88-14 and STS26T) were used to determine the effect of attenuating IGF1R. Inhibition of IGF1R in ST88-14 cells using small interfering RNAs or an IGF1R inhibitor, MK-0646, led to significant decreases in cell proliferation, invasion, and migration accompanied by attenuation of the PI3K/AKT and MAPK pathways. Conclusion These integrated genomic and molecular studies provide evidence that the IGF1R pathway is a potential therapeutic target for patients with MPNST. PMID:22042973

  13. Targeting the PI3K/mTOR axis, alone and in combination with autophagy blockade, for the treatment of malignant peripheral nerve sheath tumors

    PubMed Central

    Ghadimi, Markus P; Lopez, Gonzalo; Torres, Keila E.; Belousov, Roman; Young, Eric D.; Liu, Jeffery; Brewer, Kari J.; Hoffman, Aviad; Lusby, Kristelle; Lazar, Alexander J; Pollock, Raphael E.; Lev, Dina

    2012-01-01

    There is a critical need for efficacious therapeutic strategies to improve the outcome of patients afflicted by malignant peripheral nerve sheath tumors (MPNST). Multiple lines of evidence suggest a role for deregulated PI3K/mTOR signaling in MPNST, making this axis an attractive target for therapeutic manipulation. Based on previous observations obtained from in vitro experimentation, here we aimed to assess the effects of PI3K/mTOR blockade on MPNST growth in vivo. The anti-MPNST impact of XL765, a dual PI3K/mTOR inhibitor currently being evaluated in human cancer clinical trials, was tested in two human MPNST xenograft models (STS26T and MPNST724) and an experimental model of pulmonary metastasis (STS26T). XL765 abrogated human MPNST local and metastatic growth in SCID mice. Notably, this therapeutic approach failed to induce apoptosis in MPNST cells but rather resulted in marked productive autophagy. Importantly, genetic and pharmacologic autophagy blockade reversed apoptotic resistance and resulted in significant PI3K/mTOR inhibition-induced MPNST cell death. The addition of the autophagy inhibitor, chloroquine, to the therapeutic regimen of MPNST xenografts after pre-treatment with XL765 resulted in superior anti-tumor effects as compared to either agent alone. Together, pre-clinical studies described here expand our previous findings and suggest that PI3K/mTOR inhibition alone and (most importantly) in combination with autophagy blockade may comprise a novel and efficacious therapy for patients harboring MPNST. PMID:22848094

  14. The role of angiogenesis in the transformation of plexiform neurofibroma into malignant peripheral nerve sheath tumors in children with neurofibromatosis type 1.

    PubMed

    Gesundheit, Benjamin; Parkin, Patricia; Greenberg, Mark; Baruchel, Sylvain; Senger, Christof; Kapelushnik, Josef; Smith, Charles; Klement, Giannoula Lakka

    2010-10-01

    The role of angiogenesis in the transformation of peripheral neurofibroma (PNF) to malignant peripheral nerve sheath tumor (MPNST) in neurofibromatosis type 1 (NF1) remains elusive and forms the objective of this study. Archival tissue from 5 children with NF1 and PNF, who developed MPNST between the ages of 8 and 15 years were analyzed for differences in microvasculature. The role of proangiogenic growth factors such as Vascular Endothelial Growth Factor (VEGF), and its receptors Flk-1 and Flt-1, and vessel maturity, defined as von Willebrand factor (vWf), α-smooth muscle actin+ (SMA+), were evaluated by immuno-histochemistry. A qualitative evaluation of the vasculature showed predominantly α-SMA+/vWf+ more stable vessels in PNF, and an irregular meshwork of α-SMA-/vWf+ endothelial cells structures in MPNST. In NF and PNF tumor cells were VEGF-, in contrast to VEGF+ tumor cells in MPNST. If present, the VEGF stain was confined mainly to the perivascular spaces in PNF, unlike the mainly stromal VEGF stain in MPNST. VEGF receptors also manifested a tumor stage-specific pattern. Flk-1 and Flt-1 were restricted to the mature, well-formed vasculature in PNF, but exhibited a diffuse pattern in MPNST. Our study provides a rare opportunity to document consistent and histologically detectable differences in the vascular organization of PNF and MPNST. It permits a pair-wise evaluation of the malignant conversion of benign PNF into its malignant counterpart, in the same patients. The phenotypic variations and characteristics of the vessels in these tumors are consistent with the idea that a strong proangiogenic drive contributes to the progressive growth in MPNST.

  15. Cadaveric study of zone 2 flexor hallucis longus tendon sheath.

    PubMed

    Lui, Tun Hing; Chan, Kwok Bill; Chan, Lap Ki

    2010-06-01

    The purpose of this study was to investigate the anatomy of the zone 2 flexor hallucis longus (FHL) tendon sheath. Dissection of the zone 2 FHL tendon sheath was performed in 12 feet of 6 cadavers. The tendon sheath was subdivided into proximal fibrous (zone 2A) and distal fascial (zone 2B) parts. The lengths of the zone 2A and 2B FHL tendon were measured and represented the length of the corresponding tendon sheaths, and the relation of the medial plantar nerve to each part of the zone 2 FHL tendon sheath was studied. In all specimens there were fibrous and fascial components of the zone 2 FHL tendon sheath. The medial plantar nerve crossed the zone 2B tendon sheaths and then became plantar lateral to the sheath in 7 specimens. The distance between the medial plantar nerve and the orifice of the zone 2A tendon sheath averaged 7.6 mm. The distance between the medial plantar nerve and the junction between zones 2A and 2B averaged 3.2 mm. The distance between the medial plantar nerve and the distal end of the zone 2B tendon sheath averaged 4.2 mm. The mean length of the zone 2A tendon sheath was 35.9 mm, and the mean length of the zone 2B tendon sheath was 30.5 mm. The zone 2 FHL tendon sheath can be subdivided into a proximal fibrous zone (2A) and a distal fascial zone (2B). Because of the close proximity of the medial plantar nerve to the tendon sheath, there is a significant risk of iatrogenic nerve injury when surgical procedures are performed in zone 2B. An understanding of the anatomy of the zone 2 FHL tendon sheath is useful for the safe practice of zone 2 FHL tendoscopy. Copyright (c) 2010 Arthroscopy Association of North America. Published by Elsevier Inc. All rights reserved.

  16. Genetic landscape of meningioma.

    PubMed

    Yuzawa, Sayaka; Nishihara, Hiroshi; Tanaka, Shinya

    2016-10-01

    Meningioma is the most common intracranial tumor, arising from arachnoid cells of the meninges. Monosomy 22 and inactivating mutations of NF2 are well-known genetic alterations of meningiomas. More recently, mutations in TRAF7, AKT1, KLF4, SMO, and PIK3CA were identified by next-generation sequencing. We here reviewed 553 meningiomas for the mutational patterns of the six genes. NF2 aberration was observed in 55 % of meningiomas. Mutations of TRAF7, AKT1, KLF4, PIK3CA, and SMO were identified in 20, 9, 9, 4.5, and 3 % of cases, respectively. Altogether, 80 % of cases harbored at least one of the genetic alterations in these genes. NF2 alterations and mutations of the other genes were mutually exclusive with a few exceptions. Clinicopathologically, tumors with mutations in TRAF7/AKT1 and SMO shared specific features: they were located in the anterior fossa, median middle fossa, or anterior calvarium, and most of them were meningothelial or transitional meningiomas. TRAF7/KLF4 type meningiomas showed different characteristics in that they occurred in the lateral middle fossa and median posterior fossa as well as anterior fossa and median middle fossa, and contained a secretory meningioma component. We also discuss the mutational hotspots of these genes and other genetic/cytogenetic alterations contributing to tumorigenesis or progression of meningiomas.

  17. Temporal bone meningiomas.

    PubMed

    Hooper, R; Siu, K; Cousins, V

    1990-10-01

    Meningiomas should be considered in the differential diagnosis of space-occupying lesions of the temporal bone. Five cases of meningiomas of the temporal bone are described and the literature reviewed. These tumours may stimulate Schwannomas and glomus tumours in their presentation and radiological findings. The tumours were managed by combining standard neurosurgical approaches with temporal bone and skull base techniques.

  18. Loss of H3K27 tri-methylation is a diagnostic marker for malignant peripheral nerve sheath tumors and an indicator for an inferior survival.

    PubMed

    Cleven, Arjen H G; Sannaa, Ghadah A Al; Briaire-de Bruijn, Inge; Ingram, Davis R; van de Rijn, Matt; Rubin, Brian P; de Vries, Maurits W; Watson, Kelsey L; Torres, Kelia E; Wang, Wei-Lien; van Duinen, Sjoerd G; Hogendoorn, Pancras C W; Lazar, Alexander J; Bovée, Judith V M G

    2016-06-01

    Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive sarcomas that can show overlapping features with benign neurofibromas as well as high-grade sarcomas. Additional diagnostic markers are needed to aid in this often challenging differential diagnosis. Recently mutations in two critical components of the polycomb repressor 2 (PRC2) complex, SUZ12 and EED, were reported to occur specifically in MPNSTs while such mutations are absent in neurofibromas, both in the setting of neurofibromatosis (NF) and sporadic cases. Furthermore, both SUZ12 and EED mutations in MPNSTs were associated with loss of H3K27 tri-methylation, a downstream target of PRC2. Therefore, we tested whether H3K27me3 immunohistochemistry is useful as a diagnostic and prognostic marker for MPNSTs. We performed H3K27me3 immunohistochemistry in 162 primary MPNSTs, 97 neurofibromas and 341 other tumors using tissue microarray. We observed loss of H3K27me3 in 34% (55/162) of all MPNSTs while expression was retained in all neurofibromas including atypical (n=8) and plexiform subtypes (n=24). Within other tumors we detected loss of H3K27me3 in only 7% (24/341). Surprisingly, 60% (9/15) of synovial sarcomas and 38% (3/8) of fibrosarcomatous dermatofibrosarcoma protuberans (DFSP) showed loss of H3K27 trimethylation. Only 1 out of 44 schwannomas showed loss of H3K27me3 and all 4 perineuriomas showed intact H3K27me3. Furthermore, MPNSTs with loss of H3K27 tri-methylation showed inferior survival compared with MPNSTs with intact H3K27 tri-methylation, which was validated in two independent cohorts. Our results indicate that H3K27me3 immunohistochemistry is useful as a diagnostic marker, in which loss of H3K27me3 favors MPNST above neurofibroma. However, H3K27me3 immunohistochemistry is not suitable to distinguish MPNST from its morphological mimicker synovial sarcoma or fibrosarcomatous DFSP. Since loss of H3K27 tri-methylation was related to poorer survival in MPNST, chromatin modification mediated

  19. NF1 deficiency causes Bcl-xL upregulation in Schwann cells derived from neurofibromatosis type 1-associated malignant peripheral nerve sheath tumors.

    PubMed

    Park, Ho-Jin; Lee, Su-Jin; Sohn, Young Bae; Jin, Hyun-Seok; Han, Jae-Ho; Kim, Young-Bae; Yim, Hyunee; Jeong, Seon-Yong

    2013-02-01

    Since the bi-allelic inactivation of both neurofibromin 1 (NF1) gene alleles (NF1(-/-)) in Schwann cells (SCs) is common in both benign plexiform neurofibromas (PNs) and malignant peripheral nerve sheath tumors (MPNSTs) in patients with neurofibromatosis type 1 (NF1), other genetic alterations in SCs may be required for tumor progression of PNs to MPNSTs. We found that the anti-apoptotic Bcl-xL protein is upregulated in MPNST tissues compared to PN tissues from patients with NF1 by immunohistological staining. In addition, we investigated whether Bcl-xL is upregulated in SCs derived from MPNSTs and found a significantly higher Bcl-xL expression level in sNF96.2 MPNST-derived SCs compared to normal human SCs (HSCs). We also discovered that the increased Bcl-xL expression caused an increase in drug resistance to doxorubicin in MPNST-derived SCs. Manipulation of NF1 gene expression levels by treatment with small interfering RNA (siRNA) and overexpression of the neurofibromin GAP-related domain (NF1-GRD) demonstrated that upregulated Bcl-xL expression in MPNST-derived SCs was caused by NF1 deficiency. Treatment with the Erk1/2 inhibitor, PD98059, resulted in a slight increase in Bcl-xL levels in neurofibromin-depleted normal HSCs, indicating that Bcl-xL upregulation in MPNST-derived SCs is mediated by activated Erk1/2, which is a Ras downstream protein regulated by neurofibromin. As the reduction of Bcl-xL expression restored sensitivity to doxorubicin-induced apoptosis in sNF96.2 cells, we examined the effect of the small molecule Bcl-xL inhibitor ABT-737 on sNF96.2 cells. A very low dose of ABT-737 combined with doxorubicin synergistically enhanced sensitivity to doxorubicin-induced apoptosis in sNF96.2 cells, suggesting that ABT-737 and doxorubicin may be a good combination to effectively treat NF1-associated MPNSTs with minimal side-effects. Collectively, our results suggest that upregulation of Bcl-xL in MPNST-derived SCs may be caused by the NF1 deficiency

  20. Molecular characterization of permanent cell lines from primary, metastatic and recurrent malignant peripheral nerve sheath tumors (MPNST) with underlying neurofibromatosis-1.

    PubMed

    Fang, Yuqiang; Elahi, Abul; Denley, Ryan C; Rao, Pulivarthi H; Brennan, Murray F; Jhanwar, Suresh C

    2009-04-01

    Malignant peripheral nerve sheath tumors (MPNSTs) develop in patients with underlying NF1, and usually arise as a result of malignant transformation of a pre-existing plexiform neurofibroma. The clonal cytogenetic abnormalities reported in primary MPNST include complex karyotypes with chromosome numbers in the triploid or tetraploid range with recurrent abnormalities of several chromosomes including losses or imbalances. As a prelude to cell biological, pharmacological, and functional studies to investigate pathways and gene(s) associated with multistep tumorigenesis, which includes progression, metastasis and resistance to therapy in MPNST, detailed molecular cytogenetic and genetic analyses of cell lines from primary, metastatic and recurrent MPNST with underlying NF1 disorder have been performed. The clonal cytogenetic abnormalities detected in the primary tumor cell line were similar to those observed in primary cultures of this tumor. Due to the complexity of the rearrangements seen by G-banded karyotype analysis, further characterization of the clonal abnormalities in these three cell lines was performed by molecular cytogenetic techniques, including CGH and SKY. CGH analysis detected recurrent deletions of 9p, 12q21-q32, complete losses of the X-chromosome, and gains of the chromosomal segment 17q25 in all three cell lines. SKY analysis detected extensive clonal abnormalities in these cell lines. The nature and the alterations of the cell cycle regulators, particularly those associated with G1-S checkpoints and known to be deregulated in MPNST, were studied. These cell cycle regulators included those associated with Rb1-cyclin D1 and the p53 pathways. The findings are consistent with the argument that an imbalance between the cyclin activators of CDKs and inhibitory proteins such as p16 result in uncontrollable proliferation in the cell lines, associated with progression of the disease. LOH and expression of the p53 gene in metastatic and recurrent cell

  1. Optic nerve sheath diameter threshold by ocular ultrasonography for detection of increased intracranial pressure in Korean adult patients with brain lesions

    PubMed Central

    Lee, Si Un; Jeon, Jin Pyeong; Lee, Hannah; Han, Jung Ho; Seo, Mingu; Byoun, Hyoung Soo; Cho, Won-Sang; Ryu, Ho Geol; Kang, Hyun-Seung; Kim, Jeong Eun; Kim, Heung Cheol; Jang, Kyung-Sool

    2016-01-01

    Abstract Optic nerve sheath diameter (ONSD) seen on ocular US has been associated with increased intracranial pressure (IICP). However, most studies have analyzed normal range of ONSD and its optimal cut-off point for IICP in Caucasian populations. Considering ONSD differences according to ethnicity, previous results may not accurately reflect the association between IICP and ONSD in Koreans. Therefore, we conducted this study to investigate normal range of ONSD and its optimal threshold for detecting IICP in Korean patients. This prospective multicenter study was performed for patients with suspected IICP. ONSD was measured 3 mm behind the globe using a 13-MHz US probe. IICP was defined as significant brain edema, midline shift, compression of ventricle or basal cistern, effacement of sulci, insufficient gray/white differentiation, and transfalcine herniation by radiologic tests. The results of the ONSD are described as the median (25th–75th percentile). The differences of ONSD according to disease entity were analyzed. A receiver operator characteristic (ROC) curve was generated to determine the optimal cut-off point for identifying IICP. A total of 134 patients were enrolled. The patients were divided into 3 groups as follows: patients with IICP, n = 81 (60.5%); patients without IICP, n = 27 (20.1%); and control group, n = 26 (19.4%). ONSD in patients with IICP (5.9 mm [5.8–6.2]) is significantly higher than those without IICP (5.2 mm [4.8–5.4]) (P < 0.01) and normal control group (4.9 mm [4.6–5.2]) (P < 0.001). Between patients without IICP and normal control group, the difference of ONSD did not reach statistical significance (P = 0.31). ONSD >5.5 mm yielded a sensitivity of 98.77% (95% CI: 93.3%–100%) and a specificity of 85.19% (95% CI: 66.3%–95.8%). In conclusion, the optimal cut-off point of ONSD for identifying IICP was 5.5 mm. ONSD seen on ocular US can be a feasible method for detection and serial monitoring of ICP in

  2. The role of MAPK signaling pathway in the Her-2-positive meningiomas

    PubMed Central

    Wang, Zhaoyin; Wang, Weijia; Xu, Shan; Wang, Shanshan; Tu, Yi; Xiong, Yifeng; Mei, Jinhong; Wang, Chunliang

    2016-01-01

    Meningiomas are common types of adult nerve system tumors. Although most cases are considered benign, due to its high rate of recurrence and easy malignant progression to anaplastic meningioma they present a puzzle for the current treatment. The HER-2 oncogene has important value for meningioma cells development and progression. So far, little is known about the effect on the exact underlying signal pathway and molecular mechanisms of HER-2-positive meningioma cells. The goal of the present study was to determine the effects of HER-2 gene and possible involvement of MAPK signal pathway in human malignant meningioma. We applied q-PCR analysis, immunofluorescence (IF) staining, western blot analysis, animal model, MAPK inhibition, MTT assay and cell invasion analysis for the investigation. The results demonstrated that the downregulation of the expression of HER-2 significantly inhibited cell motility and proliferation of human meningioma cells in vivo. Accordingly, in the HER-2-overexpression meningioma cells with the inhibition of ERK1/2, ERK5, JNK, in the cells with the ERK1/2, ERK5 inhibition, protein expression was markedly suppressed as well as the cell proliferation resistance. No difference was observed in the HER-2-overexpression meningioma cells with the inhibition of JNK. These findings suggest that HER-2 gene can affect the proliferation ability of human meningioma cells in vivo and MAPK signal pathway may contribute to the carcinogenesis and development of human meningiomas combinating with HER-2. PMID:27279438

  3. [Early postoperative results of surgical treatment of patients with anterior clinoidal meningiomas].

    PubMed

    Chernov, S V; Rzaev, D A; Kalinovsky, A V; Dmitriev, A B; Kasymov, A R; Zotov, A V; Gormolysova, E V; Uzhakova, E K

    2017-01-01

    Resection of anterior clinoidal meningiomas is a challenging task due to their localization, frequent involvement of the major cerebral arteries and cranial nerves, a high risk of postoperative neurological deficits, and low radicalness of surgery.

  4. Cervical spinal meningioma mimicking intramedullary spinal tumor.

    PubMed

    Senturk, Senem; Guzel, Aslan; Guzel, Ebru; Bayrak, Aylin Hasanefendioğlu; Sav, Aydin

    2009-01-01

    Case report. To report a very unusual spinal meningioma, mimicking an intramedullary spinal tumor. Spinal meningiomas, usually associated with signs and symptoms of cord or nerve root compression, are generally encountered in women aged over 40. Radiologic diagnosis is often established by their intradural extramedullary location on magnetic resonance images. A 60-year-old woman had a 6-month history of progressive weakness in her upper extremities, difficulty in walking, and cervical pain radiating through both arms. Neurologic examination revealed motor strength deficiency in all her extremities, with extensor reflexes, clonus, and bilateral hyper-reflexiveness. A sensory deficit was present all over her body. Magnetic resonance images revealed that the spinal cord appeared expanded with an ill-defined, homogeneously contrast-enhanced, lobulated, eccentric mass at the C1-C3 level. The patient was operated with a preliminary diagnosis of an intramedullary tumor. At surgery, the mass was found to be extramedullary, and gross total resection was performed. Histopathological examination revealed a meningioma characterized by the presence of fibrous and meningothelial components. The patient was able to ambulate with a cane, and extremity strength and sensation improved 2 months after surgery. Spinal meningiomas can mimic intramedullary tumors, and should be considered in differential diagnosis of intradural tumors with atypical appearance.

  5. Disseminated extracranial metastatic meningioma.

    PubMed

    Chua, Felicia H Z; Low, Sharon Y Y; Tham, Chee K; Ding, Cristine; Wong, Chin F; Nolan, Colum P

    2016-11-01

    Meningiomas are usually low-grade, solitary lesions that rarely metastasize. In this group of central nervous system tumours, the higher grade subtypes are notorious for resistance to conventional chemo-radiation therapies. Recent studies have shown efficacy in the use of bevacizumab in patients with recurrent and, or progressive anaplastic meningioma. The authors report a case of a young patient with recurrent anaplastic meningioma who despite being treated with bevacizumab, progressed with disease dissemination to multiple extracranial sites. Although the majority of meningiomas are amendable to treatment, the higher grade subtypes remain therapeutically challenging. The unexpected resistance to anti-angiogenic therapy in this patient adds another layer of complexity to an elusive subset of a supposedly benign disease. This patient report reflects the need for in-depth studies, molecular characterization and overall, better disease understanding in order to improve prognosis for affected patients. Copyright © 2016 Elsevier Ltd. All rights reserved.

  6. Management of Spinal Meningiomas.

    PubMed

    Ravindra, Vijay M; Schmidt, Meic H

    2016-04-01

    Spinal meningiomas are the most common spinal tumors encountered in adults, and account for 6.5% of all craniospinal tumors. The treatment for these lesions is primarily surgical, but emerging modalities may include chemotherapy and radiosurgery. In this article, the current management of spinal meningiomas and the body of literature surrounding conventional treatment is reviewed and discussed. Copyright © 2016 Elsevier Inc. All rights reserved.

  7. Linear accelerator radiosurgery for cavernous sinus meningiomas.

    PubMed

    Kimball, Matthew M; Friedman, William A; Foote, Kelly D; Bova, Frank J; Chi, Yueh-Yun

    2009-01-01

    In this paper, the authors review the results of a single-center experience using linear accelerator (LINAC) radiosurgery for the treatment of cavernous sinus meningiomas. This is a retrospective analysis with a median follow-up of 50 months. All patients were treated on an outpatient basis. Fifty-five patients were treated and 6 patients were lost to follow-up. Changes in preradiosurgery cranial nerve deficits and symptoms as well as actuarial local tumor control were evaluated. The actuarial local tumor control was 100% at 5 years and 98% at 10 years. One patient had enlargement of tumor. Sixty-five percent had improvement in preradiosurgery cranial nerve deficits, 31% were unchanged and 1 patient (3.5%) was worse. Only 1 patient developed a new neurologic deficit. This is the largest LINAC radiosurgery experience for cavernous sinus meningiomas reported to date. Radiosurgery appears to offer greatly superior tumor control and much lower morbidity than surgical resection of cavernous sinus meningiomas. (c) 2009 S. Karger AG, Basel.

  8. Lipomatous meningioma: A rare subtype of benign metaplastic meningiomas

    PubMed Central

    Yüksel, Mehmet Onur; Gürbüz, Mehmet Sabri; Tanrıverdi, Osman; Özmen, Sevilay Akalp

    2017-01-01

    Lipomatous meningiomas are extremely rare subtypes of benign meningiomas and are classified as metaplastic meningioma in the World Health Organization classification. We present a 77-year-old man presented with the history of a gradually intensifying headache for the last 3 months. A right frontoparietal mass was detected on his cranial magnetic resonance imaging. The patient was operated on via a right frontoparietal craniotomy, and histopathological diagnosis was lipomatous meningioma. Distinctive characteristics of lipomatous meningiomas were discussed with special emphasis to importance of immunohistochemical examinations, particularly for its differentiation from the tumors showing similar histology though having more aggressive character. PMID:28149104

  9. The effects of tumor location on diagnostic criteria for canine malignant peripheral nerve sheath tumors (MPNSTs) and the markers for distinction between canine MPNSTs and canine perivascular wall tumors.

    PubMed

    Suzuki, S; Uchida, K; Nakayama, H

    2014-07-01

    Canine malignant peripheral nerve sheath tumors (MPNSTs) occur not only in the peripheral nervous system (PNS) but also in soft tissue and various organs (non-PNS). The most important diagnostic criterion is proof of peripheral nerve sheath origin. This is difficult in non-PNS MPNSTs, and its differential diagnosis is challenging. Canine perivascular wall tumors (PWTs) also commonly arise in soft tissue. Their histopathological features are quite similar to those of canine MPNSTs, making their differential diagnosis challenging. To elucidate whether the morphological features are applicable to diagnose non-PNS MPNSTs and to demonstrate useful markers for distinction between canine MPNSTs and PWTs, the authors examined 30 canine MPNSTs and 31 PWTs immunohistochemically for S100, nestin, NGFR, Olig2, claudin-1, CD57, PRX, α-SMA, desmin, and calponin. Among canine MPNSTs, the PNS tumors displayed significantly higher S100 and Olig2 expression than the non-PNS tumors. The expression levels of the other markers did not differ significantly, suggesting that the same morphological diagnostic criteria are applicable regardless of their location. The PWT cells displayed significantly weaker immunoreactivity than MPNSTs to markers used except α-SMA and desmin. Cluster analysis sorted most canine MPNSTs and PWTs into 2 distinctly different clusters, whereas 3 MPNSTs and 6 PWTs were assigned to the opposing cluster. These 3 MPNSTs were negative for almost all markers, while these 6 PWTs were positive for only neuronal markers. In particular, NGFR and Olig2 were almost negative in the rest of PWT cases. These findings suggest that NGFR and Olig2 are useful to distinguish these 2 tumors.

  10. Large spinal meningioma with hemorrhage after selective root block in the thoraco-lumbar spine.

    PubMed

    Lee, Heui Seung; Lee, Sang Hyung; Chung, Young Seob; Yang, Hee-Jin; Son, Young-Je; Park, Sung Bae

    2013-12-01

    Spinal meningioma accounts for 25% of all spinal cord tumors and occurs mostly in the thoracic region. Spontaneous intraspinal bleeding associated with spinal meningioma has rarely been reported. Most cases of hemorrhage associated with meningiomas are extratumoral and subarachnoid, whereas subdural and intratumoral hemorrhage cases have been reported to occur rarely. We experienced a case of a 58-year-old woman with thoracolumbar spinal meningioma accompanied by intraspinal subdural hematoma. She presented with progressively worsened back pain and newly developed weakness in the right lower extremity after a selective nerve root block in the lumbar spine. She underwent the operation and progressively showed neurological recovery during the postoperative course. We report a thoracolumbarspinal meningioma with subdural and intratumoral hemorrhage after a spinal procedure that caused a sudden neurological deterioration.

  11. Large Spinal Meningioma with Hemorrhage after Selective Root Block in the Thoraco-Lumbar Spine

    PubMed Central

    Lee, Heui Seung; Lee, Sang Hyung; Chung, Young Seob; Yang, Hee-Jin; Son, Young-Je

    2013-01-01

    Spinal meningioma accounts for 25% of all spinal cord tumors and occurs mostly in the thoracic region. Spontaneous intraspinal bleeding associated with spinal meningioma has rarely been reported. Most cases of hemorrhage associated with meningiomas are extratumoral and subarachnoid, whereas subdural and intratumoral hemorrhage cases have been reported to occur rarely. We experienced a case of a 58-year-old woman with thoracolumbar spinal meningioma accompanied by intraspinal subdural hematoma. She presented with progressively worsened back pain and newly developed weakness in the right lower extremity after a selective nerve root block in the lumbar spine. She underwent the operation and progressively showed neurological recovery during the postoperative course. We report a thoracolumbarspinal meningioma with subdural and intratumoral hemorrhage after a spinal procedure that caused a sudden neurological deterioration. PMID:24891860

  12. Primary pulmonary meningioma

    PubMed Central

    Huang, Shucheng; Chen, Li; Mao, Yuping; Tong, Hongwei

    2017-01-01

    Abstract Rationale: Primary extracranial meningiomas are rare outside the head and neck region. Patient concerns: A 44-year-old female patient had chest pain for more than 1 year. Diagnoses: Preoperative chest computed tomography (CT) scan revealed a nodule in the right lower lobe, 1.8 cm in diameter. Tumor tissues were examined by immunohistochemistry for vimentin and S-100. Interventions: Histopathologically, the tumor was characterized by whorled nests of spindle-shaped cells accompanied by psammoma bodies. Immunohistochemistry demonstrated tumor cell positivity for vimentin and S-100. This case was diagnosed as a primary pulmonary meningioma. The tumor was removed by a thoracoscopic pulmonary wedge resection. Outcomes: Postoperative cranial and spinal CT scan did not show any intracranial or spinal mass. No recurrence of the tumor was reported at the time of writing up this case report. Lessons: A primary pulmonary meningioma should be considered in the differential diagnosis workup of pulmonary nodules. PMID:28489736

  13. Definitive Embolization of Meningiomas

    PubMed Central

    Bateman, B.T.; Lin, E.; Pile-Spellman, J.

    2005-01-01

    Summary This review examines the possible role for definitive embolization as a primary therapy for intracranial meningiomas. Surgery or radiosurgery are currently considered the standard of care for most benign meningiomas. However, each of these carries substantial risks. The perioperative mortality for surgical resection, as reported in large series, is between 3.7-9.4%; these studies report a similarly high rate of new neurological deficits following surgery. The rate of complications from radiosurgery is reported between 2-16% and it may take months to years before improvement in symptoms occurs following this therapy. There are a few reports of treating meningiomas by embolization without subsequent surgery. While these studies include small numbers of patients and have limited follow-up, the initial results are very promising. Given the risks and limitations of surgery and radiosurgery, prospective trials are now needed to determine the safety and efficacy of definitive embolization. PMID:20584499

  14. Cervical Extradural Meningioma: Case Report and Literature Review

    PubMed Central

    Frank, Brian L; Harrop, James S; Hanna, Amgad; Ratliff, John

    2008-01-01

    Background: Extradural lesions are most commonly metastatic neoplasms. Extradural meningioma accounts for 2.7 to 10% of spinal neoplasms and most commonly is found in the thoracic spine. Design: Case report. Findings: A 45-year-old woman presented with posterior cervicothoracic pain for 8 months following a motor vehicle crash. Magnetic resonance imaging of the cervical spine revealed an enhancing epidural mass. Computerized tomography of the chest, abdomen, and pelvis revealed no systemic disease. Due to the lesion's unusual signal characteristics and location, an open surgical biopsy was completed, which revealed a psammomatous meningioma. Surgical decompression of the spinal cord and nerve roots was then performed. The resection was subtotal due to the extension of the tumor around the vertebral artery. Conclusion: Meningiomas should be considered in the differential diagnosis of contrast-enhancing lesions in the cervical spine. PMID:18795481

  15. Extracalvarial Meningioma in the Parapharyngeal Space: Presentation, Diagnosis and Management

    PubMed Central

    Dutta, Sirshak; Dutta, Mainak; Ghosh, Saurav Kumar; Sinha, Ramanuj

    2016-01-01

    Extracalvarial meningioma in the cervical region presenting as a parapharyngeal mass lesion is seldom encountered in clinical practice and poses great challenge in its diagnosis and surgical management. In this report, we present a case of extracranial meningioma in a middle-aged person who presented with a large, gradually progressing cervical swelling with multiple cranial nerve pareses. The difficulties in diagnosis and surgical management of this unusual neoplasm in the setting of partial encasement and thrombosis of the internal jugular vein have been discussed, along with the computed tomography and magnetic resonance imaging providing details of its extent and character. The report emphasizes the need to consider extracalvarial meningioma as a less common but important differential diagnosis of parapharyngeal space neoplasms. PMID:28208895

  16. Management of Intracranial Meningiomas Using Keyhole Techniques

    PubMed Central

    Burks, Joshua D; Conner, Andrew K; Bonney, Phillip A; Archer, Jacob B; Christensen, Blake; Smith, Jacqueline; Safavi-Abbasi, Sam

    2016-01-01

    Background: Keyhole craniotomies are increasingly being used for lesions of the skull base. Here we review our recent experience with these approaches for resection of intracranial meningiomas. Methods: Clinical and operative data were gathered on all patients treated with keyhole approaches by the senior author from January 2012 to June 2013. Thirty-one meningiomas were resected in 27 patients, including 9 supratentorial, 5 anterior fossa, 7 middle fossa, 6 posterior fossa, and 4 complex skull base tumors. Twenty-nine tumors were WHO Grade I, and 2 were Grade II.  Results: The mean operative time was 8 hours, 22 minutes (range, 2:55-16:14) for skull-base tumors, and 4 hours, 27 minutes (range, 1:45-7:13) for supratentorial tumors. Simpson Resection grades were as follows: Grade I = 8, II = 8, III = 1, IV = 15, V = 0. The median postoperative hospital stay was 4 days (range, 1-20 days). In the 9 patients presenting with some degree of visual loss, 7 saw improvement or complete resolution. In the 6 patients presenting with cranial nerve palsies, 4 experienced improvement or resolution of the deficit postoperatively. Four patients experienced new neurologic deficits, all of which were improved or resolved at the time of the last follow-up. Technical aspects and surgical nuances of these approaches for management of intracranial meningiomas are discussed.  Conclusions: With careful preoperative evaluation, keyhole approaches can be utilized singly or in combination to manage meningiomas in a wide variety of locations with satisfactory results. PMID:27284496

  17. Posterior clinoid meningioma: A case report with discussion on terminology and surgical approach

    PubMed Central

    Sodhi, Harsimrat Bir S.; Singla, Navneet; Gupta, Sunil K.

    2015-01-01

    Background: Posterior clinoid process (PCP) meningiomas are rare lesions. In close proximity to these lesions are the perforators from internal carotid artery and the oculomotor nerve, which need to be considered while deciding the appropriate surgical approach. Case Description: We describe a basal frontotemporo-orbito-zygomatic approach with splitting of the sylvian fissure to resect an eccentrically placed PCP meningioma. Conclusion: A basal frontotemporo-orbito-zygomatic approach with splitting of the sylvian fissure is a safe approach to resect an eccentrically placed PCP meningioma. PMID:25722927

  18. Surgical Treatment for Falcotentorial Meningiomas

    PubMed Central

    Hong, Chang Ki; Hong, Je Beom; Park, Hunho; Moon, Ju Hyung; Chang, Jong Hee; Lee, Kyu Sung

    2016-01-01

    Among intracranial meningiomas, falcotentorial meningiomas, occurring at the junction of the falx cerebri and tentorial dural folds, are extremely rare. Because of their deep location, they are surrounded by critical structures, and have been regarded as one of the most challenging lesions for surgical treatment. In this study, we describe our surgical strategy for falcotentorial meningiomas and provide a review of our experience. PMID:27189300

  19. Epidural Cystic Spinal Meningioma

    PubMed Central

    Zhang, Ji; Chen, Zheng-he; Wang, Zi-feng; Sun, Peng; Jin, Jie-tian; Zhang, Xiang-heng; Zhao, Yi-ying; Wang, Jian; Mou, Yong-gao; Chen, Zhong-ping

    2016-01-01

    Abstract Cystic spinal meningioma (CSM) is an uncommon meningioma variant. Extradural CSMs are particularly rare and difficult to distinguish from other intraaxial tumors. This study presents a case of a 36-year-old woman with intraspinal extradual CSM at the thoracolumbar spine. She experienced persistent weakness, progressive numbness, and sensory disturbance in the right lower limb. Magnetic resonance imaging (MRI) of the patient revealed an irregular cystic mass at the thoracic 11 to lumbar 3 levels dorsally. This case was misdiagnosed as other neoplasms prior to surgery because of the atypical radiographic features and location of the tumor. Extradural CSMs should be considered in the differential diagnosis of intraspinal extradural cystic neoplasms. Complete removal of cystic wall provides an optimal outcome, rendering the lesion curable. PMID:26986119

  20. Third ventricular meningiomas.

    PubMed

    Li, Puxian; Diao, Xingtao; Bi, Zhiyong; Hao, Shuyu; Ren, Xiaohui; Zhang, Junting; Xing, Jun

    2015-11-01

    We report 13 patients with third ventricular meningiomas (TVM) and discuss the clinical, radiological, pathological and surgical features, as well as follow-up of these tumors. TVM are rare intracranial tumors, and because of this, there are few reports in the literature. Of 11,600 intracranial meningiomas that were surgically treated and pathologically confirmed at Beijing Tian Tan Hospital over a period of 10 years (2003-2013), 13 TVM were selected for a retrospective review. We recorded the clinical, radiological, pathological, and surgical data and statistically analyzed the preoperative, postoperative and 6 month postoperative Karnofsky performance scale (KPS) scores. TVM represented 0.11% of intracranial meningiomas. Radiologically, TVM were divided into three groups: anterior (n=3), posterior (n=3), and entire third ventricle (n=7). Three patients (23.1%) were misdiagnosed preoperatively. Total removal was achieved in 61.5% (8/13) of patients, and subtotal resection was achieved in 38.5% (5/13). Pathologically, the tumors were World Health Organization (WHO) Grade I in 11 patients (84.6%) and WHO Grade II in two (15.6%). There were no statistically significant differences in the preoperative, postoperative, or 6 month postoperative KPS scores (F=0.814; p=0.401). TVM without dural attachments are rare neoplasms that should be differentiated from choroid plexus papilloma, craniopharyngioma, and pineocytoma. Surgery is the optimal treatment and may result in a favorable prognosis, and understanding of the radiological subtype can help with the choice of surgical approach.

  1. Meningioma in Down Syndrome.

    PubMed

    Yamamoto, Takahiro; Shinojima, Naoki; Todaka, Tatemi; Nishikawa, Shigeyuki; Yano, Shigetoshi; Kuratsu, Jun-ichi

    2015-09-01

    Down syndrome comprises multiple malformations and is due to trisomy of chromosome 21. There is epidemiologic evidence that individuals with Down syndrome are at decreased risk for solid tumors including brain tumors. It has been suggested that some genes expressed on the extra copy of chromosome 21 act as tumor suppressor genes and contribute to protection against tumorigenesis. We report the first case to our knowledge of a patient with Down syndrome, an 8-year-old boy, with an intracranial meningioma, in which the status of chromosome 21 was examined. The diagnosis was based on histologic examination of the surgically resected tumor. Postoperatively, the patient's neurologic status improved, and there was no tumor regrowth in the next 2 years. Fluorescence in situ hybridization for chromosome 22 confirmed high allele loss involving the neurofibromin 2 gene locus, a finding typical in meningiomas. Fluorescence in situ hybridization also revealed chromosome 21 heterogeneity in tumor cells; not only cells with trisomy 21 but also cells with disomy and monosomy 21 were present. All blood cells from the patient manifested trisomy 21. Deletion of the chromosome 21 allele may be associated with tumorigenesis of meningioma in Down syndrome. This supports the hypothesis that some genes whose expression is increased on the extra copy of chromosome 21 function as tumor suppressor genes and that they contribute to the reduced tumor incidence in individuals with Down syndrome. Copyright © 2015 Elsevier Inc. All rights reserved.

  2. Epidemiology and etiology of meningioma

    PubMed Central

    Wrensch, Margaret; Claus, Elizabeth B.

    2010-01-01

    Although most meningiomas are encapsulated and benign tumors with limited numbers of genetic aberrations, their intracranial location often leads to serious and potentially lethal consequences. They are the most frequently diagnosed primary brain tumor accounting for 33.8% of all primary brain and central nervous system tumors reported in the United States between 2002 and 2006. Inherited susceptibility to meningioma is suggested both by family history and candidate gene studies in DNA repair genes. People with certain mutations in the neurofibromatosis gene (NF2) have a very substantial increased risk for meningioma. High dose ionizing radiation exposure is an established risk factor for meningioma, and lower doses may also increase risk, but which types and doses are controversial or understudied. Because women are twice as likely as men to develop meningiomas and these tumors harbor hormone receptors, an etiologic role for hormones (both endogenous and exogenous) has been hypothesized. The extent to which immunologic factors influence meningioma etiology has been largely unexplored. Growing emphasis on brain tumor research coupled with the advent of new genetic and molecular epidemiologic tools in genetic and molecular epidemiology promise hope for advancing knowledge about the causes of intra-cranial meningioma. In this review, we highlight current knowledge about meningioma epidemiology and etiology and suggest future research directions. PMID:20821343

  3. Communication through plasma sheaths

    SciTech Connect

    Korotkevich, A. O.; Newell, A. C.; Zakharov, V. E.

    2007-10-15

    We wish to transmit messages to and from a hypersonic vehicle around which a plasma sheath has formed. For long distance transmission, the signal carrying these messages must be necessarily low frequency, typically 2 GHz, to which the plasma sheath is opaque. The idea is to use the plasma properties to make the plasma sheath appear transparent.

  4. [Extradural spinal meningioma: case report].

    PubMed

    Dagain, A; Dulou, R; Lahutte, M; Dutertre, G; Pouit, B; Delmas, J-M; Camparo, P; Pernot, P

    2009-12-01

    We report a case of purely extradural spinal meningioma and discuss the potential pitfalls in differential diagnosis. Spinal meningiomas account for 20-30% of all spinal neoplasms. Epidural meningiomas are infrequent intraspinal tumors that can be easily confused with malignant neoplasms or spinal schwannomas. A 62-year-old man with a previous history of malignant disease presented with back pain and weakness of the lower limbs. Magnetic resonance imaging revealed a well-enhanced T4 intraspinal lesion. The intraoperative histological examination showed a meningioma (confirmed by postoperative examination). Opening the dura mater confirmed the purely epidural location of the lesion. The postoperative course was uneventful with no recurrence 12 months after surgery. Purely extradural spinal meningiomas can mimic metastatic tumors or schwannomas. Intraoperative histology is mandatory for optimal surgical decision making.

  5. Bevacizumab in Treating Patients With Recurrent or Progressive Meningiomas

    ClinicalTrials.gov

    2017-03-01

    Acoustic Schwannoma; Adult Anaplastic Meningioma; Adult Ependymoma; Adult Grade I Meningioma; Adult Grade II Meningioma; Adult Meningeal Hemangiopericytoma; Adult Papillary Meningioma; Neurofibromatosis Type 1; Neurofibromatosis Type 2; Recurrent Adult Brain Tumor

  6. Unilateral vocal cord palsy and dysphagia: an unusual presentation of cerebellopontine angle meningioma

    PubMed Central

    Senior, Andrew; Douglas, James Andrew; Thompson, Stuart

    2015-01-01

    Cerebellopontine angle (CPA) tumours are the most common neoplasms in the posterior fossa, accounting for 5–10% of intracranial tumours. Most CPA tumours are benign, with most being vestibular schwannomas. Meningiomas arising from the jugular foramen are among the rarest of all with very few being described in the literature. Treatment options vary considerably as experience with these tumours is limited. One option is a skull base approach, but this depends on size, location and ability to preserve lower cranial nerve function. This can be extremely challenging and is accompanied by high mortality risk; therefore, a more conservative option must be considered. This case report highlights the difficulty in management of patients with jugular fossa meningiomas, including appropriate investigations, analysis of surgical versus conservative treatment and associated complications. Furthermore, we elaborate the decision-making process pertaining to the tailoring of the surgical route used for the resection of jugular foramen meningiomas. (Jugular Foramen Meningioma, cerebellopontine angle). PMID:26486157

  7. Linear accelerator-based stereotactic radiosurgery of intracranial meningiomas: results of the first 5 years of clinical practice.

    PubMed

    Abdelaziz, Osama S; Kandil, Alaa; El-Assaal, Shaaban; Abdelaziz, Amro; Rostom, Yosry; Rashed, Yaser

    2011-01-01

    Meningiomas are mostly benign but some are atypical or malignant. Surgical resection is curative when complete removal of benign meningiomas is contemplated. Incompletely excised and recurrent tumors are frequently treated with fractionated radiation therapy or stereotactic radiosurgery. The purpose of this study is to evaluate the short-term radiological and functional outcomes of a single center using linear accelerator (Linac) stereotactic radiosurgery for the treatment of intracranial meningiomas. Twenty-nine patients (12 males and 17 females) with 30 meningiomas, in different brain locations (skull base and non-skull base meningiomas), were treated with Linac-based stereotactic radiosurgery. The mean tumor volume was 6.3 cm³, and the mean tumor marginal and maximum doses were 10.9 and 15 Gy, respectively. The median prescribed isodose line was 80%. The patients were followed-up for a minimum of 3 years. Regarding radiological outcome, nine (30%) meningiomas demonstrated evident volume reduction, 19 (63.3%) meningiomas remained unchanged, and two (6.7%) meningiomas increased in size after radiosurgery. The local tumor control rates for skull base meningiomas and non-skull base meningiomas after radiosurgery were 90.9% and 100%, respectively. Regarding functional outcomes, 64% of patients presenting with cranial neuropathies showed improvement of their cranial nerve functions and 29% of patients remained unchanged. One patient had temporary trigeminal neuropathy. Although radiosurgery for meningiomas is generally effective and quite safe in achieving high control rates with minimum morbidity over short- and intermediate-term periods of follow-up, tumor progression might occur in a delayed manner after initial apparent control for few years. We recommend continued follow-up for longer periods to better assess the long-term outcomes.

  8. Metastasis to a spinal meningioma.

    PubMed

    Bansil, Rohit; Walia, Bipin S; Khan, Zahid; Abrari, Andleeb

    2017-01-01

    Metastasis of one cancer to another is rare. Here, we report a spinal meningioma that was infiltrated by metastatic deposits from another cancer. A 62-year-old male presented with a progressive spastic paraparesis. Magnetic resonance (MR) imaging of the spine suggested a well-defined intradural extramedullary (IDEM) T8 mass in the dorsal spinal canal. When excised, it proved histologically to be a meningothelial meningioma infiltrated by metastatic deposits from an adenocarcinoma. Tumor to tumor metastasis rarely occurs, and meningioma, owing to its biological character and increased vascularity, is one of the most common recipients of a metastases from other lesions.

  9. Frontal cutaneous meningioma - Case report*

    PubMed Central

    Ramos, Leonor; Coutinho, Ines; Cardoso, José Carlos; Garcia, Helena; Cordeiro, Margarida Robalo

    2015-01-01

    Cutaneous meningiomas are rare tumors most commonly located on the scalp. We report the case of a 55-year-old male who presented with a 2x3 cm tumoral lesion on the forehead. The lesion was hard, adherent and covered by normal skin. Incisional biopsy revelead a proliferation of monomorphic round cells, organized in nests and focally forming pseudovascular spaces. Immunohistochemical study revealed positivity for epithelial antigen membrane and vimentin. Vascular markers, cytokeratins and S100 protein were negative. A brain CT scan did not show any evidence of intracranial meningioma. The authors describe the case of a cutaneous frontal meningioma in probable relation with previous cranioencephalic trauma. PMID:26312695

  10. Intraorbital meningioma: resection through modified orbitozygomatic craniotomy.

    PubMed

    Cohen-Gadol, Aaron A

    2012-01-01

    Intraorbital meningiomas are challenging lesions to excise because of their location and the restricted surgical corridor available due to the presence of important neighboring structures. Lesions located in the posterior one-third of the orbit require skull base approaches for their exposure and safe resection. Frontoorbital and modified orbitozygomatic (OZ) craniotomies may facilitate the exposure and resection of masses in the posterior intraorbital space. Specifically, the one-piece modified OZ craniotomy provides many advantages of the "full" OZ craniotomy (which includes a more extensive zygomatic osteotomy). The modified OZ approach minimizes the extent of frontal lobe retraction and provides ample amount of space for the surgeon to exploit all the working angles to resect the tumor. The following video presentation discusses the nuances of technique for resection of an intraorbital meningioma through modified OZ approach and optic nerve decompression. The nuances of technique will be discussed. The video can be found here: http://youtu.be/fP5X2QNr5qk.

  11. Case Report: Pulmonary metastases of malignant meningioma

    PubMed Central

    Basunaid, Suhail; Franssen, Frits M.E.; Accord, Ryan; Hamid, Myrurgia Abdul; Mahesh, Shekar; Baumert, Brigitta G.; Schijns, Olaf E.M.G.

    2014-01-01

    Meningioma accounts for approximately one-third of primary central nervous system tumors. Most meningiomas are benign, although up to one third are classified as atypical or malignant. We describe a 63-year Caucasian male presenting with pleural metastases from an intracranial meningioma. Distant metastases from meningiomas are infrequently found in clinical practice and mostly are associated with atypical or malignant meningiomas. There is no standard treatment; however surgical resection of both the primary and metastatic lesions is the safest therapy. The overall prognosis of atypical meningiomas is poor. Our patient died one week after discharge from our hospital. PMID:25254095

  12. Giant sacrolumbar meningioma. Case report.

    PubMed

    Feldenzer, J A; McGillicuddy, J E; Hopkins, J W

    1990-06-01

    A case of giant sacral meningioma with presacral and lumbar extension is presented. The difficulties in diagnosis and management are emphasized including the staged multidisciplinary surgical approaches and preoperative tumor embolization.

  13. [Chronic epidural haematoma mimicking meningioma].

    PubMed

    Beculić, Hakija; Skomorac, Rasim; Jusić, Aldin; Mekić-Abazović, Alma; Bajtarević, Alma

    2011-02-01

    The study presents a rare case of organised chronic epidural haematoma that imitated a meningioma. A patient was admitted to the Department of Neurology of the Cantonal Hospital Zenica due to loss of consciousness and right hemiparesis. Non-contrast Computed Tomography (CT) scan had shown an expansive intracranial process in the left parietal region which was radiologically diagnosed as a meningioma. During the operation a linear skull fracture and organised chronic epidural haematoma were found.

  14. Intraparenchymal Angiomatous Meningioma: A Diagnostic Dilemma

    PubMed Central

    Bansal, Divya; Gogoi, Priyanka; Nazir, Wazid; Tandon, Anupama

    2015-01-01

    Meningioma arises from the arachnoid cap cells of the cerebrum. Intraparenchymal meningiomas or meningiomas without dural attachment are rare. We report a case of 40-year-old male who presented with a history of headache, dizziness and gradual loss of vision since one year. Clinicoradiological diagnosis of a high grade glioma was considered. Tumour was excised and haematoxylin and eosin stained sections revealed a tumour comprised predominantly of variable sized blood vessels showing hyalinization in a background of plump spindle cells with oval vesicular nuclei. In view of these features angiomatous meningioma was suspected. However, to confirm the diagnosis, a panel of immunohistochemical markers including vimentin, EMA and GFAP was done and a final diagnosis of angiomatous meningioma was offered. Angiomatous meningioma is a rare variant of meningioma and even much rarer in the intraparenchymal location. Angiomatous meningioma should be considered in the differential diagnosis of highly vascular intraparenchymal brain tumours. PMID:26557529

  15. Intraparenchymal Angiomatous Meningioma: A Diagnostic Dilemma.

    PubMed

    Bansal, Divya; Diwaker, Preeti; Gogoi, Priyanka; Nazir, Wazid; Tandon, Anupama

    2015-10-01

    Meningioma arises from the arachnoid cap cells of the cerebrum. Intraparenchymal meningiomas or meningiomas without dural attachment are rare. We report a case of 40-year-old male who presented with a history of headache, dizziness and gradual loss of vision since one year. Clinicoradiological diagnosis of a high grade glioma was considered. Tumour was excised and haematoxylin and eosin stained sections revealed a tumour comprised predominantly of variable sized blood vessels showing hyalinization in a background of plump spindle cells with oval vesicular nuclei. In view of these features angiomatous meningioma was suspected. However, to confirm the diagnosis, a panel of immunohistochemical markers including vimentin, EMA and GFAP was done and a final diagnosis of angiomatous meningioma was offered. Angiomatous meningioma is a rare variant of meningioma and even much rarer in the intraparenchymal location. Angiomatous meningioma should be considered in the differential diagnosis of highly vascular intraparenchymal brain tumours.

  16. A Pilocytic Astrocytoma Mimicking a Clinoidal Meningioma

    PubMed Central

    Hong, Christopher S.; Lehman, Norman L.; Sauvageau, Eric

    2014-01-01

    Pilocytic astrocytomas and meningiomas are benign, primary brain tumors that may involve the optic tract. Classically, the presence of a dural “tail” sign may differentiate a meningioma from other intracranial lesions. In this report, we describe a mass with the typical appearance of a clinoidal meningioma on magnetic resonance imaging (MRI) but postoperatively diagnosed as a pilocytic astrocytoma. This case illustrates the rare occurrence of a pilocytic astrocytoma mimicking a meningioma on MRI. PMID:24744944

  17. Large and giant skull base meningiomas: The role of radical surgical removal

    PubMed Central

    da Silva, Carlos Eduardo; de Freitas, Paulo Eduardo Peixoto

    2015-01-01

    Background: The large and giant skull base meningiomas are challenging lesions, and the involvement of crucial neurovascular structures needs the surgical removal as the primordial treatment. The authors report on a series of patients with large and giant skull base meningiomas who were treated with the goal of radical removal. Methods: A retrospective study including 49 patients with large and giant skull base meningiomas was carried out. Tumors presenting 3 cm or larger were included. Results: The meningiomas in the sample included the following types: 10 olfactory groove, 8 sphenoorbital, 8 petroclival, 8 tentorial, 4 clinoidal, 4 cavernous sinus, 3 temporal floor, 2 tuberculum sellae and 2 foramen magnum. The average age was 53 years, the mean follow-up period was 52 months, Simpson Grades I and II were obtained in 75.5%. The overall mortality was 5%. Transient cranial nerve deficits occurred in 32% with definite cranial nerve lesion in 18%. Cerebrospinal fluid leak occurred in 14%. Conclusions: The surgical treatment is a mandatory option for large and giant skull base meningiomas. The radical removal is achievable and should be considered an alternative with a good outcome and an acceptable morbidity for such challenge lesions. PMID:26167365

  18. Meningioma after radiotherapy for malignancy.

    PubMed

    Morgenstern, Peter F; Shah, Kalee; Dunkel, Ira J; Reiner, Anne S; Khakoo, Yasmin; Rosenblum, Marc K; Gutin, Philip

    2016-08-01

    Complications of radiation exposure have gained importance with increasing cancer survivorship. Secondary malignancies have been associated with cranial radiation exposure. We present our experience with intracranial radiation-induced meningioma (RIM) and discuss the implications of its presentation and natural history for patient management. Patients diagnosed with meningioma who had received radiation therapy between 1960 and 2014 were identified. Records were retrospectively reviewed for details of radiation exposure, previous malignancies, meningioma subtypes, multiplicity and pathologic descriptions, treatment and follow-up. Thirty patients were diagnosed with RIM. Initial malignancies included acute lymphocytic leukemia (33.3%), medulloblastoma (26.7%) and glioma (16.7%) at a mean age of 8.1years (range 0.04-33years). The mean radiation dose was 34Gy (range 16-60Gy) and latency time to meningioma was 26years (range 8-51years). Twenty-one patients (70%) underwent surgery. Of these, 57.1% of tumors were World Health Organization (WHO) grade I while 42.9% were WHO II (atypical). The mean MIB-1 labeling index for patients with WHO I tumors was 5.44%, with 33.3% exhibiting at least 5% staining. Mean follow-up after meningioma diagnosis was 5.8years. Mortality was zero during the follow-up period. Meningioma is an important long-term complication of therapeutic radiation. While more aggressive pathology occurs more frequently in RIM than in sporadic meningioma, it remains unclear whether this translates into an effect on survival. Further study should be aimed at delineating the risks and benefits of routine surveillance for the development of secondary neoplasms after radiation therapy.

  19. Meningioma after radiotherapy for malignancy

    PubMed Central

    Morgenstern, Peter F.; Shah, Kalee; Dunkel, Ira J.; Reiner, Anne S.; Khakoo, Yasmin; Rosenblum, Marc K.; Gutin, Philip

    2017-01-01

    Complications of radiation exposure have gained importance with increasing cancer survivorship. Secondary malignancies have been associated with cranial radiation exposure. We present our experience with intracranial radiation-induced meningioma (RIM) and discuss the implications of its presentation and natural history for patient management. Patients diagnosed with meningioma who had received radiation therapy between 1960 and 2014 were identified. Records were retrospectively reviewed for details of radiation exposure, previous malignancies, meningioma subtypes, multiplicity and pathologic descriptions, treatment and follow-up. Thirty patients were diagnosed with RIM. Initial malignancies included acute lymphocytic leukemia (33.3%), medulloblastoma (26.7%) and glioma (16.7%) at a mean age of 8.1 years (range 0.04–33 years). The mean radiation dose was 34 Gy (range 16–60 Gy) and latency time to meningioma was 26 years (range 8–51 years). Twenty-one patients (70%) underwent surgery. Of these, 57.1% of tumors were World Health Organization (WHO) grade I while 42.9% were WHO II (atypical). The mean MIB-1 labeling index for patients with WHO I tumors was 5.44%, with 33.3% exhibiting at least 5% staining. Mean follow-up after meningioma diagnosis was 5.8 years. Mortality was zero during the follow-up period. Meningioma is an important long-term complication of therapeutic radiation. While more aggressive pathology occurs more frequently in RIM than in sporadic meningioma, it remains unclear whether this translates into an effect on survival. Further study should be aimed at delineating the risks and benefits of routine surveillance for the development of secondary neoplasms after radiation therapy. PMID:27068012

  20. Characteristics and surgical strategies for posterior clinoid process meningioma: two case reports and review of the literature.

    PubMed

    Takase, Hajime; Kawasaki, Takafumi; Tateishi, Kensuke; Yokoyama, Taka-Akira; Murata, Hidetoshi; Kawahara, Nobutaka

    2017-01-01

    Clinical and radiological features or characteristics of posterior clinoid process (PCP) meningiomas have rarely been described because of their extreme scarcity and terminological confusion. Therefore, the strategies in the surgical intervention for PCP meningiomas have not been well established. Moreover, the presence of deep and critical neuroanatomical structures and relatively high morbidity, which can be difficult to predict preoperatively, make their surgical excision more challenging. We report two surgical cases of PCP meningioma and discuss the appropriate assessment of preoperative features and surgical strategies with review of the literature. Our study suggests that PCP meningioma may be characterized by the anterior displacement of internal carotid artery, and infero-laterally shifted posterior communicating arteries, and homonymous hemianopsia, a distinctive clinical feature. One of the key issues in PCP meningioma surgery is preservation of the optic nerve. Unlocking the optic nerve by anterior clinoidectomy and dissection, the falciform ligament is the important step to preserve vision for larger tumors. Complication with the perforators is also hazardous of these challenging surgeries than anterior clinoid meningiomas for their specific neuroanatomical structures and might not be feasible to avoid even with additional techniques and critical monitoring. A combination and multi-staged-surgical approach can be options of tailor-made surgical strategy in cases with tumor adhesion to the perforators.

  1. Genomic landscape of high-grade meningiomas

    PubMed Central

    Bi, Wenya Linda; Greenwald, Noah F.; Abedalthagafi, Malak; Wala, Jeremiah; Gibson, Will J.; Agarwalla, Pankaj K.; Horowitz, Peleg; Schumacher, Steven E.; Esaulova, Ekaterina; Mei, Yu; Chevalier, Aaron; Ducar, Matthew; Thorner, Aaron R.; van Hummelen, Paul; Stemmer-Rachamimov, Anat; Artyomov, Maksym; Al-Mefty, Ossama; Dunn, Gavin P.; Santagata, Sandro; Dunn, Ian F.; Beroukhim, Rameen

    2017-01-01

    High-grade meningiomas frequently recur and are associated with high rates of morbidity and mortality. To determine the factors that promote the development and evolution of these tumors, we analyzed the genomes of 134 high-grade meningiomas and compared this information with data from 587 previously published meningiomas. High-grade meningiomas had a higher mutation burden than low-grade meningiomas but did not harbor any statistically significant mutated genes aside from NF2. High-grade meningiomas also possessed significantly elevated rates of chromosomal gains and losses, especially among tumors with monosomy 22. Meningiomas previously treated with adjuvant radiation had significantly more copy number alterations than radiation-induced or radiation-naïve meningiomas. Across serial recurrences, genomic disruption preceded the emergence of nearly all mutations, remained largely uniform across time, and when present in low-grade meningiomas, correlated with subsequent progression to a higher grade. In contrast to the largely stable copy number alterations, mutations were strikingly heterogeneous across tumor recurrences, likely due to extensive geographic heterogeneity in the primary tumor. While high-grade meningiomas harbored significantly fewer overtly targetable alterations than low-grade meningiomas, they contained numerous mutations that are predicted to be neoantigens, suggesting that immunologic targeting may be of therapeutic value. PMID:28713588

  2. Surgical Resectability of Skull Base Meningiomas

    PubMed Central

    GOTO, Takeo; OHATA, Kenji

    2016-01-01

    With recent advances in surgical technology such as preoperative imaging, neuro-monitoring, and surgical instruments, the surgical resectability of intracranial meningiomas has increased over the last two decades. This study reviewed clinical articles regarding the surgical treatment of meningiomas to clarify the role of surgical excision, with a focus on skull base meningiomas. We sub-classified clinical articles about skull base meningiomas into two categories (anterior and middle fossa meningiomas; and posterior fossa meningiomas) and reviewed papers in each category. In cases with anterior and middle fossa meningiomas, surgical resectability has reached a sufficient level to maximize functional preservation. In cases of posterior fossa meningioma, however, surgical respectability remains insufficient even with full use of recent surgical modalities. Continuous refining of operative procedures is required to obtain more satisfactory outcomes, especially for posterior fossa meningioma. In addition, recent long-term outcomes of stereotactic radiosurgery (SRS) were acceptable for controlling the skull base meningiomas. Therefore, combination with surgical excision and SRS should be considered in complicated skull base meningiomas. PMID:27076382

  3. Totally ossified metaplastic spinal meningioma.

    PubMed

    Ju, Chang Il; Hida, Kazutoshi; Yamauchi, Tomohiro; Houkin, Kiyohiro

    2013-09-01

    A 61-year-old woman with a very rare case of totally ossified large thoracic spinal metaplastic meningioma, showing progressing myelopathy is presented. Computed tomographic images showed a large totally ossfied intradural round mass occupying the spinal canal on T9-10 level. Magnetic resonance imaging revealed a large T9-10 intradural extramedullary mass that was hypointense to spinal cord on T1- and T2-weighted sequences, partial enhancement was apparent after Gadolinium administration. The spinal cord was severely compressed and displaced toward the right at the level of T9-10. Surgical removal of the tumor was successfully accomplished via the posterior midline approach and the histological diagnosis verified an ossified metaplastic meningioma. The clinical neurological symptoms of patient were improved postoperatively. In this article we discuss the surgical and pathological aspects of rare case of spinal totally ossified metaplastic meningioma.

  4. Totally Ossified Metaplastic Spinal Meningioma

    PubMed Central

    Hida, Kazutoshi; Yamauchi, Tomohiro; Houkin, Kiyohiro

    2013-01-01

    A 61-year-old woman with a very rare case of totally ossified large thoracic spinal metaplastic meningioma, showing progressing myelopathy is presented. Computed tomographic images showed a large totally ossfied intradural round mass occupying the spinal canal on T9-10 level. Magnetic resonance imaging revealed a large T9-10 intradural extramedullary mass that was hypointense to spinal cord on T1- and T2-weighted sequences, partial enhancement was apparent after Gadolinium administration. The spinal cord was severely compressed and displaced toward the right at the level of T9-10. Surgical removal of the tumor was successfully accomplished via the posterior midline approach and the histological diagnosis verified an ossified metaplastic meningioma. The clinical neurological symptoms of patient were improved postoperatively. In this article we discuss the surgical and pathological aspects of rare case of spinal totally ossified metaplastic meningioma. PMID:24278660

  5. [Linear accelerator-based stereotactic radiation treatment of patients with medial middle fossa meningiomas].

    PubMed

    Golanov, A V; Cherekaev, V A; Serova, N K; Pronin, I N; Gorlachev, G E; Kotel'nikova, T M; Podoprigora, A E; Kudriavtseva, P A; Galkin, M V

    2010-01-01

    Medial middle fossa meningiomas are challenging for neurosurgical treatment. Invasion of cranial nerves and vessels leads to high risk of complications after removal of such meningiomas. Currently methods of conformal stereotactic radiation treatment are applied wider and wider for the discussed lesions. During a 3.5-year period 80 patients with medial middle fossa meningiomas were treated in Burdenko Moscow Neurosurgical Institute using linear accelerator "Novalis". In 31 case radiation treatment was preceded by surgical resection. In majority of patients symptoms included cranial nerve dysfunction: oculomotor disturbances in 62.5%, trigeminal impairment--in 37.5%, visual deficit--in 43.8%, facial nerve palsy--in 1.25%. 74 patients underwent radiotherapy with classical fractioning, 2--in hypofractionated mode and 4 received radiosurgery. In cases of classical fractioning mean marginal dose reached 46.3 Gy during 28-33 fractions, in hypofractioning (7 fractions)--31.5 Gy, in radiosurgery--16.25 Gy. Mean follow-up period was 18.4 months (6-42 months). Control of tumor growth was achieved in 97.5% of cases (78 patients): in 42 (52.5%) lesion shrinked, in 36 (45%) stabilization was observed. Clinical examination revealed improvement of visual function in 15 patients (18%) and deterioration in 2 (2.5%). No new neuropathies were found. Stereotactic radiation treatment is the method of choice for medial anterior and middle fossa meningiomas due to effective control of tumor progression and minimal rate of complications.

  6. Transpetrosal approach to petro-clival meningioma.

    PubMed

    Elarjani, Turki; Shetty, Rakshith; Singh, Harminder; da Silva, Harley B; Sekhar, Laligam N

    2017-10-01

    A 38-year-old woman had a 3-week gradual onset of right-sided weakness in the upper and lower extremities. MRI showed a large left petro-clival meningioma encasing the basilar and left superior cerebellar artery and compressing the brainstem. A posterior transpetrosal approach, with a left temporal and retrosigmoid craniotomy and mastoidectomy, was performed. The tumor was removed in a gross-total resection with questionable remnants adherent to the brainstem. Intraoperative partial iatrogenic injury to the left oculomotor nerve was repaired with fibrin glue. Postoperatively, the hemiparesis improved, and the patient was discharged to the rehabilitation center with left oculomotor and abducens palsies. A postoperative MRI scan showed complete resection of tumor with no remnants on the brainstem. A 6-month follow-up examination showed complete resolution of motor symptoms and complete recovery of cranial nerve (CN) palsies affecting CN III and CN VI. The video can be found here: https://youtu.be/vOu6YFA8uoo .

  7. Malignant spinal meningioma in a CD-1 mouse.

    PubMed

    Balme, Emmanuelle; Roth, Daniel Robert; Perentes, Elias

    2008-08-01

    Spontaneous meningiomas are extremely rare tumors in small laboratory animals, except in some strains of rats and in the B6C3F1 mouse. We report the case of a male CD-1 mouse in poor health condition, with severe apathy and partial paresis of hindlegs. No macroscopic lesion was noticed at necropsy. Microscopic examination revealed the presence of a malignant meningioma (approximately 3mm in diameter) at the distal lumbar level of the spinal cord, invading the vertebral canal, and bilaterally the ventral and dorsal nerve roots and the dorsal root ganglia. The tumor displayed highly cellular sheets of polygonal cells with a well-delineated, abundant, eosinophilic cytoplasm and scarce fibrovascular stroma; epithelioid and sarcomatous areas were also present. The mitotic activity was moderate. Tumor cells expressed vimentin immunoreactivity and were negative for periodic acid-Schiff (PAS), silver impregnation for reticulin, chromogranin A, glial fibrillary acidic protein (GFAP), cytokeratin (CK) and S-100 protein. The diagnosis of malignant spinal meningioma was based on the morphologic features of the neoplasm, the evidence of local invasion and the immunohistochemical results.

  8. Stereotactic fractionated radiotherapy for the treatment of benign meningiomas

    SciTech Connect

    Candish, Charles; McKenzie, Michael . E-mail: mmckenzi@bccancer.bc.edu; Clark, Brenda G.; Ma, Roy; Lee, Richard; Vollans, Emily; Robar, James; Gete, Ermias; Martin, Monty

    2006-11-15

    Purpose: To assess the use of stereotactic fractionated radiotherapy (SRT) for the treatment of meningiomas. Methods and Materials: Between April 1999 and October 2004, 38 patients underwent SRT. Of 34 patients (36 tumors) assessed, the median age was 53 years. The indication was primary treatment in 26 cases (no histology) and postoperative in 10 cases. The most common sites were cavernous sinus (17), optic nerve (6), and cerebellopontine angle (5). The median gross target volume and planning target volume were 8.9 cm{sup 3} and 18.9 cm{sup 3}, respectively. Stereotactic treatment was delivered with 6-MV photons with static conformal fields (custom-made blocks, 9 patients, and micromultileaf collimator, 25 patients). Median number of fields was six. The median dose prescribed was 50 Gy (range, 45-50.4 Gy) in 28 fractions. The median homogeneity and conformality indices were 1.1 and 1.79, respectively. Results: Treatment was well tolerated. Median follow-up was 26 months with 100% progression-free survival. One patient developed an area of possible radionecrosis related to previous radiotherapy, and 2 men developed mild hypogonadism necessitating testosterone replacement. The vision of 5 of 6 patients with optic pathway meningiomas improved or remained static. Conclusions: Stereotactic fractionated radiotherapy for the treatment of meningiomas is practical, and with early follow-up, seems to be effective.

  9. Pulmonary metastases from benign calvarial meningioma: a case report.

    PubMed

    Cho, Byung-Rae; Yoon, Wan-Soo

    2017-04-01

    The most common intracranial tumour is meningioma, which rarely presents with extracranial metastasis, especially in benign cases. We report a case of meningioma recurrence with multiple pulmonary metastases in a patient who had a benign meningioma removed 12 years prior.

  10. Cystic Meningioma Masquerading as a Metastatic Tumor: A Case Report

    PubMed Central

    Ramanathan, Nithya; Kamaruddin, Khairul Azmi; Othman, Aizzat; Mustafa, Fadhli; Awang, Mohamed Saufi

    2016-01-01

    Cystic meningioma is a rare form of intracranial meningioma. Meningiomas are typically solid tumors but may rarely have cystic components. The diagnosis of cystic meningioma is clinically challenging as the finding of multiple intra-axial tumors, including metastatic tumors, is relatively common. We report a case of cystic meningioma initially diagnosed as a metastatic tumor from a recurrence of acute lymphoid leukemia. However, postoperative histopathological examination demonstrated an atypical meningioma. PMID:27418876

  11. Preoperative particle and glue embolization of meningiomas: indications, results, and lessons learned from 117 consecutive patients.

    PubMed

    Borg, Anouk; Ekanayake, Jinendra; Mair, Richard; Smedley, Thomas; Brew, Stefan; Kitchen, Neil; Samandouras, George; Robertson, Fergus

    2013-12-01

    Preoperative embolization of meningiomas remains contentious, with persisting uncertainty over the safety and efficacy of this adjunctive technique. To evaluate the safety of presurgical embolization of meningiomas and its impact on subsequent transfusion requirement with respect to the extent of embolization and technique used. One hundred seventeen consecutive patients between 2001 and 2010 were referred for embolization of presumed intracranial meningioma before surgical resection. Glue and/or particles were used to devascularize the tumor in 107 patients, all of whom went on to operative resection. The extent and nature of embolization-related complications, degree of angiographic devascularization, and the intraoperative blood transfusion requirements were analyzed. Mean blood transfusion requirement during surgery was 0.8 units per case (range, 1-14 units). Blood transfusion was significantly lower in patients whose meningiomas were completely, angiographically devascularized (P = .035). Four patients had complications as a direct result of the embolization procedure. These included intratumoral hemorrhage in 2, sixth cranial nerve palsy in 1, and scalp necrosis requiring reconstructive surgery in 1 patient. The complication rate was 3.7%. No relationship between the embolic agent and the degree of devascularization was observed. Achieving a complete devascularization resulted in a lower blood transfusion requirement, considered an indirect measure of operative blood loss. This series demonstrates that preoperative meningioma embolization is safe and may reduce operative blood loss. We present distal intratumoral injection of liquid embolic as a safe and effective alternative to more established particle embolization techniques.

  12. Radiation-induced meningiomas in pediatric patients

    SciTech Connect

    Moss, S.D.; Rockswold, G.L.; Chou, S.N.; Yock, D.; Berger, M.S.

    1988-04-01

    Radiation-induced meningiomas rarely have latency periods short enough from the time of irradiation to the clinical presentation of the tumor to present in the pediatric patient. Three cases of radiation-induced intracranial meningiomas in pediatric patients are presented. The first involved a meningioma of the right frontal region in a 10-year-old boy 6 years after the resection and irradiation of a 4th ventricular medulloblastoma. Review of our pediatric tumor cases produced a second case of a left temporal fossa meningioma presenting in a 15-year-old boy with a history of irradiation for retinoblastoma at age 3 years and a third case of a right frontoparietal meningioma in a 15-year-old girl after irradiation for acute lymphoblastic leukemia. Only three cases of meningiomas presenting in the pediatric age group after radiation therapy to the head were detected in our review of the literature.

  13. Recurrent spinal meningioma: a case report.

    PubMed

    Choi, Hoi Jung; Paeng, Sung Hwa; Kim, Sung Tae; Jung, Yong Tae

    2012-09-01

    Meningiomas are the second most common intradural spinal tumors accounting for 25% of all spinal tumors. Being a slow growing and invariably benign tumor, it responds favorably to surgical excision. In addition, spinal meningioma has low recurrence rates. However, we experienced a case of intradural extramedullary spinal meningioma which recurred 16 years after the initial surgery on a 64-year-old woman. She presented with progressive neurological symptoms and had a surgical history of removal of thoracic spinal meningioma 16 years ago due to bilateral low leg weakness. She underwent a second operation at the same site and a pale yellowish tumor was excised, which was histopathologically confirmed as meningothelial meningioma, compared with previously transitional type. she showed neurological recovery after the operation. We, therefore, report the good results of this recurrent intradural spinal meningioma case developed after 16 years with literature review.

  14. Radiation-induced meningiomas in pediatric patients.

    PubMed

    Moss, S D; Rockswold, G L; Chou, S N; Yock, D; Berger, M S

    1988-04-01

    Radiation-induced meningiomas rarely have latency periods short enough from the time of irradiation to the clinical presentation of the tumor to present in the pediatric patient. Three cases of radiation-induced intracranial meningiomas in pediatric patients are presented. The first involved a meningioma of the right frontal region in a 10-year-old boy 6 years after the resection and irradiation of a 4th ventricular medulloblastoma. Review of our pediatric tumor cases produced a second case of a left temporal fossa meningioma presenting in a 15-year-old boy with a history of irradiation for retinoblastoma at age 3 years and a third case of a right frontoparietal meningioma in a 15-year-old girl after irradiation for acute lymphoblastic leukemia. Only three cases of meningiomas presenting in the pediatric age group after radiation therapy to the head were detected in our review of the literature.

  15. Primary cutaneous extravertebral meningioma. Case report.

    PubMed

    Zaaroor, M; Borovich, B; Bassan, L; Doron, Y; Gruszkiewicz, J

    1984-05-01

    A case of cutaneous extravertebral meningioma is presented. It was diagnosed in infancy as a lumbar meningocele. Operation was initially refused but was subsequently demanded for cosmetic reasons. The findings were a very thick corrugated skin and a cutaneous meningioma connected by a fibrous tract to the dura mater. The presence of a fibrous stalk linking the tumor to the dura mater might have been the pathogenetic connection between the meningocele and cutaneous meningioma.

  16. Spinal meningiomas: surgical management and outcome.

    PubMed

    Gottfried, Oren N; Gluf, Wayne; Quinones-Hinojosa, Alfredo; Kan, Peter; Schmidt, Meic H

    2003-06-15

    Advances in imaging and surgical technique have improved the treatment of spinal meningiomas; these include magnetic resonance imaging, intraoperative ultrasonography, neuromonitoring, the operative microscope, and ultrasonic cavitation aspirators. This study is a retrospective review of all patients treated at a single institution and with a pathologically confirmed diagnosis of spinal meningioma. Additionally the authors analyze data obtained in 556 patients reported in six large series in the literature, evaluating surgical techniques, results, and functional outcomes. Overall, surgical treatment of spinal meningiomas is associated with favorable outcomes. Spinal meningiomas can be completely resected, are associated with postoperative functional improvement, and the rate of recurrence is low.

  17. Clinical and Radiological Characteristics of Angiomatous Meningiomas

    PubMed Central

    Hwang, Juyoung; Kong, Doo-Sik; Seol, Ho Jun; Nam, Do-Hyun; Lee, Jung-Il

    2016-01-01

    Background Angiomatous meningioma is a rare histological subtype of meningioma. Therefore, this specific medical condition is rarely reviewed in the literature. In the present work, we report the clinical and radiological features with postoperative outcomes of angiomatous meningioma. Methods This retrospective study included the patients who were pathologically diagnosed with angiomatous meningioma after surgical resection between February 2010 and September 2015 in our institute. We analyzed the clinical data, radiological manifestation, treatment and prognosis of all patients. Results The 15 patients (5 males and 10 females) were diagnosed with angiomatous meningioma during the study period. The median age of patients at the time of surgery was 63 years (range: 40 to 80 years). According to Simpson classification, 7, 5, and 3 patients achieved Simpson grade I, II, and IV resection, respectively. In the follow-up period, recurrence was noted in one patient. Ten out of the 15 patients showed homogeneous enhancement. Two patients demonstrated cystic changes. There was no occurrence of calcification or hemorrhage in our patients. Characteristically, 14 out of 15 patients showed signal voids of vessels. Significant peritumoral edema was observed in the majority of tumors (67%). Conclusion Angiomatous meningiomas are rare benign meningioma. Brain images of angiomatous meningioma usually demonstrate signal void signs and peritumoral edema. In the present study, angiomatous meningiomas showed good prognosis after surgical resection. PMID:27867918

  18. Treatment of cranial base meningiomas with linear accelerator radiosurgery.

    PubMed

    Chang, S D; Adler, J R

    1997-11-01

    Radiosurgery is increasingly being used to treat cranial base tumors. Since 1989, 55 patients with cranial base meningiomas were treated at Stanford University Medical Center with linear accelerator radiosurgery. An analysis of the clinical and radiographic results of this patient population was the focus of this study. The mean patient age was 55.1 years (range, 28-82 yr). The mean tumor volume was 7.33 cm3 (range, 0.45-27.65 cm3). The radiation dose averaged 18.3 Gy (range, 12-25 Gy), delivered with an average of 2.2 isocenters (range, 1-5). Patients were evaluated retrospectively through clinic notes from follow-up examinations, and residual tumor volume was measured during follow-up imaging studies. The length of follow-up averaged 48.4 months (range, 17-81 mo). Tumor stabilization after radiosurgery was noted in 38 patients (69%), shrinkage in 16 patients (29%), and enlargement in only 1 patient (2%). The results of follow-up magnetic resonance imaging demonstrated decreased central contrast uptake in 11 meningiomas (20%), possibly indicating evidence of central tumor necrosis or tumor vessel obliteration. Neurological status was improved in 15 patients in the series (27%) and unchanged in 34 patients (62%). Three patients (5%) died during the follow-up period, all as a result of causes other than tumor progression. Three patients (5%) developed new permanent symptoms (one patient with seizures, one patient with mild right hemiparesis, and one patient with both vagal and hypoglossal nerve palsy). All other complications were transient, including partial trigeminal nerve palsy in seven patients and diplopia in three patients. The 2-year actuarial tumor control rate was 98%. Although our follow-up period is short, this experience corroborates previous reports that radiosurgery can be used to ablate selected small cranial base meningiomas, with good clinical results and modest morbidity.

  19. Endonasal transsphenoidal removal of tuberculum sellae meningiomas: technical note.

    PubMed

    Cook, Shon W; Smith, Zachary; Kelly, Daniel F

    2004-07-01

    Tuberculum sellae meningiomas traditionally have been removed through a transcranial approach. More recently, the sublabial transsphenoidal approach has been used to remove such tumors. Here, we describe use of the direct endonasal transsphenoidal approach for removal of suprasellar meningiomas. Three women, aged 32, 34, and 55 years, each sought treatment for visual loss and headaches. In each patient, magnetic resonance imaging (MRI) showed a suprasellar mass causing optic chiasmal and optic nerve compression (average size, 2 x 2 cm). All three patients underwent tumor removal via an endonasal approach with the operating microscope. Suprasellar exposure was facilitated by removal of the posterior planum sphenoidale. Ultrasound was used to help define tumor location before dural opening. The extent of tumor removal was verified with angled endoscopes in all patients, and with intraoperative MRI in one patient. The surgical dural and bony defects were repaired in all patients with abdominal fat, titanium mesh, and 2 to 3 days of cerebrospinal fluid lumbar drainage. Nasal packing was not used. There were no postoperative cerebrospinal fluid leaks or meningitis. One patient required a reoperation 2 weeks after surgery to reduce the size of her fat graft, which was causing optic nerve compression; within 24 hours, her vision rapidly improved. At 3 months after surgery, all three patients had normal vision, no new endocrinopathy, and no residual tumor on MRI. At 10 months after surgery, one patient had a small asymptomatic tumor regrowth seen on MRI. The endonasal approach with the operating microscope appears to be an effective minimally invasive method for removing relatively small midline tuberculum sellae meningiomas. Intraoperative ultrasound, the micro-Doppler probe, and angled endoscopes are useful adjuncts for safely and completely removing such tumors. Longer follow-up is needed to monitor for tumor recurrence in these patients.

  20. Meningioma mimics: five key imaging features to differentiate them from meningiomas.

    PubMed

    Starr, C J; Cha, S

    2017-09-01

    There are a wide variety of intracranial mass lesions, both benign and malignant, which can closely mimic meningioma on imaging. We present five characteristic imaging features that can alert the radiologist to consider other differential diagnoses. Of the five imaging characteristics that were rarely seen in meningiomas, but common and specific for meningioma mimics, absence of dural tail is the most common (83.7%). Homogeneous T2 hyperintensity or T2 hypointensity are seen in nearly half of meningioma mimics and osseous destruction and leptomeningeal extension are present in 40.5% and 21.6% of meningioma mimics, respectively. The distinction between meningioma and its mimics is important because a large portion of the meningioma mimics requires substantially different clinical and surgical management. Copyright © 2017 The Royal College of Radiologists. Published by Elsevier Ltd. All rights reserved.

  1. Intradural extramedullary hemangiopericytoma of the thoracic spine infiltrating a nerve root: a case report and literature review.

    PubMed

    Moscovici, Samuel; Ramirez-DeNoriega, Fernando; Fellig, Yakov; Rosenthal, Guy; Cohen, José E; Itshayek, Eyal

    2011-11-01

    Review the presentation and diagnosis of an intradural extramedullary hemangiopericytoma of the thoracic spine. To present a rare case of intradural, subpial hemangiopericytoma in the thoracic spine, with a brief overview of the literature. Spinal intradural extramedullary hemangiopericytoma is rare entity that radiographically mimics nerve-sheath tumors. These lesions are typically diagnosed at surgery performed due to suspicion of tumor. A 20-year-old man who presented with back pain, leg weakness, and sphincter incontinence. MR imaging demonstrated an intradural extramedullary lesion at the T9-T10 level that was isointense on T1- and T2-weighted images and homogeneously enhancing after administration of gadolinium, with cystic components seen on T2-weighted images. The preoperative diagnosis was meningioma or schwannoma. At surgery, the lesion was bluish and completely subpial, with apparent nerve root invasion. Pathological examination revealed a neoplasm adjacent to a nerve root with possible focal infiltration. Abundant reticulin fibers and widened, branching vascular channels imparting a staghorn appearance were seen. Up to five mitotic figures were counted in one high-power field. On immunostaining, the neoplastic cells were diffusely immunoreactive for CD99 and immunonegative for EMA, CD34, and S-100 protein. The pathological diagnosis was consistent with anaplastic hemangiopericytoma, WHO grade III. This is the ninth report of spinal intradural hemangiopericytoma. The location of the neoplasm supports the hypothesis that hemangiopericytoma may arise from the spinal pial capillaries.

  2. Trigeminal complications arising after surgery of cranial base meningiomas.

    PubMed

    Westerlund, Ulf; Linderoth, Bengt; Mathiesen, Tiit

    2012-04-01

    Chronic severe facial pain is a feared sequel of cranial base surgery. This study explores the symptomatology, incidence and impact on the individual of postoperative de novo trigeminal nerve affection as well as the recovery potential. Out of 231 patients operated for cranial base meningiomas at the Karolinska University Hospital during 7 years, 25 complained of de novo trigeminal symptoms at clinical follow-up 3 months after surgery. Six were later lost to follow-up leaving 19 participants in the study, which was conducted using a questionnaire and a structured telephone interview. All patients complained of facial numbness, affecting the V1 branch in 10/19 patients (53%), the V2 branch in 18/19 (95%) and the V3 branch in 9/19 (47%). Surprisingly, only three (16%) suffered from trigeminal pain, which could be adequately managed by pharmacotherapy. However, five patients (26%) demonstrated ocular dysaesthetic problems. Twelve (63%) described their handicap to be mild, while seven (37%) had daily or severe symptoms. Five patients (26%) reported no improvement over time, while nine (47%) showed improvement and four (21%) stated good recovery. Only one patient (5%) claimed complete symptom remission. In the present study, 11% of the patients presented with a de novo postoperative affection of the trigeminal nerve after removal of a cranial base meningioma; 37% of these reported daily/severe symptoms. Only 26% showed good recovery, observed in patients without tumour infiltration of the nerve or intraoperative nerve damage. In spite of frequent complaints of numbness, pain was uncommon (16%) and often manageable by pharmacotherapy, while ocular symptoms turned out to be more frequent and more disabling than expected.

  3. Sheath energy transmission in a collisional plasma with collisionless sheath

    SciTech Connect

    Tang, Xian-Zhu Guo, Zehua

    2015-10-15

    Sheath energy transmission governs the plasma energy exhaust onto a material surface. The ion channel is dominated by convection, but the electron channel has a significant thermal conduction component, which is dominated by the Knudsen layer effect in the presence of an absorbing wall. First-principle kinetic simulations reveal a robustly supersonic sheath entry flow. The ion sheath energy transmission and the sheath potential are accurately predicted by a sheath model of truncated bi-Maxwellian electron distribution. The electron energy transmission is further enhanced by a parallel heat flux of the perpendicular degrees of freedom.

  4. Osteoblastic meningioma of the fourth ventricle.

    PubMed

    Johnson, M D; Tulipan, N; Whetsell, W O

    1989-04-01

    Meningiomas of the fourth ventricle are rare neoplasms. Only meningothelial and fibroblastic subtypes, purportedly arising from the tela choroidea, have been described. In this report we describe clinical, neuroradiological and pathological findings in a 52-year-old man with mild hydrocephalus produced by a large, calcified, osteoblastic meningioma of the fourth ventricle.

  5. Acute recurrent haemorrhage of an intracranial meningioma.

    PubMed

    Bellut, David; Nern, Christian; Burkhardt, Jan-Karl; Könü, Dilek; Bertalanffy, Helmut; Krayenbühl, Niklaus

    2011-07-01

    Meningioma-associated haemorrhages are rare. To our knowledge this is the first report of a patient with an acute two-stage haemorrhage of a benign intracranial meningioma (World Health Organization grade I) verified by cranial CT scan and histopathological examination. Early surgery with complete tumour removal led to a good outcome for the patient. Copyright © 2011 Elsevier Ltd. All rights reserved.

  6. Paraventricular meningioma revealed by mental disorder

    PubMed Central

    Hilmani, Said; Houass, Yassine; El Azhari, Abdessamad

    2016-01-01

    Background: Ventricular meningioma constitutes 2% of intracranial meningioma, representing a challenging disease for neurosurgeons. Although cognitive impairment is one of the major symptoms of ventricular tumors, few studies have reported the details of cognitive impairment before and after their surgical removal. The expected effects on cognitive function should also be considered when choosing a surgical approach. Case Descriptions: We report the case of a large lateral ventricle meningioma revealed by cognitive dysfunction and moderate intellectual disability. The patient underwent subtotal resection of the tumor which had partial improvement in cognitive disorders. It is important to precisely assess neuropsychological function in patients with large brain tumors, and judicious preoperative plan, adequate knowledge of anatomy, and use of correct microsurgical techniques are fundamental in achieving complete resection of paraventricular meningioma with low morbidity. Conclusion: Pre and postoperative precise neuropsychological examinations may identify the potential cognitive impairment and beneficial effects of surgery in patients with large lateral ventricle meningiomas. PMID:28144473

  7. Linear accelerator radiosurgery for meningiomas in and around the cavernous sinus.

    PubMed

    Spiegelmann, Roberto; Nissim, Ouzi; Menhel, Janna; Alezra, Dror; Pfeffer, M Raphael

    2002-12-01

    A retrospective study to evaluate the efficacy and side effects of linear accelerator radiosurgery in the treatment of cavernous sinus meningiomas. Between 1993 and 2001, 42 patients with meningiomas involving the cavernous sinus underwent linear accelerator radiosurgery at our institution. A mean radiation dose of 14 Gy was delivered to the tumor margin. The median tumor volume was 8.2 cm3 (mean, 8.4 cm3). Median follow-up was 36 months (mean, 38 mo). Control of tumor growth was achieved in 97.5% of the patients. There was no mortality or permanent extraocular motor or pituitary dysfunction. Treatment-related complications included new trigeminal neuropathy in 4.7% and a new visual field defect in 2.8%. Two patients required shunt placement after developing hydrocephalus. One patient with symptomatic temporal lobe edema underwent partial excision of the tumor. Improvement of existing cranial neuropathies was noted in 29% of affected trigeminal nerves, in 22% of oculomotor nerves, and in 13% of Cranial Nerves IV and VI. This study indicates that linear accelerator radiosurgery can achieve a high control rate of meningiomas involving the cavernous sinus with no mortality and a low incidence of morbidity.

  8. Vein, silastic, and polyglycolic acid fine mesh: a comparative study in peripheral nerve repair.

    PubMed

    Lolley, R D; Bose, W J; Bastian, F; Bassam, B; Meyer, F N; Anderson, L D

    1995-09-01

    We investigated three sheathing materials (autogenous vein, silastic, and polyglycolic acid fine mesh) using the rat model. Forty rats were divided into five groups of eight animals each. Group A animals underwent transection of the sciatic nerve but had no repair. In Group B, a standard epineural repair was performed. In Groups C, D, and E, the nerve was repaired as in Group B with the addition of autogenous vein, Silastic, and polyglycolic acid fine mesh sheaths, respectively. Nerve regeneration and function were assessed using sciatic functional index, nerve conduction studies, and light microscopy. Sheathing methods showed no statistically significant advantage to standard epineural repair without a sheath.

  9. Quantitative and qualitative 5-aminolevulinic acid–induced protoporphyrin IX fluorescence in skull base meningiomas

    PubMed Central

    Bekelis, Kimon; Valdés, Pablo A.; Erkmen, Kadir; Leblond, Frederic; Kim, Anthony; Wilson, Brian C.; Harris, Brent T.; Paulsen, Keith D.; Roberts, David W.

    2011-01-01

    Object Complete resection of skull base meningiomas provides patients with the best chance for a cure; however, surgery is frequently difficult given the proximity of lesions to vital structures, such as cranial nerves, major vessels, and venous sinuses. Accurate discrimination between tumor and normal tissue is crucial for optimal tumor resection. Qualitative assessment of protoporphyrin IX (PpIX) fluorescence following the exogenous administration of 5-aminolevulinic acid (ALA) has demonstrated utility in malignant glioma resection but limited use in meningiomas. Here the authors demonstrate the use of ALA-induced PpIX fluorescence guidance in resecting a skull base meningioma and elaborate on the advantages and disadvantages provided by both quantitative and qualitative fluorescence methodologies in skull base meningioma resection. Methods A 52-year-old patient with a sphenoid wing WHO Grade I meningioma underwent tumor resection as part of an institutional review board–approved prospective study of fluorescence-guided resection. A surgical microscope modified for fluorescence imaging was used for the qualitative assessment of visible fluorescence, and an intraoperative probe for in situ fluorescence detection was utilized for quantitative measurements of PpIX. The authors assessed the detection capabilities of both the qualitative and quantitative fluorescence approaches. Results The patient harboring a sphenoid wing meningioma with intraorbital extension underwent radical resection of the tumor with both visibly and nonvisibly fluorescent regions. The patient underwent a complete resection without any complications. Some areas of the tumor demonstrated visible fluorescence. The quantitative probe detected neoplastic tissue better than the qualitative modified surgical microscope. The intraoperative probe was particularly useful in areas that did not reveal visible fluorescence, and tissue from these areas was confirmed as tumor following histopathological

  10. SPECT and PET Imaging of Meningiomas

    PubMed Central

    Valotassiou, Varvara; Leondi, Anastasia; Angelidis, George; Psimadas, Dimitrios; Georgoulias, Panagiotis

    2012-01-01

    Meningiomas arise from the meningothelial cells of the arachnoid membranes. They are the most common primary intracranial neoplasms and represent about 20% of all intracranial tumors. They are usually diagnosed after the third decade of life and they are more frequent in women than in men. According to the World Health Organization (WHO) criteria, meningiomas can be classified into grade I meningiomas, which are benign, grade II (atypical) and grade III (anaplastic) meningiomas, which have a much more aggressive clinical behaviour. Computed Tomography (CT) and Magnetic Resonance Imaging (MRI) are routinely used in the diagnostic workup of patients with meningiomas. Molecular Nuclear Medicine Imaging with Single Photon Emission Computed Tomography (SPECT) and Positron Emission Tomography (PET) could provide complementary information to CT and MRI. Various SPECT and PET tracers may provide information about cellular processes and biological characteristics of meningiomas. Therefore, SPECT and PET imaging could be used for the preoperative noninvasive diagnosis and differential diagnosis of meningiomas, prediction of tumor grade and tumor recurrence, response to treatment, target volume delineation for radiation therapy planning, and distinction between residual or recurrent tumour from scar tissue. PMID:22623896

  11. SPECT and PET imaging of meningiomas.

    PubMed

    Valotassiou, Varvara; Leondi, Anastasia; Angelidis, George; Psimadas, Dimitrios; Georgoulias, Panagiotis

    2012-01-01

    Meningiomas arise from the meningothelial cells of the arachnoid membranes. They are the most common primary intracranial neoplasms and represent about 20% of all intracranial tumors. They are usually diagnosed after the third decade of life and they are more frequent in women than in men. According to the World Health Organization (WHO) criteria, meningiomas can be classified into grade I meningiomas, which are benign, grade II (atypical) and grade III (anaplastic) meningiomas, which have a much more aggressive clinical behaviour. Computed Tomography (CT) and Magnetic Resonance Imaging (MRI) are routinely used in the diagnostic workup of patients with meningiomas. Molecular Nuclear Medicine Imaging with Single Photon Emission Computed Tomography (SPECT) and Positron Emission Tomography (PET) could provide complementary information to CT and MRI. Various SPECT and PET tracers may provide information about cellular processes and biological characteristics of meningiomas. Therefore, SPECT and PET imaging could be used for the preoperative noninvasive diagnosis and differential diagnosis of meningiomas, prediction of tumor grade and tumor recurrence, response to treatment, target volume delineation for radiation therapy planning, and distinction between residual or recurrent tumour from scar tissue.

  12. Meningioma Genomics: Diagnostic, Prognostic, and Therapeutic Applications

    PubMed Central

    Bi, Wenya Linda; Zhang, Michael; Wu, Winona W.; Mei, Yu; Dunn, Ian F.

    2016-01-01

    There has been a recent revolution in our understanding of the genetic factors that drive meningioma, punctuating an equilibrium that has existed since Cushing’s germinal studies nearly a century ago. A growing appreciation that meningiomas share similar biologic features with other malignancies has allowed extrapolation of management strategies and lessons from intra-axial central nervous system neoplasms and systemic cancers to meningiomas. These features include a natural proclivity for invasion, frequent intratumoral heterogeneity, and correlation between biologic profile and clinical behavior. Next-generation sequencing has characterized recurrent somatic mutations in NF2, TRAF7, KLF4, AKT1, SMO, and PIK3CA, which are collectively present in ~80% of sporadic meningiomas. Genomic features of meningioma further associate with tumor location, histologic subtype, and possibly clinical behavior. Such genomic decryption, along with advances in targeted pharmacotherapy, provides a maturing integrated view of meningiomas. We review recent advances in meningioma genomics and probe their potential applications in diagnostic, therapeutic, and prognostic frontiers. PMID:27458586

  13. Preoperative Particle and Glue Embolization of Meningiomas: Indications, Results and Lessons Learned from 117 Consecutive Patients.

    PubMed

    Borg, Anouk; Ekanayake, Jinendra; Mair, Richard; Smedley, Thomas; Brew, Stefan; Kitchen, Neil; Samandouras, George; Robertson, Fergus

    2013-02-25

    BACKGROUND:: Preoperative embolization of meningiomas remains contentious, with persisting uncertainty over the safety and efficacy of this adjunctive technique. OBJECTIVE:: To evaluate the safety of presurgicalembolization of meningiomas and its impact on subsequent transfusion requirement, with respect to the extent of embolization and technique used. METHODS:: 117 consecutive patients between 2001 and 2010 were referred for embolization of presumed intracranial meningioma prior to surgical resection. Glue and/or particles were used to devascularize the tumor in 107 patients, all of whom went on to operative resection. The extent and nature of embolization-related complications, degree of angiographic devascularization, and the intraoperative blood transfusion requirements were analyzed. RESULTS:: Mean blood transfusion requirement during surgery was 0.8 units per case (range 1-14 units). Blood transfusion was significantly lower in patients whose meningiomas were completely, angiographicallydevascularized (P= .035). Four patients had complications as a direct result of the embolization procedure. These included intratumoral haemorrhage in two, sixth cranial nerve palsy in one, and scalp necrosis requiring reconstructive surgery in a further patient. CONCLUSION:: The complication rate was 3.7%. No relationship between the embolic agent and the degree of devascularization was observed. Achieving a complete devascularization resulted in a lower blood transfusion requirement, considered an indirect measure of operative blood loss. This series demonstrates that pre-operative meningiomaembolization is safe and may reduce operative blood loss. We present distal intratumoral injection of liquid embolic as a safe and effective alternative to more established particle embolization techniques.

  14. Clear cell meningioma of the cauda equina in an adult: case report and literature review.

    PubMed

    Epstein, Nancy E; Drexler, Steven; Schneider, Jeffrey

    2005-12-01

    In the pediatric population, clear cell meningiomas are more frequently intracranial than intraspinal in location. Tumors recur in up to 40% of cases within 15 postoperative months and are often managed with repeated resection with or without radiation therapy. The management strategy for adults with clear cell meningiomas involving the lumbar spinal canal (cauda equina) is less clearly defined. A 41-year-old woman presented with mild, right greater than left, lower extremity paresis. An enhanced magnetic resonance (MR) scan revealed a homogeneously enhancing intradural lesion filling the spinal canal at the L3-L4 level. Preoperative noncontrast MR studies of the brain and cervical and thoracic spine were negative. An L2-L5 laminectomy was performed for gross total excision of the intradural lesion, which was adherent to one nerve root of the cauda equina. Frozen-section diagnosis confirmed clear cell tumor. Differential diagnoses included meningioma versus renal cell carcinoma. Negative postoperative chest, abdominal, and pelvic computed tomography studies ruled out tumor of renal cell origin. Enhanced MR studies of the neuraxis proved negative. Consultations with multiple oncologists and radiation therapists recommended neither radiation nor chemotherapy following this initial surgery. She remains disease-free 1 year postoperatively. The high recurrence rate for clear cell meningiomas in children requires repeated tumor resection with or without secondary radiation therapy. Following gross total resection of lumbar tumors in adults, reserving radiation therapy for secondary recurrences provides optimal management.

  15. Postpartum Regression of a Presumed Cavernous Meningioma

    PubMed Central

    Phang, See Yung; Whitfield, Peter

    2016-01-01

    Meningiomas are known to be more common in females than males. They are also known in rare cases to grow in size during pregnancy, which can complicate its management. We describe a 31-year-old Caucasian woman who presented with blurring of her vision and diplopia during the third trimester of her pregnancy. Magnetic resonance imaging (MRI) showed a small left cavernous sinus meningioma. The patient was treated conservatively until her uncomplicated delivery. A postpartum MRI scan showed complete regression of the suspected meningioma. Currently the patient is contemplating a further pregnancy. PMID:27066285

  16. Frontal cutaneous meningioma--Case report.

    PubMed

    Ramos, Leonor; Coutinho, Ines; Cardoso, José Carlos; Garcia, Helena; Cordeiro, Margarida Robalo

    2015-01-01

    Cutaneous meningiomas are rare tumors most commonly located on the scalp. We report the case of a 55-year-old male who presented with a 2 x 3 cm tumoral lesion on the forehead. The lesion was hard, adherent and covered by normal skin. Incisional biopsy revealed a proliferation of monomorphic round cells, organized in nests and focally forming pseudovascular spaces. Immunohistochemical study revealed positivity for epithelial antigen membrane and vimentin. Vascular markers, cytokeratins and S100 protein were negative. A brain CT scan did not show any evidence of intracranial meningioma. The authors describe the case of a cutaneous frontal meningioma in probable relation with previous cranioencephalic trauma.

  17. Meckel's cave meningiomas with subarachnoid hemorrhage.

    PubMed

    Rosenberg, G A; Herz, D A; Leeds, N; Strully, K

    1975-06-01

    Two patients with Meckel's Cave meningiomas were initially hospitalized as a result of subarachnoid hemorrhage. Four-vessel angiography was necessary to exclude other causes of bleeding while demonstrating these lesions. Apoplectic presentation in both cases led to early diagnosis and successful surgical therapy. A review of the literature reveals subarachnoid hemorrhage to be a rarity in association with meningiomas. The two patients currently reported are believed to be the only examples on record of hemorrhagic meningiomas arising from the region of Meckel's Cave.

  18. Meningioma with metastasis from follicular carcinoma thyroid.

    PubMed

    Chaturvedi, Sujata; Gupta, Sanjeev; Kumari, Rima

    2010-01-01

    A 45-year-old female presented with loss of vision in the left eye, numbness on left half of face and left-sided hemicrania for two months. On the basis of radiological investigations, provisional diagnosis of basal meningioma was made. Tissue sent for histopathological evaluation revealed a dual tumor-meningioma with metastasis from follicular carcinoma, thyroid. To the best of authors' knowledge, this is the first report of a tumor metastasizing to another tumor, where a follicular carcinoma thyroid metastasized to meningioma.

  19. A neurogenic tumor containing a low-grade malignant peripheral nerve sheath tumor (MPNST) component with loss of p16 expression and homozygous deletion of CDKN2A/p16: a case report showing progression from a neurofibroma to a high-grade MPNST.

    PubMed

    Tajima, Shogo; Koda, Kenji

    2015-01-01

    Development of malignant peripheral nerve sheath tumors (MPNSTs) is a stepwise process that involves the alteration of many cell cycle regulators and the double inactivation of the NF1 gene. Inactivation of the TP53 gene and deletion of the CDKN2A/p16 gene are known to play an important role in the process. Herein, we present a 19-year-old man with a familial history of neurofibromatosis type 1, in whom the tumor arose from the intercostal nerve and showed 3 components: a neurofibroma, a low-grade MPNST, and a high-grade MPNST. Loss of p16 expression and homozygous deletion of the CDKN2A/p16 gene were observed in both the low-grade and the high-grade MPNST. In contrast to low-grade MPNSTs, high-grade MPNSTs generally tend to lose expression of p16 and harbor homozygous deletion of the CDKN2A/p16 gene. Loss of p16 expression and homozygous deletion of the CDKN2A/p16 gene in low-grade MPNST in our case might be related to its progression to high-grade MPNST. To the best of our knowledge, this is the first study correlating the p16 expression status and CDKN2A/p16 gene alteration in low-grade MPNSTs.

  20. Peripheral nerve surgery.

    PubMed

    McQuarrie, I G

    1985-05-01

    In treating the three main surgical problems of peripheral nerves--nerve sheath tumors, entrapment neuropathies, and acute nerve injuries--the overriding consideration is the preservation and restoration of neurologic function. Because of this, certain other principles may need to be compromised. These include achieving a gross total excision of benign tumors, employing conservative therapy as long as a disease process is not clearly progressing, and delaying repair of a nerve transection until the skin wound has healed. Only three pathophysiologic processes need be considered: neurapraxia (focal segmental dymyelination), axonotmesis (wallerian degeneration caused by a lesion that does not disrupt fascicles of nerve fibers), and neurotmesis (wallerian degeneration caused by a lesion that interrupts fascicles). With nerve sheath tumors and entrapment neuropathies, the goal is minimize the extent to which neurapraxia progresses to axonotmesis. The compressive force is relieved without carrying out internal neurolysis, a procedure that is poorly tolerated, presumably because a degree of nerve ischemia exists with any long-standing compression. When the nerve has sustained blunt trauma (through acute compression, percussion, or traction), the result can be a total loss of function and an extensive neuroma-in-continuity (scarring within the nerve). However, the neural pathophysiology may amount to nothing more than axonotmesis. Although this lesion, in time, leads to full and spontaneous recovery, it must be differentiated from the neuroma-in-continuity that contains disrupted fascicles requiring surgery. Finally, with open nerve transection, the priority is to match the fascicles of the proximal stump with those of the distal stump, a goal that is best achieved if primary neurorrhaphy is carried out.

  1. Pathological Location of Cranial Nerves in Petroclival Lesions: How to Avoid Their Injury during Anterior Petrosal Approach

    PubMed Central

    Borghei-Razavi, Hamid; Tomio, Ryosuke; Fereshtehnejad, Seyed-Mohammad; Shibao, Shunsuke; Schick, Uta; Toda, Masahiro; Yoshida, Kazunari; Kawase, Takeshi

    2015-01-01

    Objectives Numerous surgical approaches have been developed to access the petroclival region. The Kawase approach, through the middle fossa, is a well-described option for addressing cranial base lesions of the petroclival region. Our aim was to gather data about the variation of cranial nerve locations in diverse petroclival pathologies and clarify the most common pathologic variations confirmed during the anterior petrosal approach. Method A retrospective analysis was made of both videos and operative and histologic records of 40 petroclival tumors from January 2009 to September 2013 in which the Kawase approach was used. The anatomical variations of cranial nerves IV–VI related to the tumor were divided into several location categories: superior lateral (SL), inferior lateral (IL), superior medial (SM), inferior medial (IM), and encased (E). These data were then analyzed taking into consideration pathologic subgroups of meningioma, epidermoid, and schwannoma. Results In 41% of meningiomas, the trigeminal nerve is encased by the tumor. In 38% of the meningiomas, the trigeminal nerve is in the SL part of the tumor, and it is in 20% of the IL portion of the tumor. In 38% of the meningiomas, the trochlear nerve is encased by the tumor. The abducens nerve is not always visible (35%). The pathologic nerve pattern differs from that of meningiomas for epidermoid and trigeminal schwannomas. Conclusion The pattern of cranial nerves IV–VI is linked to the type of petroclival tumor. In a meningioma, tumor origin (cavernous, upper clival, tentorial, and petrous apex) is the most important predictor of the location of cranial nerves IV–VI. Classification of four subtypes of petroclival meningiomas using magnetic resonance imaging is very useful to predict the location of deviated cranial nerves IV–VI intraoperatively. PMID:28035290

  2. Pathological Location of Cranial Nerves in Petroclival Lesions: How to Avoid Their Injury during Anterior Petrosal Approach.

    PubMed

    Borghei-Razavi, Hamid; Tomio, Ryosuke; Fereshtehnejad, Seyed-Mohammad; Shibao, Shunsuke; Schick, Uta; Toda, Masahiro; Yoshida, Kazunari; Kawase, Takeshi

    2016-02-01

    Objectives Numerous surgical approaches have been developed to access the petroclival region. The Kawase approach, through the middle fossa, is a well-described option for addressing cranial base lesions of the petroclival region. Our aim was to gather data about the variation of cranial nerve locations in diverse petroclival pathologies and clarify the most common pathologic variations confirmed during the anterior petrosal approach. Method A retrospective analysis was made of both videos and operative and histologic records of 40 petroclival tumors from January 2009 to September 2013 in which the Kawase approach was used. The anatomical variations of cranial nerves IV-VI related to the tumor were divided into several location categories: superior lateral (SL), inferior lateral (IL), superior medial (SM), inferior medial (IM), and encased (E). These data were then analyzed taking into consideration pathologic subgroups of meningioma, epidermoid, and schwannoma. Results In 41% of meningiomas, the trigeminal nerve is encased by the tumor. In 38% of the meningiomas, the trigeminal nerve is in the SL part of the tumor, and it is in 20% of the IL portion of the tumor. In 38% of the meningiomas, the trochlear nerve is encased by the tumor. The abducens nerve is not always visible (35%). The pathologic nerve pattern differs from that of meningiomas for epidermoid and trigeminal schwannomas. Conclusion The pattern of cranial nerves IV-VI is linked to the type of petroclival tumor. In a meningioma, tumor origin (cavernous, upper clival, tentorial, and petrous apex) is the most important predictor of the location of cranial nerves IV-VI. Classification of four subtypes of petroclival meningiomas using magnetic resonance imaging is very useful to predict the location of deviated cranial nerves IV-VI intraoperatively.

  3. Spontaneous Regression of an Incidental Spinal Meningioma.

    PubMed

    Yilmaz, Ali; Kizilay, Zahir; Sair, Ahmet; Avcil, Mucahit; Ozkul, Ayca

    2016-03-15

    The regression of meningioma has been reported in literature before. In spite of the fact that the regression may be involved by hemorrhage, calcification or some drugs withdrawal, it is rarely observed spontaneously. We report a 17 year old man with a cervical meningioma which was incidentally detected. In his cervical MRI an extradural, cranio-caudal contrast enchanced lesion at C2-C3 levels of the cervical spinal cord was detected. Despite the slight compression towards the spinal cord, he had no symptoms and refused any kind of surgical approach. The meningioma was followed by control MRI and it spontaneously regressed within six months. There were no signs of hemorrhage or calcification. Although it is a rare condition, the clinicians should consider that meningiomas especially incidentally diagnosed may be regressed spontaneously.

  4. Meningioma with hemorrhagic onset: two case reports.

    PubMed

    Vij, Mukul; Jaiswal, Sushila; Jaiswal, Awadhesh Kumar; Kumar, Sheo; Behari, Sanjay

    2012-01-01

    Haemorrhage is a rare complication of meningiomas that can occur spontaneously, after embolization, stereotactic radiation and perioperatively. Our first case was a 16 year old male, admitted with spastic quadriparesis, and retention of urine. Magnetic Resonance Imaging (MRI) revealed anteriorly placed cervical intradural extramedullary mass. Patient underwent emergency surgery following sudden worsening of neurological symptoms and intratumoral bleed was noted peroperatively. Tumor was labeled as angiomatous meningioma with hemorrhage. The second case was of a 45 year female who presented with history of sudden onset weakness in right upper and lower limb followed by unconsciousness. MRI revealed heterogeneous lesion in left parasagittal area with intratumoral bleed. Left frontal craniotomy with tumour decompression was performed. Tumour was labelled as meningothelial meningioma with haemorrhage. Meningiomas with hemorrhagic onset remain rare, and pathophysiology is still incompletely understood. Prevention and outcome of intratumoral haemorrhage highly depends on early diagnosis and adequate treatment.

  5. Retrosigmoid approach for resection of petrous apex meningioma.

    PubMed

    de Souza, Daniel G; Ditzel Filho, Leo F S; Makonnen, Girma; Zoli, Matteo; Naudy, Cristian; Muto, Jun; Prevedello, Daniel M

    2014-01-01

    We present the case of a 50-year-old female with a 1-year history of right-side facial numbness, as well as an electric shock-like sensation on the right-side of the face and tongue. She was previously diagnosed with vertigo and trigeminal neuralgia. MRI was obtained showing a large right cerebellopontine angle mass. A retrosigmoid approach was performed and total removal was achieved after dissection of tumor from brainstem and cranial nerves IV, V, VI, VII and VIII. Pathology confirmed the diagnosis of a meningioma (WHO Grade I). The patient was discharged neurologically intact on the third postoperative day free of complications. The video can be found here: http://youtu.be/-tR0FtMiUDg .

  6. 21 CFR 884.5320 - Glans sheath.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ... sheath. (a) Identification. A glans sheath device is a sheath which covers only the glans penis or part... the entire shaft of the penis. It is indicated only for the prevention of pregnancy and not for the...

  7. 21 CFR 884.5320 - Glans sheath.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ... sheath. (a) Identification. A glans sheath device is a sheath which covers only the glans penis or part... the entire shaft of the penis. It is indicated only for the prevention of pregnancy and not for the...

  8. Atypical Growth Pattern of an Intraparenchymal Meningioma

    PubMed Central

    Chen, Xiaoxi

    2016-01-01

    Meningiomas are the most common primary nonneuroglial extra-axial neoplasms, which commonly present as spherical or oval masses with a dural attachment. Meningiomas without dural attachment are rare and, according to their locations, are classified into 5 varieties, including intraventricular, deep Sylvain fissure, pineal region, intraparenchymal, or subcortical meningiomas. To the best of our knowledge, intraparenchymal meningioma with cerebriform pattern has never been reported. In this paper, we report a 34-year-old Chinese male patient who presented with paroxysmal headaches and progressive loss of vision for 10 months and blindness for 2 weeks. A thorough physical examination revealed loss of bilateral direct and indirect light reflex. No other relevant medical history and neurologic deficits were noted. Computed tomography and magnetic resonance imaging scans showed an irregular mass with a unique cerebriform pattern and extensive peritumoral edema in the parietal-occipital-temporal region of the right cerebral hemisphere. The initial diagnosis was lymphoma. Intraoperatively, the tumor was completely buried in a sulcus in the parietal-occipital-temporal region without connecting to the dura. The histological diagnosis was intracranial meningioma based on pathological examination. Therefore, when an unusual cerebriform growth pattern of a tumor is encountered, an intraparenchymal meningioma should be considered as a differential diagnosis. PMID:27752384

  9. Comparative Analysis of the Magnetic Resonance Imaging Features Between Anaplastic Meningioma and Atypical Meningioma.

    PubMed

    Liu, Hong; Zhou, Junlin; Li, Wenyi; Liu, Guangyao

    2016-05-01

    The aim of the study was to investigate the differences in the imaging feature between anaplastic meningioma (World Health Organization grade III) and atypical meningioma (World Health Organization grade II), summarize its specificity of image features, and provide the basis for accurate preoperative diagnosis. The magnetic resonance imaging features of 20 patients of anaplastic meningioma were compared with those of 30 patients of atypical meningioma retrospectively, all of which were confirmed by surgery and pathology. The imaging features of the 2 groups of tumors were statistically analyzed using χ tests. The 2 tumor types differed in several features, including lobulated or irregular shape (P < 0.05), cystic and necrotic changes (P < 0.05), peritumoral edema (P < 0.05), and brain-tumor interface (P < 0.01). There were no significant differences in hemorrhage, homogeneous enhancement of the tumor, dural tail sign, or adjacent bone change (P > 0.05). Differences in the imaging feature between anaplastic meningioma and atypical meningioma can improve the differential diagnosis and allow a more appropriate approach to therapy. The article focuses on examining the differences of the magnetic resonance imaging features between anaplastic meningiomas and atypical meningiomas have been examined in only a few studies.

  10. Analysis of Gene Expression Profiling in Meningioma: Deregulated Signaling Pathways Associated with Meningioma and EGFL6 Overexpression in Benign Meningioma Tissue and Serum

    PubMed Central

    Wang, Xuanchun; Gong, Ye; Wang, Daijun; Xie, Qing; Zheng, Mingzhe; Zhou, Yu; Li, Qin; Yang, Zhen; Tang, Hailiang; Li, Yiming; Hu, Renming; Chen, Xiancheng; Mao, Ying

    2012-01-01

    Molecular mechanisms underlying the pathogenesis of meningioma are not fully elucidated. In this study, we established differential gene expression profiles between meningiomas and brain arachnoidal tissue by using Affymetrix GeneChip Human U133 Plus 2.0 Array. KEGG pathway analysis demonstrated that PI3K/Akt and TGFβ signaling pathways were up-regulated in fibroblastic meningioma, and focal adhesion and ECM-receptor interaction pathways were activated in anaplastic meningioma. EGFL6 was one of the most up-regulated genes in fibroblastic meningioma by microarray analysis. Quantitative real-time PCR demonstrated that benign meningiomas had significantly higher levels of EGFL6 mRNA than brain arachnoidal tissue and atypical and anaplastic meningiomas (P<0.001). EGFL6 gene was also highly expressed in ovarian cancer, but expressed lowly in other investigated tumors. ELISA analysis showed that patients with benign meningiomas and ovarian cancers had the highest serum levels of EGFL6 (mean concentration: 672 pg/ml for benign meningiomas, and 616 pg/ml for ovarian cancers). Healthy people and patients with other tumors, however, had low levels of serum EGFL6. In conclusion, we proposed that activation of PI3K/Akt and integrin-mediated signaling pathways was involved in the pathogenesis of benign and anaplastic meningiomas, respectively. We also presented evidence that EGFL6 was overexpressed in benign meningioma tissues and serum. PMID:23285163

  11. Analysis of gene expression profiling in meningioma: deregulated signaling pathways associated with meningioma and EGFL6 overexpression in benign meningioma tissue and serum.

    PubMed

    Wang, Xuanchun; Gong, Ye; Wang, Daijun; Xie, Qing; Zheng, Mingzhe; Zhou, Yu; Li, Qin; Yang, Zhen; Tang, Hailiang; Li, Yiming; Hu, Renming; Chen, Xiancheng; Mao, Ying

    2012-01-01

    Molecular mechanisms underlying the pathogenesis of meningioma are not fully elucidated. In this study, we established differential gene expression profiles between meningiomas and brain arachnoidal tissue by using Affymetrix GeneChip Human U133 Plus 2.0 Array. KEGG pathway analysis demonstrated that PI3K/Akt and TGFβ signaling pathways were up-regulated in fibroblastic meningioma, and focal adhesion and ECM-receptor interaction pathways were activated in anaplastic meningioma. EGFL6 was one of the most up-regulated genes in fibroblastic meningioma by microarray analysis. Quantitative real-time PCR demonstrated that benign meningiomas had significantly higher levels of EGFL6 mRNA than brain arachnoidal tissue and atypical and anaplastic meningiomas (P<0.001). EGFL6 gene was also highly expressed in ovarian cancer, but expressed lowly in other investigated tumors. ELISA analysis showed that patients with benign meningiomas and ovarian cancers had the highest serum levels of EGFL6 (mean concentration: 672 pg/ml for benign meningiomas, and 616 pg/ml for ovarian cancers). Healthy people and patients with other tumors, however, had low levels of serum EGFL6. In conclusion, we proposed that activation of PI3K/Akt and integrin-mediated signaling pathways was involved in the pathogenesis of benign and anaplastic meningiomas, respectively. We also presented evidence that EGFL6 was overexpressed in benign meningioma tissues and serum.

  12. Sheaths: A Comparison of Magnetospheric, ICME, and Heliospheric Sheaths

    NASA Technical Reports Server (NTRS)

    Sibeck, D. G.; Richardson, J. D.; Liu, W.

    2007-01-01

    When a supersonic flow encounters an obstacles, shocks form to divert the flow around the obstacle. The region between the shock and the obstacle is the sheath, where the supersonic flow is compressed, heated, decelerated, and deflected. Supersonic flows, obstacles, and thus sheaths are observed on many scales throughout the Universe. We compare three examples seen in the heliosphere, illustrating the interaction of the solar wind with obstacles of three very different scales lengths. Magnetosheaths form behind planetary bow shocks on scales ranging from tens to 100 planetary radii. ICME sheath form behind shocks driven by solar disturbances on scale lengths of a few to tens of AU. The heliosheath forms behind the termination shock due to the obstacle presented by the interstellar medium on scale lengths of tens to a hundred AU. Despite this range in scales some common features have been observed. Magnetic holes, possibly due to mirror mode waves, have been observed in all three of these sheaths. Plasma depletion layers are observed in planetary and ICME sheaths. Other features observed in some sheaths are wave activity (ion cyclotron, plasma), energetic particles, transmission of Alfven waves/shocks, tangential discontinuities turbulence behind quasi-parallel shocks, standing slow mode waves, and reconnection on the obstacle boundary. We compare these sheath regions, discussing similarities and differences and how these may relate to the scale lengths of these regions.

  13. [Psychopathology and neuro-ophthalmology in meningioma of the olfactory groove].

    PubMed

    Lange, E; Bräunig, K P; Fötzsch, R

    1982-01-01

    In 14 patients with a surgically nad histopathologically verified meningioma of the olfactory nerve groove (OGM) (12 women, 2 men), clinical findings and diagnosis were analysed, especially with respect to neuropathology and psychopathology. The apodictic theorem that an OGM leads to a Foster-Kennedy syndrome as well as to a psychopathological orbital brain syndrome cannot be maintained. The Foster-Kennedy syndrome does not occur in a characteristically high incidence, and psychopathologically an orbital brain syndrome can only be diagnosed in case of an anterior location of the OGM. The more the OGM shows starting point or growth development to or from the anterior chiasmal angle, the more is resembles that psychopathological syndrome that we have found and described for the meningioma of the Tuberculus sellae.

  14. Spheno-Orbital Meningioma Resection and Reconstruction: The Role of Piezosurgery and Premolded Titanium Mesh

    PubMed Central

    Jung, Susana Heredero; Ferrer, Alicia Dean; Vela, Juan Solivera; Granados, Francisco Alamillos

    2011-01-01

    We present the clinical case of a patient with a spheno-orbital meningioma. Literature review of the treatment options, including the application of piezoelectric or ultrasound surgery and orbital reconstruction after meningioma resection, is also presented. Complete resection was performed by means of a frontotemporal craniotomy and an orbitozygomatic approach. Piezoelectric osteotomy was used around the optic nerve canal and the superior orbital fissure to minimize the damage to soft tissues. Orbital wall reconstruction was done using a titanium mesh previously premolded using a skull model. The superior orbital rim was reconstructed with calvarial bone grafts, and the sphenotemporal bone defect was covered with a titanium mesh cranioplasty. Ultrasonic vibrations to perform osteotomies in craniofacial surgery provide an interesting tool to reduce damage to surrounding soft tissues. Reconstruction of the roof and lateral orbital wall with premolded titanium meshes with a skull model is a safe and easy method to achieve a good orbital reconstruction and to avoid secondary sequelae. PMID:23205170

  15. Surgical management of tuberculum sellae meningioma: role of selective extradural anterior clinoidectomy.

    PubMed

    Otani, Naoki; Muroi, Carl; Yano, Hirohito; Khan, Nadia; Pangalu, Athina; Yonekawa, Yasuhiro

    2006-06-01

    A retrospective analysis of 32 patients with tuberculum sellae meningiomas who underwent surgery via a unilateral pterional approach was performed. A selective extradural anterior clinoidectomy (SEAC) technique was added in 20 patients. All patients had visual dysfunction preoperatively. Macroscopically complete removal with Simpson grade II was performed in 28 patients (87.5%). The postoperative visual function improved in 25 (78.1%), did not change in 3 (9.4%), and worsened in 4 patients (12.5%). The SEAC technique was effective, especially for removal of the tumour extending into the sellae/pituitary stalk (9 patients), the optic canal (4 patients) and hypothalamus (4 patients) with preservation of the visual and endocrinological function. These results were superior to those of surgery without SEAC technique. This technique is therefore recommended for complete resection of the tuberculum sellae meningiomas extending to the surrounding anatomical structures as the SEAC procedure reduces the risk of intraoperative optic nerve injury considerably.

  16. Meningiomas with Rhabdoid or Papillary Components : Prognosis and Comparison with Anaplastic Meningiomas

    PubMed Central

    Kim, Jeong-Kwon; Jung, Shin; Lee, Kyung-Hwa; Kim, Seul-Kee; Lee, Eun Jung

    2016-01-01

    Papillary and rhabdoid meningiomas are pathologically World Health Organization (WHO) grade III. Any correlation between clinical prognosis and pathologic component is not clear. We analyzed the prognoses of patients with meningiomas with a rhabdoid or papillary component compared to those of patients with anaplastic meningiomas. From 1994 to June 2013, 14 anaplastic meningiomas, 6 meningiomas with a rhabdoid component, and 5 meningiomas with papillary component were pathologically diagnosed. We analyzed magnetic resonance imaging (MRI) findings, extent of removal, adjuvant treatment, progression-free survival (PFS), overall survival (OS), and pathologic features of 14 anaplastic meningiomas (group A), 5 meningiomas with a predominant (≥50%) papillary or rhabdoid component (group B1), and 6 meningiomas without a predominant (<50%) rhabdoid or papillary component (group B2). Homogeneous enhancement on MRI was associated with improved PFS compared to heterogeneous enhancement (p=0.025). Depending on pathology, the mean PFS was 134.9±31.6 months for group A, 46.6±13.4 months for group B1, and 118.7±19.2 months for group B2. The mean OS was 138.5±24.6 months for group A and 59.7±16.8 months for group B1. All recurrent tumors were of the previously diagnosed pathology, except for one tumor from group B1, which recurred as an atypical meningioma without a papillary component. Group B1 tumors showed a more aggressive behavior than group B2 tumors. In group B2 cases, the pathologic findings of non-rhabdoid/papillary portion could be considered for further adjuvant treatment. PMID:27446516

  17. Nerve Degeneration and Regeneration Associated with NF1 Tumors

    DTIC Science & Technology

    2014-09-01

    the potential for nerve regeneration after PNST eradication. Additional aims will test photodynamic therapy as modality for eradication of PNST without...induced  nerve  regeneration  after   tumor  eradication.     Photodynamic   therapy  (PDT)  is  a  promising  modality...nerve graft implanted to bridge the gap and restore nerve continuity. 15. SUBJECT TERMS peripheral nerve sheath tumor, neurofibroma, photodynamic

  18. Sclerosing Meningioma : Radiological and Clinical Characteristics of 21 Cases

    PubMed Central

    Kang, Ho; Se, Young-Bem; Dho, Yun-Sik; Choi, Seung Hong; Park, Sung-Hye

    2016-01-01

    Objective A rare subtype of meningioma, sclerosing meningioma is not included in the current World Health Organization classification of meningiomas and is classified into the category of other morphological variation subtypes. Sclerosing meningioma is often misdiagnosed to other non-benign meningioma or malignant neoplasm, so it is important to diagnose sclerosing type correctly. We analyzed the radiological and clinical characteristics of a series of sclerosing meningiomas. Methods Twenty-one patients who underwent surgery in one institute with a histopathologically proven sclerosing meningioma were included from 2006 to 2014. Eighteen tumors were diagnosed as a pure sclerosing-type meningioma, and 3 as mixed type. Magnetic resonance image was taken for all patients including contrast enhancement image. Computed tomography (CT) scan was taken for 16 patients. One neuroradiologist and 1 neurosurgeon reviewed all images retrospectively. Results In the all 16 patients with preoperative CT images, higher attenuation was observed in the meningioma than in the brain parenchyma, and calcification was observed in 11 (69%). In 15 of the 21 patients (71%), a distinctive very low signal intensity appeared as a dark color in T2-weighted images. Nine of these 15 tumors (60%) exhibited heterogeneous enhancement, and 6 (40%) exhibited homogeneous enhancement that was unlike the homogeneous enhancing pattern shown by conventional meningiomas. Ten patients had a clear tumor margin without peritumoral edema. Conclusion Although these peculiar radiological characteristics are not unique to sclerosing meningioma, we believe that they are distinctive features that may be helpful for distinguishing sclerosing meningioma from other subtypes. PMID:27847571

  19. Case Report: Trigeminal Neuralgia Caused by a Minute Meningioma with Hyperostosed Suprameatal Tubercle.

    PubMed

    Ishi, Yukitomo; Asaoka, Katsuyuki; Sugiyama, Taku; Yokoyama, Yuka; Yamazaki, Kazuyoshi; Echizenya, Sumire; Itamoto, Koji; Echizenya, Kohei

    2015-01-01

    Cerebellopontine angle tumors might occasionally provoke trigeminal neuralgia but are usually large enough to be diagnosed radiographically. We present a case of trigeminal neuralgia caused by a very small meningioma covering the suprameatal tubercle that displayed hyperostosis at the entrance of Meckel's cave and was not obvious on routine magnetic resonance (MR) images. A 72-year-old woman with intractable trigeminal neuralgia in the left V3 territory was referred to our institution. Preoperative imaging studies revealed that the left trigeminal nerve was medially distorted at the entrance of Meckel's cave by a laterally seated bone bulge covered by a minute enhanced lesion. Trigeminal nerve decompression surgery was performed via a retrosigmoid intradural suprameatal approach. We found a small meningioma that had compressed and flattened the trigeminal nerve root at the entrance of Meckel's cave, which was grossly and totally removed by suprameatal tubercle resection. There was no vascular compression of the trigeminal nerve root. The trigeminal neuralgia ceased completely after the operation. Accurate preoperative determination of the causative pathologies is essential to achieve adequate surgical results after microvascular decompression for neurovascular compression syndrome. Because conventional MR sequences are inadequate for the precise interpretation of complex neurovascular anatomy in the cerebellopontine angle and such small tumors can be overlooked on routine MR studies, high-resolution thin-slice MR examinations and careful radiological interpretations are required for correct diagnosis and treatment.

  20. Case Report: Trigeminal Neuralgia Caused by a Minute Meningioma with Hyperostosed Suprameatal Tubercle

    PubMed Central

    Ishi, Yukitomo; Asaoka, Katsuyuki; Sugiyama, Taku; Yokoyama, Yuka; Yamazaki, Kazuyoshi; Echizenya, Sumire; Itamoto, Koji; Echizenya, Kohei

    2015-01-01

    Cerebellopontine angle tumors might occasionally provoke trigeminal neuralgia but are usually large enough to be diagnosed radiographically. We present a case of trigeminal neuralgia caused by a very small meningioma covering the suprameatal tubercle that displayed hyperostosis at the entrance of Meckel's cave and was not obvious on routine magnetic resonance (MR) images. A 72-year-old woman with intractable trigeminal neuralgia in the left V3 territory was referred to our institution. Preoperative imaging studies revealed that the left trigeminal nerve was medially distorted at the entrance of Meckel's cave by a laterally seated bone bulge covered by a minute enhanced lesion. Trigeminal nerve decompression surgery was performed via a retrosigmoid intradural suprameatal approach. We found a small meningioma that had compressed and flattened the trigeminal nerve root at the entrance of Meckel's cave, which was grossly and totally removed by suprameatal tubercle resection. There was no vascular compression of the trigeminal nerve root. The trigeminal neuralgia ceased completely after the operation. Accurate preoperative determination of the causative pathologies is essential to achieve adequate surgical results after microvascular decompression for neurovascular compression syndrome. Because conventional MR sequences are inadequate for the precise interpretation of complex neurovascular anatomy in the cerebellopontine angle and such small tumors can be overlooked on routine MR studies, high-resolution thin-slice MR examinations and careful radiological interpretations are required for correct diagnosis and treatment. PMID:26351448

  1. [Skull base meningiomas: a predictive system to know the extent of their surgical resection and patient outcome].

    PubMed

    Morales, F; Maillo, A; Díaz-Alvarez, A; Merino, M; Muñoz-Herrera, A; Hernández, J; Santamarta, D

    2005-12-01

    The aim of this study was to build a preoperative predictive system which could provide reliable information about: 1 degrees which skull base meningiomas can be total or partially removed, and 2 degrees their surgical outcome. Patient histories and imaging data were reviewed retrospectively from 85 consecutive skull base meningiomas patients who underwent surgery from 1990 and 2002. From the preoperative data, nine variables were selected for conventional statistical analysis as regards their relationship with: 1 degrees total vs partial tumor resection and 2 degrees with patients outcome according to the degree of tumour removal. From the nine variables analysed only two had a statistical association with the type of tumour resection performed (total vs partial) and the patient outcome: 1) arteries encasement and 2) cranial nerves involvement. Upon correlating these two variables with the type of tumour resection performed (total vs partial) and with the Karnofsky'scale to evaluate patients surgical outcome, the following grading groups were identified: Grade I: skull base meningiomas which did not involve cranial nerves or artery or only encased one artery or one cranial nerve. In these cases the incidence of gross tumour resection was 98.3% (p< 0.0001) and the perspective to reach 70 points in the Karnofsky'scale was of 96.5% ( p=0.001). Grade II: skull base meningiomas which involved one cranial nerve and encased, at least, two main cerebral arteries. In these cases, the frequency of total resection, decreased to 83.3% (p<0.0001) and the probability to reach 70 points in the Karnofsky'scale was 70.6% (p=0.001). Grade III: skull base meningiomas which involved two or more cranial nerves and encased several arteries In this group, the frequency of a total resection was of 42.9% (p<0.0001) and the probability of reaching 70 points in the Karnofsky'scale was only 60% (p=0.001). We propose a preoperative grading system for skull base meningiomas that helps

  2. Ossified spinal meningiomas: Clinical and surgical features.

    PubMed

    Alafaci, Concetta; Grasso, Giovanni; Granata, Francesca; Salpietro, Francesco M; Tomasello, Francesco

    2016-03-01

    Meningiomas constitute 25% of primary spinal tumors and predominantly involve the thoracic spinal cord. Although calcifications are commonly seen in intracranial meningiomas, gross calcifications are observed in only 1-5% of all spinal meningiomas. We report the clinical findings, surgical strategy and histological features of 9 patients with ossified spinal meningiomas (OSMs). Clinical and surgical features of 9 patients with ossified spinal meningiomas were retrospectively reviewed. There were 8 women and 1 man with a mean age of 59 years. In 7 patients, the lesions were localized in the thoracic segment of the spine while in 2 patients in the lower cervical segment. All patients presented with weakness of the lower limbs and hypoesthesia below the site level of the lesion. Only 2 patients presented with urinary incontinence. Gross-total resection of the tumor was achieved in 6 patients while in 3 a subtotal removal of the meningioma was obtained. In all patients the postoperative course was uneventful. Six patients presented with a significant neurological improvement while in 3 patients a mild improvement was observed. Microscopically, all tumors showed typical histological pattern of ossified meningioma. OSMs are amenable to surgery if the complete removal can be achieved. Because of their hard-rock consistency complete resection can be challenging. In difficult cases, subtotal removal can be advised and follow-up imaging is mandatory. Overall, the risk of long-term recurrence of the lesions is low, and a good clinical outcome after total or subtotal removal can be expected. Copyright © 2016 Elsevier B.V. All rights reserved.

  3. Endoscopic endonasal approach for a tuberculum sellae meningioma.

    PubMed

    Fernandez-Miranda, Juan C; Pinheiro-Neto, Carlos D; Gardner, Paul A; Snyderman, Carl H

    2012-01-01

    The authors present the technical and anatomical nuances needed to perform an endoscopic endonasal removal of a tuberculum sellae meningioma. The patient is a 47-year-old female with headaches and an incidental finding of a small tuberculum sellae meningioma with no vascular encasement, no optic canal invasion, but mild inferior to superior compression of the cisternal segment of the left optic nerve. Neuroophthalmology assessment revealed no visual defects. Treatment options included clinical observation with imaging follow-up studies, radiosurgery, and resection. The patient elected to undergo surgical removal and an endonasal endoscopic approach was the preferred surgical option. Preoperative radiological studies showed the presence of an osseous ring between the left middle and anterior clinoids, the so-called carotico-clinoidal ring. The surgical implications of this finding and its management are illustrated. The surgical anatomy of the suprasellar region is reviewed, including concepts such as the chiasmatic sulcus and limbus sphenoidale, medial and lateral optico-carotid recesses, and the paraclinoidal and supraclinoidal segments of the internal carotid artery. Emphasis is made in the importance of exposing the distal dural ring of the internal carotid artery and the precanalicular segment of the optic nerve for adequate intradural dissection. The endonasal route allows for early coagulation of the tumor meningeal supply and extensive resection of dural attachments, and importantly, provides an inferior to superior access to the infrachiasmatic region that facilitates complete tumor removal without any manipulation of the optic nerve. The lateral limit of dural removal is formed by the distal dural ring, which is gently coagulated after the tumor is resected. A 45° scope is used to inspect for any residual tumor, in particular at the entrance of the optic nerve into the optic canal and at the most anterior margin of the exposure (limbus sphenoidale). The

  4. Endoscopic endonasal approach for a tuberculum sellae meningioma.

    PubMed

    Fernandez-Miranda, Juan C; Pinheiro-Nieto, Carlos; Gardner, Paul A; Snyderman, Carl H

    2012-01-01

    The authors present the technical and anatomical nuances needed to perform an endoscopic endonasal removal of a tuberculum sellae meningioma. The patient is a 47-year-old female with headaches and an incidental finding of a small tuberculum sellae meningioma with no vascular encasement, no optic canal invasion, but mild inferior to superior compression of the cisternal segment of the left optic nerve. Neuroophthalmology assessment revealed no visual defects. Treatment options included clinical observation with imaging follow-up studies, radiosurgery, and resection. The patient elected to undergo surgical removal and an endonasal endoscopic approach was the preferred surgical option. Preoperative radiological studies showed the presence of an osseous ring between the left middle and anterior clinoids, the so-called carotico-clinoidal ring. The surgical implications of this finding and its management are illustrated. The surgical anatomy of the suprasellar region is reviewed, including concepts such as the chiasmatic sulcus and limbus sphenoidale, medial and lateral optico-carotid recesses, and the paraclinoidal and supraclinoidal segments of the internal carotid artery. Emphasis is made in the importance of exposing the distal dural ring of the internal carotid artery and the precanalicular segment of the optic nerve for adequate intradural dissection. The endonasal route allows for early coagulation of the tumor meningeal supply and extensive resection of dural attachments, and importantly, provides an inferior to superior access to the infrachiasmatic region that facilitates complete tumor removal without any manipulation of the optic nerve. The lateral limit of dural removal is formed by the distal dural ring, which is gently coagulated after the tumor is resected. A 45° scope is used to inspect for any residual tumor, in particular at the entrance of the optic nerve into the optic canal and at the most anterior margin of the exposure (limbus sphenoidale). The

  5. Electromagnetic induction between axons and their schwann cell myelin-protein sheaths.

    PubMed

    Goodman, G; Bercovich, D

    2013-12-01

    Two concepts have long dominated vertebrate nerve electrophysiology: (a) Schwann cell-formed myelin sheaths separated by minute non-myelinated nodal gaps and spiraling around axons of peripheral motor nerves reduce current leakage during propagation of trains of axon action potentials; (b) "jumping" by action potentials between successive nodes greatly increases signal conduction velocity. Long-held and more recent assumptions and issues underlying those concepts have been obscured by research emphasis on axon-sheath biochemical symbiosis and nerve regeneration. We hypothesize: mutual electromagnetic induction in the axon-glial sheath association, is fundamental in signal conduction in peripheral and central myelinated axons, explains the g-ratio and is relevant to animal navigation.

  6. Laminated magnet field coil sheath

    DOEpatents

    Skaritka, J.R.

    1987-05-15

    A method for manufacturing a magnetic cable trim coil in a sheath assembly for use in a cryogenic particle accelerator. A precisely positioned pattern of trim coil turns is bonded to a flexible substrate sheath that is capable of withstanding cryogenic operating conditions. In the method of the invention the flexible substrate sheath, with the trim coil pattern precisely location relative to a bore tube assembly of an accelerator and is then bonded to the bore tube with a tape suitable for cryogenic application. The resultant assembly can be readily handled and installed within an iron magnet yoke assembly of a suitable cryogenic particle accelerator. 1 fig.

  7. Laminated magnet field coil sheath

    DOEpatents

    Skaritka, John R.

    1987-12-01

    a method for manufacturing a magnet cable trim coil in a sheath assembly for use in a cryogenic particle accelerator. A precisely positioned pattern of trim coil turns is bonded to a flexible substrate sheath that is capable of withstanding cryogenic operating conditions. In the method of the invention the flexible sheath, with the trim coil pattern precisely positioned thereon, is accurately positioned at a precise location relative to a bore tube assembly of an accelerator and is then bonded to the bore tube with a tape suitable for cryogenic application. The resultant assembly can be readily handled and installed within an iron magnet yoke assembly of a suitable cryogenic particle accelerator.

  8. Laminated magnet field coil sheath

    DOEpatents

    Skaritka, John R.

    1987-12-01

    a method for manufacturing a magnet cable trim coil in a sheath assembly for use in a cryogenic particle accelerator. A precisely positioned pattern of trim coil turns is bonded to a flexible substrate sheath that is capable of withstanding cryogenic operating conditions. In the method of the invention the flexible sheath, with the trim coil pattern precisely positioned thereon, is accurately positioned at a precise location relative to a bore tube assembly of an accelerator and is then bonded to the bore tube with a tape suitable for cryogenic application. The resultant assembly can be readily handled and installed within an iron magnet yoke assembly of a suitable cryogenic particle accelerator.

  9. Snare sheath versus evolution sheath in transvenous lead extraction.

    PubMed

    Kong, Jihua; Tian, Yilun; Guo, Fei; Ze, Feng; Duan, Jiangbo; Wang, Long; Li, Xuebin; Guo, Jihong

    2015-01-01

    Lead extraction remains a challenging procedure with significant success. Owing to the increasing use of cardiac implantable electronic devices, there is a growing need for effective techniques management, to avoid the occurrence of device-related complications and lead dysfunction. We made a comparison of Evolution sheath and Needles Eye Snare sheath (abbreviated to Snare sheath) by analyzed the results using two kind of the methods in the Cardiology Center of Peking University People's Hospital. In the retrospective study we evaluated patients who underwent lead extraction from July 2013 to July 2014. Those who underwent lead removal without using evolution or snare were excluded. Primary endpoints included total exposure time, operation time, and complications. Data on clinical characteristics, indications, and outcomes were prospectively collected and analyzed. A total of 76 patients were included in the study (65.8% male; aged 68.1±14.34 years old). Snare and Evolution were used in 59 and 17 patients, respectively. A total of 134 leads were removed with 103 leads (76.87%) extracted using the Snare sheath. Lead age was 10.8±7.0 years. Complete extraction was observed in 67 patients of 124 leads. Evolution sheath was associated with significantly lower complication even after adjustment of the number of leads, type of leads, and lead age, compared to the Snare group (P<0.05). In the Snare sheath group, mortality rate was 1.69% and minor complication rate was 3.39%. When compared to the Snare sheath, lead extraction with the Evolution sheath has less X-ray exposure time and less operation time (P<0.05), while with a higher success rate (P<0.05). Besides, the Evolution group has fewer major and minor complications.

  10. Coexistence of cervico-thoracic extradural en-plaque meningioma with multiple intracranial meningiomas.

    PubMed

    Kale, Aydemir; Akyol, Cetin; Keskin, Emrah; Aydoğmuş, Evren; Aydın, Hasan Ali; Barut, Figen; Gül, Sanser; Kalaycı, Murat

    2014-01-01

    Meningioma is one of the most common tumors in the spinal cord. Extradural and en-plaque variety of meningioma occur less frequently. A 47-year-old woman is presented with radiculopathy signs. Magnetic resonance imaging revealed a lesion from C6 through T3 vertebral levels compressing the cord both anteriorly and posteriorly. Subtotally excision was performed and histopathologic signs showed transitional type of meningioma (WHO Grade 1). Post operatively, she had good neurological recovery. Intraoperative findings point out that the en-plaque meningioma was pure extradural. Twelve cases of pure extradural en-plaque meningioma have been reported in the literature. Besides, to the best our knowledge coexistence of "en plaque" spinal epidural meningioma with meningiomas in cranial cavity has not been reported. Complete resection is mandatory to prevent recurrence. Moreover, it is considerably difficult to remove the parts of tumor over anterior of the dura without complication. Copyright © 2014 Polish Neurological Society. Published by Elsevier Urban & Partner Sp. z o.o. All rights reserved.

  11. The WHO Grade I Collagen-forming Meningioma Produces Angiogenic Substances. A New Meningioma Entity.

    PubMed

    Haybaeck, Johannes; Smolle, Elisabeth; Schökler, Bernadette; Kleinert, Reinhold

    2016-03-01

    Meningiomas arise from arachnoid cap cells, the so-called meningiothelial cells. They account for 20-36% of all primary intracranial tumours, and arise with an annual incidence of 1.8-13 per 100,000 individuals/year. According to their histopathological features meningiomas are classified either as grade I (meningiothelial, fibrous/fibroblastic, transitional/mixed, psammomatous, angiomatous, microcystic, secretory and the lympholasmacyterich sub-type), grade II (atypical and clear-cell sub-type) or grade III (malignant or anaplastic phenotype). A 62-year-old female patient presented to the hospital because of progressive obliviousness and concentration difficulties. In the magnetic resonance imaging (MRI) of the brain, an occipital convexity-meningioma was found in the left hemisphere, which was subsequently resected. Within the tumour tissue there were multiple spheroid precipitates, i.e. secretion products that turned out to consist of collagen. Part of the tumour cells displayed positive reactions for vasogenic substances, namely for vascular endothelial growth factor (VEGF) and epidermal growth factor receptor (EGFR). Correspondingly, the diagnosis "WHO Grade I collagen-forming meningioma" seems to be most appropriate. The "WHO Grade I collagen-forming meningioma" reported herein produces collagen and angiogenic substances. To the best of our knowledge, no such entity has been reported on in previous literature. We propose this collagen-producing meningioma as a novel WHO grade I meningioma sub-type. Copyright© 2016 International Institute of Anticancer Research (Dr. John G. Delinassios), All rights reserved.

  12. Transformation of a meningioma with atypical imaging

    PubMed Central

    Kumar, Ashish; Deopujari, Chandrashekhar; Karmarkar, Vikram

    2016-01-01

    Meningiomas are benign tumors of the central nervous system. They have long term curability if they are excised completely. If not, they can recur after a prolonged period and can lead to increased morbidity during re-surgery. Recurrence is rarely associated with invasiveness. Usually de-differentiation in case of meningiomas is uncommon without any predisposing factors including different genetic mutations or radiation to the involved region. We report a case of a 38-year-old female who was operated for a benign para-sagittal meningioma 8 years back and subsequently developed an invasive recurrence off late. Also this time, the imaging morphology was slightly different for a meningioma and gross as well as microscopic findings were very atypical. Awareness for such cases must be there while dealing with recurrent meningiomas as invasiveness may not always be associated with adverse predisposing factors like radiation. As invasiveness is always a histopathological diagnosis, picking up such features on imaging is a daunting task and if done, can help neurosurgeons prognosticate such invasive recurrences in a better fashion. PMID:27366271

  13. Dorsal spinal epidural psammomatous meningioma in an adult male

    PubMed Central

    Pandey, Sharad; Singh, Kulwant; Sharma, Vivek; Ghosh, Amrita; Suman, Saurabh

    2016-01-01

    Meningiomas are benign in nature and arise from the arachnoid cells. They are mostly situated in the intracranial compartment, whereas spinal meningiomas are rare. Approximately, in 10% of cases, an extradural component is seen but an exclusively extradural meningioma is quite uncommon. However, WHO Grade II (atypical) and Grade III (anaplastic) tumors can behave aggressively. We reported a case of purely extradural psammomatous meningioma in an adult male affecting the dorsal spine although uncommon meningiomas should be included in the differential diagnosis of extradural intraspinal masses. PMID:26933358

  14. Dorsal spinal epidural psammomatous meningioma in an adult male.

    PubMed

    Pandey, Sharad; Singh, Kulwant; Sharma, Vivek; Ghosh, Amrita; Suman, Saurabh

    2016-01-01

    Meningiomas are benign in nature and arise from the arachnoid cells. They are mostly situated in the intracranial compartment, whereas spinal meningiomas are rare. Approximately, in 10% of cases, an extradural component is seen but an exclusively extradural meningioma is quite uncommon. However, WHO Grade II (atypical) and Grade III (anaplastic) tumors can behave aggressively. We reported a case of purely extradural psammomatous meningioma in an adult male affecting the dorsal spine although uncommon meningiomas should be included in the differential diagnosis of extradural intraspinal masses.

  15. Intraventricular meningioma after cranial irradiation for childhood leukemia.

    PubMed

    Ney, Douglas E; Huse, Jason T; Dunkel, Ira J; Steinherz, Peter G; Haque, Sofia; Khakoo, Yasmin

    2010-10-01

    Meningiomas are among the most common brain tumors in adults. They are most commonly located over the cerebral convexities and are infrequently found in an intraventricular location. Ionizing cranial radiation is a risk factor for late occurrence of meningiomas within the radiation field. While pathologic grading of meningiomas is straightforward, significant variability often exists between pathologists in applying standard grading criteria. This has implications for prognosis. Radiation-induced meningiomas may also have predilection to recur. The authors describe a case of an intraventricular meningioma occurring 23 years after cranial irradiation for childhood acute lymphoblastic leukemia.

  16. Gamma Knife radiosurgery of large skull base meningiomas.

    PubMed

    Starke, Robert M; Przybylowski, Colin J; Sugoto, Mukherjee; Fezeu, Francis; Awad, Ahmed J; Ding, Dale; Nguyen, James H; Sheehan, Jason P

    2015-02-01

    Stereotactic radiosurgery (SRS) has become a common treatment modality for intracranial meningiomas. Skull base meningiomas greater than 8 cm3 in volume have been found to have worse outcomes following SRS. When symptomatic, patients with these tumors are often initially treated with resection. For tumors located in close proximity to eloquent structures or in patients unwilling or unable to undergo a resection, SRS may be an acceptable therapeutic approach. In this study, the authors review the SRS outcomes of skull base meningiomas greater than 8 cm3 in volume, which corresponds to a lesion with an approximate diameter of 2.5 cm. The authors reviewed the data in a prospectively compiled database documenting the outcomes of 469 patients with skull base meningiomas treated with single-session Gamma Knife radiosurgery (GKRS). Seventy-five patients had tumors greater than 8 cm3 in volume, which was defined as a large tumor. All patients had a minimum follow-up of 6 months, but patients were included if they had a complication at any time point. Thirty patients were treated with upfront GKRS, and 45 were treated following microsurgery. Patient and tumor characteristics were assessed to determine predictors of new or worsening neurological function and tumor progression following GKRS. After a mean follow-up of 6.5 years (range 0.5-21 years), the tumor volume was unchanged in 37 patients (49%), decreased in 26 patients (35%), and increased in 12 patients (16%). Actuarial rates of progression-free survival at 3, 5, and 10 years were 90.3%, 88.6%, and 77.2%, respectively. Four patients had new or worsened edema following GKRS, but preexisting edema decreased in 3 patients. In Cox multivariable analysis, covariates associated with tumor progression were 1) presentation with any cranial nerve (CN) deficit from III to VI (hazard ratio [HR] 3.78, 95% CI 1.91-7.45; p<0.001), history of radiotherapy (HR 12.06, 95% CI 2.04-71.27; p=0.006), and tumor volume greater than 14 cm3

  17. FURTHER OBSERVATIONS ON THE STRUCTURE OF MYELIN SHEATHS IN THE CENTRAL NERVOUS SYSTEM

    PubMed Central

    Peters, A.

    1964-01-01

    Direct evidence has been presented to confirm the existence of a spiral in the myelin sheaths of the central nervous system. An account of some of the variations in structure of central myelin sheaths has been given and it has been shown that the radial component of myelin sheaths has the form of a series of rod-like thickenings of the intraperiod line. These thickenings extend along the intraperiod line in a direction parallel to the length of the axon. The relative position of the internal mesaxon and external tongue of cytoplasm has been determined in a number of transverse sections of sheaths from the optic nerves of adult mice, adult rats, and young rats. In about 75 per cent of the mature sheaths examined, these two structures were found within the same quadrant of the sheath, so that the cytoplasm of the external tongue process tends to lie directly outside that associated with the internal mesaxon. The frequency with which the internal mesaxon and external tongue lie within the same quadrant of the sheath increases both with the age of the animal and with the number of lamellae present within a sheath. The possible significance of these findings is discussed. PMID:14126873

  18. Intraspinal meningioma with malignant transformation and distant metastasis.

    PubMed

    Ito, Kenyu; Imagama, Shiro; Ando, Kei; Kobayashi, Kazuyoshi; Shido, Yoji; Go, Yoshida; Arima, Hideyuki; Kanbara, Shunsuke; Hirose, Takanori; Matsuyama, Yukihiro; Nishida, Yoshihiro; Ishiguro, Naoki

    2017-02-01

    Meningioma is typically considered to be a benign tumor. Malignant transformation and metastasis of meningiomas are rare. Moreover, most meningiomas are intracranial, and there are few reports on intraspinal meningiomas. This report aimed to describe the clinical features and pathological findings of a case of malignant transformation and distant metastasis of intraspinal meningioma, with a review of the literature. A 44-year-old man with a bilateral lower limb paresis was diagnosed with an intradural extramedullary tumor of the thoracic spine. Primary tumor resection was performed, and the histological findings revealed atypical meningioma. The meningioma recurred 2 years after the primary surgery, and a second resection was performed, but only partial resection was possible because of decreased motor evoked potential. At age 48, the patient's lower limb weakness returned, and a third resection was performed, and the histological finding remained atypical meningioma. At age 54, the tumor increased and stereotactic irradiation was performed. At age 60, the patient was diagnosed with metastatic tumors of the rib, lumbar vertebra, cervical spine, and sacrum. Biopsy of the rib metastatic tumor was performed, and the histological findings revealed anaplastic meningioma. This case is the first report of an intraspinal meningioma that transformed from atypical to anaplastic meningioma with distant hematogenous metastasis.

  19. Intraspinal meningioma with malignant transformation and distant metastasis

    PubMed Central

    Ito, Kenyu; Imagama, Shiro; Ando, Kei; Kobayashi, Kazuyoshi; Shido, Yoji; Go, Yoshida; Arima, Hideyuki; Kanbara, Shunsuke; Hirose, Takanori; Matsuyama, Yukihiro; Nishida, Yoshihiro; Ishiguro, Naoki

    2017-01-01

    ABSTRACT Meningioma is typically considered to be a benign tumor. Malignant transformation and metastasis of meningiomas are rare. Moreover, most meningiomas are intracranial, and there are few reports on intraspinal meningiomas. This report aimed to describe the clinical features and pathological findings of a case of malignant transformation and distant metastasis of intraspinal meningioma, with a review of the literature. A 44-year-old man with a bilateral lower limb paresis was diagnosed with an intradural extramedullary tumor of the thoracic spine. Primary tumor resection was performed, and the histological findings revealed atypical meningioma. The meningioma recurred 2 years after the primary surgery, and a second resection was performed, but only partial resection was possible because of decreased motor evoked potential. At age 48, the patient’s lower limb weakness returned, and a third resection was performed, and the histological finding remained atypical meningioma. At age 54, the tumor increased and stereotactic irradiation was performed. At age 60, the patient was diagnosed with metastatic tumors of the rib, lumbar vertebra, cervical spine, and sacrum. Biopsy of the rib metastatic tumor was performed, and the histological findings revealed anaplastic meningioma. This case is the first report of an intraspinal meningioma that transformed from atypical to anaplastic meningioma with distant hematogenous metastasis. PMID:28303067

  20. Radiosurgical planning of meningiomas: compromises with conformity.

    PubMed

    Rowe, Jeremy G; Walton, Lee; Vaughan, Paul; Malik, Irfan; Radatz, Matthias; Kemeny, Andras

    2004-01-01

    The radiosurgical planning of meningiomas frequently necessitates compromises between irradiating tumour and risking damage to adjacent structures. In selected cases, we resolved this by excluding part of the tumour from the prescription isodose volume. Most of these compromises or 'suboptimal' plans achieved growth control. Growth control could not be related to conformity indices or to various measures of the radiation dose received by the meningioma. Examining recurrences, 75% arose from dura outside the original treatment field. These findings are discussed in terms of dose prescription protocols and the use of conformity indices in planning. The importance of the dural origin of meningiomas is well established in surgical practice, as reflected by Simpson's grades, but may be equally significant in radiosurgical practice.

  1. Pediatric spinal clear cell meningioma. Case report.

    PubMed

    Colen, Chaim B; Rayes, Mahmoud; McClendon, Jamal; Rabah, Raja; Ham, Steven D

    2009-01-01

    In this report the authors describe a unique case of spinal clear cell meningioma in a 13-year-old girl. Clear cell meningiomas (CCMs) are not uncommon. To the authors' knowledge, 14 cases of pediatric CCM occurring in the spinal canal have been reported. Factors lending resistance to meningioma initiation and invasion are analyzed. This 13-year-old girl presented with pain radiating down her left leg. Admission MR imaging showed an inhomogeneous enhancing intradural-extramedullary mass at the L4-5 level. Resection revealed a CCM, and radiotherapy was subsequently administered. Postoperatively there has been no recurrence in > 2 years. In this paper the authors report a case of CCM and provide a comprehensive literature review on this disease. Current recommendations for its management are still debatable, especially in the pediatric population, and the authors propose an algorithm for its treatment and surveillance.

  2. Cervical spinal extradural meningioma: Case report.

    PubMed

    Benzagmout, M; Azzal, F; Bitar, A; El Faïz Chaoui, M; Van Effenterre, R

    2010-10-01

    Extradural meningiomas account for approximately 7% of all spinal meningiomas and are most commonly located in the thoracic spine. Although rare, they should be included in the differential diagnosis of an extradural contrast-enhancing mass. Prognosis depends greatly on the extent of surgical resection. If considered safe, complete resection should be attempted to decrease the risk of recurrence. We report a case of spinal epidural meningioma diagnosed in an elderly woman complaining of right cervicobrachial pain of several years duration, associated with diffuse paresthesia and permanent numbness of her right index finger. The surgical removal of the tumor was incomplete because of the deep lateral extension of the lesion. Copyright © 2010 Elsevier Masson SAS. All rights reserved.

  3. Spinal subdural hematoma following meningioma removal operation.

    PubMed

    Jun, Hyo Sub; Oh, Jae Keun; Park, Young Seok; Song, Joon Ho

    2014-03-01

    Although blood contamination of cerebrospinal fluid (CSF) after an intracranial operation can occur, the development of a symptomatic spinal hematoma after craniotomy has been anecdotally reported and it is uncommon reported after a supratentorial meningioma removal operation. We report a case of spinal subdural hematoma following a supratentorial meningioma removal operation and discuss the mechanism of spinal subdural hematoma (SSDH) development. A 54-year-old woman presented with lumbago and radicular pain on both legs 4 days after a right parietooccipital craniotomy for meningioma removal. Only the straight leg raising sign was positive on neurologic examination but the magnetic resonance imaging (MRI) demonstrated a lumbosacral spinal subdural hematoma. The patient received serial lumbar tapping, after which her symptoms showed improvement.

  4. Spinal Subdural Hematoma Following Meningioma Removal Operation

    PubMed Central

    Jun, Hyo Sub; Oh, Jae Keun; Park, Young Seok

    2014-01-01

    Although blood contamination of cerebrospinal fluid (CSF) after an intracranial operation can occur, the development of a symptomatic spinal hematoma after craniotomy has been anecdotally reported and it is uncommon reported after a supratentorial meningioma removal operation. We report a case of spinal subdural hematoma following a supratentorial meningioma removal operation and discuss the mechanism of spinal subdural hematoma (SSDH) development. A 54-year-old woman presented with lumbago and radicular pain on both legs 4 days after a right parietooccipital craniotomy for meningioma removal. Only the straight leg raising sign was positive on neurologic examination but the magnetic resonance imaging (MRI) demonstrated a lumbosacral spinal subdural hematoma. The patient received serial lumbar tapping, after which her symptoms showed improvement. PMID:24891866

  5. Primary pulmonary meningioma: A case report.

    PubMed

    Huang, Shucheng; Chen, Li; Mao, Yuping; Tong, Hongwei

    2017-05-01

    Primary extracranial meningiomas are rare outside the head and neck region. A 44-year-old female patient had chest pain for more than 1 year. Preoperative chest computed tomography (CT) scan revealed a nodule in the right lower lobe, 1.8 cm in diameter. Tumor tissues were examined by immunohistochemistry for vimentin and S-100. Histopathologically, the tumor was characterized by whorled nests of spindle-shaped cells accompanied by psammoma bodies. Immunohistochemistry demonstrated tumor cell positivity for vimentin and S-100. This case was diagnosed as a primary pulmonary meningioma. The tumor was removed by a thoracoscopic pulmonary wedge resection. Postoperative cranial and spinal CT scan did not show any intracranial or spinal mass. No recurrence of the tumor was reported at the time of writing up this case report. A primary pulmonary meningioma should be considered in the differential diagnosis workup of pulmonary nodules.

  6. Posterior approach to ventrally located spinal meningiomas

    PubMed Central

    Voulgaris, Spyridon; Mihos, Evaggelos; Karagiorgiadis, Dimitrios; Zigouris, Andreas; Fotakopoulos, George; Drosos, Dimitrios; Pahaturidis, Dimitrios

    2010-01-01

    For the resection of anteriorly located meningiomas, various approaches have been used. Posterior approach is less invasive and demanding; however, it has been associated with increased risk of spinal cord injury. We evaluated ten consecutive patients that underwent surgery for spinal meningiomas. All patients were preoperative assessed by neurological examination, computed tomography and magnetic resonance imaging. All tumors were ventrally located and removed via a posterior approach. Transcranial motor-evoked potentials (TcMEPs), somatosensory-evoked potential (SSEP) and free running electromyography (EMG) were monitored intraoperative. Postoperative all patients had regular follow-up examinations. There were four males and six females. The mean age was 68.2 years (range 39–82 years). In nine out of ten cases, the tumor was located in the thoracic spine. A case of a lumbar meningioma was recorded. The most common presenting symptom was motor and sensory deficits and unsteady gait, whereas no patient presented with paraplegia. All meningiomas were operated using a microsurgical technique via a posterior approach. During the operation, free running EMG monitoring prompted a surgical alert in case of irritation, whereas TcMEP and SSEP amplitudes remained unchanged. Histopathology revealed the presence of typical (World Health Organisation grade I) meningiomas. The mean Ki-67/MIB-1 index was 2.75% (range 0.5–7). None of our patients sustained a transient or permanent motor deficit. After a mean follow-up period of 26 months (range 56–16 months), no tumor recurrence and no instability were found. Posterior approach for anteriorly located meningiomas is a safe procedure with the use of intraoperative monitoring, less invasive and well-tolerated especially in older patients. Complete tumor excision can be performed with satisfactory results. PMID:20127494

  7. Papillary meningioma: clinical and histopathological observations

    PubMed Central

    Wang, Dai-Jun; Zheng, Ming-Zhe; Gong, Ye; Xie, Qing; Wang, Yin; Cheng, Hai-Xia; Mao, Ying; Zhong, Ping; Che, Xiao-Ming; Jiang, Cheng-Chuan; Huang, Feng-Ping; Zheng, Kang; Li, Shi-Qi; Gu, Yu-Xiang; Bao, Wei-Min; Yang, Bo-Jie; Wu, Jing-Song; Xie, Li-Qian; Tang, Hai-Liang; Zhu, Hong-Da; Chen, Xian-Cheng; Zhou, Liang-Fu

    2013-01-01

    Papillary meningioma is a rare subtype of malignant meningiomas, which is classified by the World Health Organization as Grade III. Because of lack of large sample size case studies, many of the specific characteristics of papillary meningioma are unclear. This study investigated by retrospective analysis the clinical, radiological and histopathological findings of 17 papillary meningioma patients who underwent surgical resection or biopsy, to assess the characteristics of papillary meningioma. Eight female and nine male patients were included, with a mean age of 40 (range: 6 to 55) years. Tumors were mostly located in the cerebral convexity and showed irregular margins, absence of a peritumoral rim, heterogeneous enhancement and severe peritumoral brain edema on preoperative images. Brain invasion was often confirmed during the operations, with abundant to exceedingly abundant blood supply. Intratumoral necrosis and mitosis was frequently observed on routinely stained sections. The average MIB-1 labeling index was 6.9%. Seven cases experienced tumor recurrence or progression, while seven patients died 6 to 29 months after operation. Radiation therapy was given in 52.9% of all cases. Univariate analysis showed that only the existence of intratumoral necrosis and incomplete resection correlated with tumor recurrence. The 3-year progression free survival was 66.7% after gross total resection and 63.6% for other cases. The 3-year mortality rate was 50% after gross total resection and 63.6% for other cases. Papillary meningioma has specific clinical and histopathological characteristics. Tumor recurrence (or progression) and mortality are common. Gross total tumor resection resulted in less recurrence and mortality. PMID:23638219

  8. Posterior approach to ventrally located spinal meningiomas.

    PubMed

    Voulgaris, Spyridon; Alexiou, George A; Mihos, Evaggelos; Karagiorgiadis, Dimitrios; Zigouris, Andreas; Fotakopoulos, George; Drosos, Dimitrios; Pahaturidis, Dimitrios

    2010-07-01

    For the resection of anteriorly located meningiomas, various approaches have been used. Posterior approach is less invasive and demanding; however, it has been associated with increased risk of spinal cord injury. We evaluated ten consecutive patients that underwent surgery for spinal meningiomas. All patients were preoperative assessed by neurological examination, computed tomography and magnetic resonance imaging. All tumors were ventrally located and removed via a posterior approach. Transcranial motor-evoked potentials (TcMEPs), somatosensory-evoked potential (SSEP) and free running electromyography (EMG) were monitored intraoperative. Postoperative all patients had regular follow-up examinations. There were four males and six females. The mean age was 68.2 years (range 39-82 years). In nine out of ten cases, the tumor was located in the thoracic spine. A case of a lumbar meningioma was recorded. The most common presenting symptom was motor and sensory deficits and unsteady gait, whereas no patient presented with paraplegia. All meningiomas were operated using a microsurgical technique via a posterior approach. During the operation, free running EMG monitoring prompted a surgical alert in case of irritation, whereas TcMEP and SSEP amplitudes remained unchanged. Histopathology revealed the presence of typical (World Health Organisation grade I) meningiomas. The mean Ki-67/MIB-1 index was 2.75% (range 0.5-7). None of our patients sustained a transient or permanent motor deficit. After a mean follow-up period of 26 months (range 56-16 months), no tumor recurrence and no instability were found. Posterior approach for anteriorly located meningiomas is a safe procedure with the use of intraoperative monitoring, less invasive and well-tolerated especially in older patients. Complete tumor excision can be performed with satisfactory results.

  9. Glioblastoma Mimicking Meningioma: Report of 2 Cases.

    PubMed

    Patel, Mohit; Nguyen, Ha Son; Doan, Ninh; Gelsomino, Michael; Shabani, Saman; Mueller, Wade

    2016-11-01

    Glioblastoma can mimic various pathologies, including arteriovenous malformation, hemorrhage from ischemic stroke, cerebral contusion, metastatic disease, lymphoma, and infection. The literature is limited regarding diagnostic confusion with meningioma. Herein, we present 2 patients that exhibited imaging, including cerebral angiography during preoperative embolization, which was consistent with meningioma, but where final surgical diagnosis revealed glioblastoma. Case 1 was a 57-year-old woman presenting with headache, ataxia, and memory lapses for the past month. Brain magnetic resonance imaging (MRI) demonstrated a heterogeneous-enhancing right temporoparietal mass with broad contact along the right tentorium, cerebrospinal fluid (CSF) cleft sign, and dural tail sign-consistent with meningioma. Patient underwent angiography with successful polyvinyl alcohol foam (PVA) particle embolization of the petrosquamosal branch of the right middle meningeal artery (MMA) and meningeal branch of the right occipital artery, resulting in significant devascularization of the tumor blush. Subsequently, the patient underwent tumor resection, where pathology revealed glioblastoma. Case 2 was a 60-year-old man presenting with right hemiparesis. Brain MRI demonstrated a left parasagittal, heterogeneous-enhancing mass abutting the falx with a dural tail sign-consistent with meningioma. Patient underwent angiography with successful PVA particle embolization of the left MMA, resulting in significant devascularization of the tumor blush. Patient underwent a tumor resection where pathology revealed glioblastoma. Glioblastoma can mimic meningioma on MRI with dural tail sign, CSF cleft sign, and broad dural contact. Moreover, cerebral angiography can reveal tumor feeders commonly associated with meningioma. These features can contribute to diagnostic confusion. Based on these 2 cases, preoperative embolization of tumor feeders is possible with glioblastoma. Copyright © 2016 The Author

  10. Contemporary Endovascular Embolotherapy for Meningioma

    PubMed Central

    Dubel, Gregory J.; Ahn, Sun Ho; Soares, Gregory M.

    2013-01-01

    Preoperative endovascular tumor embolization has been used for 40 years. Meningiomas are the most common benign intracranial tumor in which preoperative embolization has been most extensively described in the literature. Advocates of embolization report that it reduces operative blood-loss, and softens the tumor, thus making surgery safer and easier. Opponents suggest that it adds additional risk and cost for patients without controlled studies showing conclusive benefit. The literature suggests a 3 to 6% neurological complication rate related to embolization. The combined external and internal carotid artery blood supply and complex anastomoses of the meninges can make embolization challenging. Positive outcomes require thorough knowledge of the pertinent vascular anatomy, familiarity with the neurovascular equipment and embolics, and meticulous technique. There remains debate on several aspects of embolization, including tumors most appropriate for embolization, embolic agent of choice, ideal size of embolic, and the choice of vessel(s) to embolize. This detailed review of pertinent vascular anatomy, embolization technique, results, and complications should allow practitioners to maximize treatment outcomes in this setting. PMID:24436548

  11. Glioblastoma induction after radiosurgery for meningioma.

    PubMed

    Yu, J S; Yong, W H; Wilson, D; Black, K L

    2000-11-04

    A 70-year-old woman developed a glioblastoma in the irradiated field 7 years after stereotactic radiosurgery for meningioma. Glioma induction has been previously reported after external beam radiation for leukaemia, pituitary adenoma, tinea capitus, and meningioma. This radiosurgery-induced malignancy may portend further reports of tumour induction. The theoretical risk of tumour induction by low doses of radiation to normal neural tissue after radiosurgery is now confirmed. Reports of additional cases of radiosurgery-induced tumours might temper the use of this increasingly used technique for benign surgically accessible lesions.

  12. Meningioma after radiotherapy for Hodgkin's disease.

    PubMed

    Deutsch, M; Rosenstein, M; Figura, J H

    1999-08-01

    The most common second primary tumors after treatment of childhood Hodgkin's disease are leukemia, lymphoma, breast cancer, soft tissue sarcoma, and thyroid cancer. Although intracranial meningioma has been reported after radiotherapy to the scalp for benign conditions and for intracranial primary brain tumors, this appears to be an extremely rare sequelae of treatment for Hodgkin's disease. The authors describe a 15-year-old boy who underwent radiotherapy for Hodgkin's disease and in whom a meningioma developed in the posterior fossa 27 years later.

  13. Predicting Meningioma Consistency on Preoperative Neuroimaging Studies

    PubMed Central

    Shiroishi, Mark S.; Cen, Steven Y.; Tamrazi, Benita; D'Amore, Francesco; Lerner, Alexander; King, Kevin S.; Kim, Paul E.; Law, Meng; Hwang, Darryl H.; Boyko, Orest B.; Liu, C. Jason

    2016-01-01

    Synopsis This article provides an overview of the neuroimaging literature focused on pre-operative prediction of meningioma consistency. A validated, non-invasive neuroimaging method to predict tumor consistency can provide valuable information regarding neurosurgical planning and patient counseling. Most of the neuroimaging literature indicates conventional MRI using T2-weighted imaging (T2WI) may be helpful to predict meningioma consistency, however, further rigorous validation is necessary. Much less is known about advanced MRI techniques such as diffusion MRI, MR elastography (MRE) and MR spectroscopy (MRS). Of these methods, MRE and DTI appear particularly promising. PMID:27012379

  14. Meningiomas of the velum interpositum: surgical considerations.

    PubMed

    Lozier, Alan P; Bruce, Jeffrey N

    2003-07-15

    Meningiomas of the third ventricle are a rare subtype of pineal region tumor that arise from the posterior portion of the velum interpositum, the double layer of pia mater that forms the roof of the third ventricle. The authors review the literature concerning these meningiomas and present a case in which the lesion was resected via the supracerebellar-infratentorial approach. The relationship of the tumor to the deep venous system and the splenium of the corpus callosum guides the selection of the most advantageous surgical approach. Posterior displacement of the internal cerebral veins demonstrated on preoperative imaging provides a strong rationale for use of the supracerebellar-infratentorial approach.

  15. Giant velum interpositum meningioma in a child.

    PubMed

    Moiyadi, Aliasgar V; Shetty, Prakash

    2012-07-01

    Intraventricular meningiomas are rare, but are relatively more often seen in children. Large size at presentation often obscures anatomical details. A particular subset of such tumors arising from the velum interpositum pose a significant surgical challenge. Thorough preoperative imaging, especially with respect to the course of the deep venous structures, provides useful evidence as to the origin. Preservation of venous anatomy at surgery is vital. We describe a 3-year-old girl with a giant velum interpositum meningioma that was completely excised with excellent outcome. This is probably the youngest such case reported.

  16. Successful CyberKnife Irradiation of 1000 cc Hemicranial Meningioma: 6-year Follow-up

    PubMed Central

    Golanov, Andrey V.; Antipina, Natalia; Gorlachev, Gennady

    2015-01-01

    Meningiomas are common benign tumors with accepted treatment approaches and usually don't challenge healthcare specialists. We present a case of a huge unresectable hemicranial meningioma, which was successfully treated with hypofractionated irradiation. A male patient, sixty-two years of age, suffered for over 12 years from headaches, facial deformity, right eye displacement, right eye movement restriction, right-sided ptosis, and facial hypoesthesia. MRI and CT studies revealed an extended hemicranial meningioma. Prior to irradiation, the patient underwent four operations. Eventually, the tumor was irradiated with the CyberKnife in August 2009. Tumor volume composed 1085 cc. The mean dose of 35.3 Gy was delivered in 7 fractions (31.5 Gy at 72% isodose line comprising 95% of tumor volume). The patient was followed during six years and experienced only mild (Grade 1-2 CTCAE) acute skin and mucosa reactions. During the follow-up period, we observed target volume shrinkage for 17% (for 26% after excluding hyperostosis) and regression of intracranial hypertension signs. Due to the extreme volume and complex shape of the tumor, spreading along the surface of the hemisphere as well as an optic nerve involvement, the case presented would not be generally considered suitable for irradiation, especially for hypofractionation. We regard this clinical situation not as a treatment recommendation, but as a demonstration of the underestimated possibilities of hypofractionation regimen and CyberKnife system, both of which are limited with our habit of conventional treatments. PMID:26719827

  17. Successful CyberKnife Irradiation of 1000 cc Hemicranial Meningioma: 6-year Follow-up.

    PubMed

    Galkin, Mikhail; Golanov, Andrey V; Antipina, Natalia; Gorlachev, Gennady

    2015-11-20

    Meningiomas are common benign tumors with accepted treatment approaches and usually don't challenge healthcare specialists. We present a case of a huge unresectable hemicranial meningioma, which was successfully treated with hypofractionated irradiation. A male patient, sixty-two years of age, suffered for over 12 years from headaches, facial deformity, right eye displacement, right eye movement restriction, right-sided ptosis, and facial hypoesthesia. MRI and CT studies revealed an extended hemicranial meningioma. Prior to irradiation, the patient underwent four operations. Eventually, the tumor was irradiated with the CyberKnife in August 2009. Tumor volume composed 1085 cc. The mean dose of 35.3 Gy was delivered in 7 fractions (31.5 Gy at 72% isodose line comprising 95% of tumor volume). The patient was followed during six years and experienced only mild (Grade 1-2 CTCAE) acute skin and mucosa reactions. During the follow-up period, we observed target volume shrinkage for 17% (for 26% after excluding hyperostosis) and regression of intracranial hypertension signs. Due to the extreme volume and complex shape of the tumor, spreading along the surface of the hemisphere as well as an optic nerve involvement, the case presented would not be generally considered suitable for irradiation, especially for hypofractionation. We regard this clinical situation not as a treatment recommendation, but as a demonstration of the underestimated possibilities of hypofractionation regimen and CyberKnife system, both of which are limited with our habit of conventional treatments.

  18. Endoscopic endonasal extended transsphenoidal removal of tuberculum sellae meningioma (TSM): an experience of six cases.

    PubMed

    Chowdhury, Forhad H; Haque, Mohammod R; Goel, Atul H; Kawsar, Khandkar A

    2012-10-01

    Tuberculum sellae meningiomas (TSMs) are usually removed through a transcranial approach. Recently, the sublabial transsphenoidal microscopic approach has been used to remove such tumours. More recently, endonasal extended transsphenoidal approach is getting popular for removal of tuberculum sellae meningioma. Here, we describe our initial experience of endonasal extended transsphenoidal approach for removal of suprasellar meningiomas in six consecutive cases. Six patients (four female and two male) who presented for headache and visual loss were investigated with MRI of brain that showed tuberculum sellae meningioma compressing visual apparatus. Average size was 3 × 3 cm in three cases and 4 × 4 cm in rest of the three. All patients underwent endoscopic endonasal extended transsphenoidal tumour removal, but in two patients with large tumour, microscopic assistance was needed. Complete tumour removal was done in all cases except one case where perforators seemed to be encased by the tumour and resulted in incomplete removal. The surgical dural and bony defects were repaired in all patients with thigh fat graft. Nasal packing was not used, but inflated balloon of Foley's catheter was used to keep fat in position. There was mild postoperative cerebrospinal fluid (CSF) leakage in one patient on the fourth postoperative day after removal of lumbar CSF drain and stopped spontaneously on the seventh postoperative day. There were no postoperative CSF leaks or meningitis in the rest of the cases. In one patient, there was visual deterioration due to pressure on optic nerve by grafted fat and improved within 4 weeks. At 4 months after surgery, three patients had normal vision, two patients improved vision comparing with that of preoperative state but with some persisting deficit; one patient had static vision, no new endocrinopathy and no residual tumour on MRI in five cases but residual tumour in remaining case was static at the end of the ninth month. The endoscopic

  19. Erlotinib in Treating Patients With Recurrent Malignant Glioma or Recurrent or Progressive Meningioma

    ClinicalTrials.gov

    2014-07-09

    Adult Anaplastic Astrocytoma; Adult Anaplastic Oligodendroglioma; Adult Giant Cell Glioblastoma; Adult Glioblastoma; Adult Gliosarcoma; Adult Grade I Meningioma; Adult Grade II Meningioma; Adult Grade III Meningioma; Adult Mixed Glioma; Recurrent Adult Brain Tumor

  20. Discrimination between spinal extradural meningioma and both intra and extradural meningioma: Case Report.

    PubMed

    Ito, Kenyu; Imagama, Shiro; Ando, Kei; Kobayashi, Kazuyoshi; Hida, Tetsuro; Tsushima, Mikito; Matsumoto, Akiyuki; Morozumi, Masayoshi; Tanaka, Satoshi; Nishida, Yoshihiro; Ishiguro, Naoki

    2017-02-01

    Meningiomas are common spinal tumor and mostly located at intradura. Recurrence rate after surgery for extradural meningioma was higher than intradural meningioma. A patient with intra and extradural spinal meningioma was treated and discussed its features and clinical management. A 41-year-old woman noted numbness of bilateral legs, gait disturbance, and mild bladder disturbance for over the two-month period. Magnetic resonance imaging revealed an epidural mass at T8-9 involving the dura mater from the left side. Intraoperatively, ultrasonography showed extradural tumor suppress the dura from the left side and no subarachnoid space. Therefore, it was difficult to diagnose the tumor was located at only extradural or both intra and extradural. The extradural tumor was resected as much as possible, then ultrasonography was performed for the second time. The spinal cord was decompressed and subarachnoid space was appeared with intradural tumor. The dura mater was opened in the midline for intradural exploration, and the intradural tumor appeared beside the spinal cord. The dura mater with attachment to the tumor was rescected. To prevent a recurrence, ultrasonography after removing extradural tumor is recommended to detect intra dural tumor and invasion of tumor into dura mater. In such case, removing only extradural meningioma is not enough, and gross total resection including intradural meningioma and dura mater is required.

  1. Discrimination between spinal extradural meningioma and both intra and extradural meningioma: Case Report

    PubMed Central

    Ito, Kenyu; Imagama, Shiro; Ando, Kei; Kobayashi, Kazuyoshi; Hida, Tetsuro; Tsushima, Mikito; Matsumoto, Akiyuki; Morozumi, Masayoshi; Tanaka, Satoshi; Nishida, Yoshihiro; Ishiguro, Naoki

    2017-01-01

    ABSTRACT Meningiomas are common spinal tumor and mostly located at intradura. Recurrence rate after surgery for extradural meningioma was higher than intradural meningioma. A patient with intra and extradural spinal meningioma was treated and discussed its features and clinical management. A 41-year-old woman noted numbness of bilateral legs, gait disturbance, and mild bladder disturbance for over the two-month period. Magnetic resonance imaging revealed an epidural mass at T8-9 involving the dura mater from the left side. Intraoperatively, ultrasonography showed extradural tumor suppress the dura from the left side and no subarachnoid space. Therefore, it was difficult to diagnose the tumor was located at only extradural or both intra and extradural. The extradural tumor was resected as much as possible, then ultrasonography was performed for the second time. The spinal cord was decompressed and subarachnoid space was appeared with intradural tumor. The dura mater was opened in the midline for intradural exploration, and the intradural tumor appeared beside the spinal cord. The dura mater with attachment to the tumor was rescected. To prevent a recurrence, ultrasonography after removing extradural tumor is recommended to detect intra dural tumor and invasion of tumor into dura mater. In such case, removing only extradural meningioma is not enough, and gross total resection including intradural meningioma and dura mater is required. PMID:28303070

  2. Anaplastic and meningothelial meningiomas in a single tumor: A "dedifferentiated meningioma"?

    PubMed

    Shintaku, Masayuki; Adachi, Yasushi; Arai, Atsushi; Koyama, Junji

    2016-12-01

    The patient was a 74-year-old man, who developed progressive cognitive impairment and gait instability. Neuroradiological examination demonstrated a large and predominantly extra-axial tumor spreading over the bilateral frontal base, indicative of olfactory groove meningioma. The greater part of the resected tumor consisted of a dense, patternless proliferation of large, round or polygonal cells, and compactly fascicular growth of spindle cells. Tumor cells showed markedly anaplastic cytological features. In small areas of the tumor, a typical meningothelial meningioma showing no cellular atypism was found. Both tumor components were closely juxtaposed and no pathological features of an intermediate grade (atypical meningioma) were noted. Shortly after the operation, the patient developed a local recurrence of the tumor and multiple metastases to the cerebrum, bone and skin. Anaplastic meningioma is a rare, highly malignant neoplasm which arises de novo or as a result of the progressive transformation of a low-grade meningioma. The coexistence of anaplastic and low-grade components in a single meningeal tumor has been rarely reported. This dimorphic appearance is reminiscent of "dedifferentiation", a phenomenon infrequently seen in various mesenchymal and salivary gland neoplasms. We think that the term "dedifferentiated meningioma" can be appropriately applied to tumors such as that reported herein. © 2016 Japanese Society of Neuropathology.

  3. Signals of apoptotic pathways in several types of meningioma.

    PubMed

    Sabbatini, Maurizio; Comi, Cristoforo; Chiocchetti, Annalisa; Piffanelli, Valentina; Car, Pier Giorgio; Dianzani, Umberto; Monaco, Francesco; Cannas, Mario

    2011-03-01

    Meningiomas are intracranial tumour derived from meningothelial cells, which aggressive behaviour has been frequently associated to cell apoptosis. In this paper activation of several factors involved in apoptosis has been investigated on biopsies of primary, non recurrent meningiomas. Benign (meningotheliomatous, transitional, fibrous, angiomatous), atypical and anaplastic meningiomas were analysed by immunohistochemistry and western blot, to visualize the occurring of different apoptotic pathways and their association with clinical grading. Apoptotic cell have been detected by a double colorimetric staining for TUNEL and caspase-3 active form. Apoptotic signal positive cells have been detected in all type of meningiomas analysed, with exception of meningotheliomatous meningiomas. Differences have been found in the activation of apoptotic pathways between several types of grade I meningiomas and among benign, anaplastic and atypical meningiomas. An intense expression of several apoptotic inhibitor occurred in grade I meningiomas. The correlation among expression of apoptotic and inhibitory factors and cell proliferation index may suggest that in grade I meningiomas apoptosis may be related to mechanisms involved into tumor cells surviving. Instead in grade II and III meningiomas the same correlation seems indicate an high turnover of tumor cells that might be useful as index of cell proliferation and tumor mass growth.

  4. [Cytogenetic aberrations in histologically benign infiltratively growing sphenoid wing meningiomas].

    PubMed

    Korshunov, A G; Cherekaev, V A; Bekiashev, A Kh; Sycheva, R V

    2007-01-01

    Meningiomas of the sphenoid wing (SW) frequently show an invasive pattern of growth and cause destruction of the adjacent structures. As a result, the rate of recurrent SW meningiomas is as high as 30%. Cytogenetic investigations showed no aberrations specific to invasively growing meningiomas. During this study, the authors evaluated 10 invasive and 5 non-invasive SW meningiomas via comparative genome hybridization (CGH) (matrix CGH), by using the gene chips of GenoSensor Array micromatrixes. The mean number of aberrations in the tumor cells was much greater in case of invasive meningiomas (67.4 versus 40.5 in case of non-invasive SW meningiomas. Furthermore, in invasive SW meningiomas, there were frequently losses in loci 1p, 6q, and 14q and gains in loci 15q and 10, which had been predetermined as molecular markers of stepwise progression of meningioma. Thus, the presence of a complex cytogenetic profile and progression-associated chromosome aberrations in benign SW meningiomas is linked with the increase of their invasive potential. Due to the fact that there are no well-defined adjuvant therapy regimens for recurring meningiomas at present, the revealed genomic aberrations may become potential targets for searching for drugs and a therapeutic intervention in future.

  5. SU11657 Enhances Radiosensitivity of Human Meningioma Cells

    SciTech Connect

    Milker-Zabel, Stefanie Bois, Angelika Zabel-du; Ranai, Gholamreza; Trinh, Thuy; Unterberg, Andreas; Debus, Juergen; Lipson, Kenneth E.; Abdollahi, Amir; Huber, Peter E.

    2008-03-15

    Purpose: To analyze the effect of the multireceptor tyrosine kinase inhibitor SU11657 (primarily vascular endothelial growth factor, platelet-derived growth factor) in combination with irradiation in freshly isolated primary human meningioma cells. Methods and Materials: Tumor specimens were obtained from meningioma patients undergoing surgery at the Department of Neurosurgery, University of Heidelberg, Germany. For the present study only cells up to passage 6 were used. Benign and atypical meningioma cells and human umbilical vein endothelial cells (HUVEC) were treated with SU11657 alone and in combination with 6-MV photons (0-10 Gy). Clonogenic survival and cell proliferation were determined alone and in coculture assays to determine direct and paracrine effects. Results: Radiation and SU11657 alone reduced cell proliferation in atypical and benign meningioma cells as well as in HUVEC in a dose-dependent manner. SU11657 alone also reduced clonogenic survival of benign and atypical meningioma cells. SU11657 increased radiosensitivity of human meningioma cells in clonogenic survival and cell number/proliferation assays. The anticlonogenic and antiproliferative effects alone and the radiosensitization effects of SU11657 were more pronounced in atypical meningioma cells compared with benign meningioma cells. Conclusion: Small-molecule tyrosine kinase inhibitors like SU11657 are capable of amplifying the growth inhibitory effects of irradiation in meningioma cells. These data provide a rationale for further clinical evaluation of this combination concept, especially in atypical and malignant meningioma patients.

  6. Dust in the magnetized sheath

    SciTech Connect

    Pandey, B. P.; Samarian, A.; Vladimirov, S. V.

    2007-09-15

    In the present work the structure of the magnetized sheath is investigated in the multifluid framework. The ambient magnetic field is assumed parallel to the wall and the effect of the plasma magnetization, plasma ionization, and plasma-neutral collisions on the sheath is examined. It is shown that the width of the non-neutral boundary layer is dependent on the collision frequencies as well as on the plasma magnetization. The size of the sheath layer can decrease with the increase in magnetic field. The increase in the ion-neutral collision can also adversely affect the sheath size. The equilibrium and levitation of the dust particles in a collisional magnetized sheath are shown to depend on the collision frequencies and on the magnetization. Further, the increase in the collision or magnetization invariably leads to the presence of the positively charged grains near the plasma wall suggesting that the grain levitation inside the charged layer is implicitly dependent on the plasma parameters in a nontrivial way.

  7. Intraventricular trigonal meningioma: Neuronavigation? No, thanks!

    PubMed Central

    Silva, Danilo O. A.; Matis, Georgios K.; Costa, Leonardo F.; Kitamura, Matheus A. P.; Birbilis, Theodossios A.; Azevedo Filho, Hildo R. C.

    2011-01-01

    Background: Most of the time meningiomas are benign brain tumors and surgical removal ensures cure in the vast majority of the cases. Thus, whenever possible, complete surgical resection should be the goal of the treatment. Methods: This is a report of our surgical technique for the operative resection of a trigonal meningioma in a resource-limited setting. The necessity of accurate and deep knowledge of the regional anatomy is outlined. Results: A 44-year-old male presented to our outpatient clinic complaining of cephalalgia increasing in frequency and intensity over the last month. His neurological exam was normal, yet a brain computed tomography scan revealed a lesion in the right trigone of the ventricular system. The diagnosis of possible meningioma was set. After thoroughly informing the patient, tumor resection was decided. An intraparietal sulcus approach was favored without the use of any modern technological aids such as intraoperative magnetic resonance imaging or neuronavigation. The postoperative course was uneventful and a postoperative computed tomography scan demonstrated the complete resection of the tumor. The patient was discharged two days later with no neurological deficits. In a two-year-follow-up he remains recurrence-free. Conclusion: In the current cost-effective era it is still possible to safely remove an intraventricular trigonal meningioma without the convenience of neuronavigation. Since the best neuronavigator is the profound neuroanatomical knowledge, no technological advancement could replace a well-educated and trained neurosurgeon. PMID:21886886

  8. Occipital Falcine Anaplastic Hemangiopericytoma Mimicking Meningioma

    PubMed Central

    Kanesen, Davendran; Kandasamy, Regunath; Idris, Zamzuri

    2016-01-01

    The rarity of hemangiopericytoma (HPC) and its controversial histological classification result in its frequent misdiagnosis and thus make the treatment quite challenging. It is often difficult to distinguish these tumors from meningiomas based on clinical features and radiological findings. This is a case report of a man, diagnosed clinically and radiologically as meningioma, which turned out to be anaplastic HPC on histological examination. A 30-year-old man presented with 3 months of progressively worsening of headache and blurring of vision. Clinical examination revealed the right homonymous hemianopia with reduced visual acuity and papilledema bilaterally. Magnetic resonance imaging revealed a multilobulated and heterogenous extraaxial lesion attached to the occipital falx. It measured 9.0 cm (AP) × 5.5 cm (W) × 5.8 cm (CC) and expands bilaterally with major bulk on the left. An occipital craniotomy followed by a subtotal tumor excision was only achieved due to profuse bleeding intraoperatively. Histopathology confirmed an anaplastic HPC (WHO Grade 3). The importance of differentiation between HPCs and meningiomas cannot be overemphasized. A preoperative correct diagnosis is difficult, but it is important that it should be made. Multilobulated (mushroom appearance), prominent internal signal voids, relatively narrow dural attachment, and lytic destruction without calcifications are useful findings to distinguish HPCs from meningiomas. PMID:28163517

  9. Occipital Falcine Anaplastic Hemangiopericytoma Mimicking Meningioma.

    PubMed

    Kanesen, Davendran; Kandasamy, Regunath; Idris, Zamzuri

    2016-12-01

    The rarity of hemangiopericytoma (HPC) and its controversial histological classification result in its frequent misdiagnosis and thus make the treatment quite challenging. It is often difficult to distinguish these tumors from meningiomas based on clinical features and radiological findings. This is a case report of a man, diagnosed clinically and radiologically as meningioma, which turned out to be anaplastic HPC on histological examination. A 30-year-old man presented with 3 months of progressively worsening of headache and blurring of vision. Clinical examination revealed the right homonymous hemianopia with reduced visual acuity and papilledema bilaterally. Magnetic resonance imaging revealed a multilobulated and heterogenous extraaxial lesion attached to the occipital falx. It measured 9.0 cm (AP) × 5.5 cm (W) × 5.8 cm (CC) and expands bilaterally with major bulk on the left. An occipital craniotomy followed by a subtotal tumor excision was only achieved due to profuse bleeding intraoperatively. Histopathology confirmed an anaplastic HPC (WHO Grade 3). The importance of differentiation between HPCs and meningiomas cannot be overemphasized. A preoperative correct diagnosis is difficult, but it is important that it should be made. Multilobulated (mushroom appearance), prominent internal signal voids, relatively narrow dural attachment, and lytic destruction without calcifications are useful findings to distinguish HPCs from meningiomas.

  10. Paraclinoid aneurysm concealed by sphenoid wing meningioma.

    PubMed

    Petrecca, Kevin; Sirhan, Denis

    2009-02-01

    The coexistence of brain tumours and aneurysms is rare. In all previously reported cases the aneurysm was detectable by angiography. We report here a case in which a paraclinoid internal carotid artery aneurysm was coexistent and concealed from angiographic detection by an adjacent parasellar meningioma.

  11. Metaplastic conus meningioma and its applied aspects

    PubMed Central

    Reddy, Srikanth; Pulligopu, Aneel Kumar; Kumar, Ashish; Sahu, Barada Prasad

    2015-01-01

    Cranial meningiomas may present with or without major deficits depending on their location and morphology. Spinal meningiomas account for 25% of all the spinal tumors. Most commonly encountered in the dorsal spine in middle-aged females, these intradural-extramedullary tumors can usually be excised without difficulty. Rarely, they can be encountered in the lumbar spine and may create many tactical problems for the surgical team especially if it is calcified and contains metaplastic bone. We report a 55-year-old patient who presented with backache and investigations revealed a calcified lesion at L1 level that was thought of as an ependymoma initially. Intraoperatively, the lesion was calcified in parts, and the histopathology revealed it to be a metaplastic meningioma with calcification. The patient had a transient hip weakness, which recovered completely later on. Calcification in meningiomas is common. However, frank bony metaplasia is extremely rare. More importantly, the applied aspect of their presence in the region of conus lies in the careful dissection to prevent damage to the surrounding neural structures. Awareness regarding the same may help us preventing complications in the future. PMID:25972958

  12. Metaplastic conus meningioma and its applied aspects.

    PubMed

    Reddy, Srikanth; Pulligopu, Aneel Kumar; Kumar, Ashish; Sahu, Barada Prasad

    2015-01-01

    Cranial meningiomas may present with or without major deficits depending on their location and morphology. Spinal meningiomas account for 25% of all the spinal tumors. Most commonly encountered in the dorsal spine in middle-aged females, these intradural-extramedullary tumors can usually be excised without difficulty. Rarely, they can be encountered in the lumbar spine and may create many tactical problems for the surgical team especially if it is calcified and contains metaplastic bone. We report a 55-year-old patient who presented with backache and investigations revealed a calcified lesion at L1 level that was thought of as an ependymoma initially. Intraoperatively, the lesion was calcified in parts, and the histopathology revealed it to be a metaplastic meningioma with calcification. The patient had a transient hip weakness, which recovered completely later on. Calcification in meningiomas is common. However, frank bony metaplasia is extremely rare. More importantly, the applied aspect of their presence in the region of conus lies in the careful dissection to prevent damage to the surrounding neural structures. Awareness regarding the same may help us preventing complications in the future.

  13. Paraneoplastic symptoms caused by extracranial meningioma metastases?

    PubMed Central

    Mindermann, Thomas

    2016-01-01

    Background: There are only few reports on distant metastases of cranial meningiomas WHO I. In one-third of the cases, distant metastases seem to be clinically silent. This is the first case of distant metastases which may have manifested with a paraneoplastic syndrome. Case Description: A 52-year-old white male patient was diagnosed with distant metastases to the bones and liver 11 and 12 years following craniotomy and removal of a tentorial meningioma WHO I. At that time, the patient had developed paresthesia, unsteady gait, and a slight cognitive impairment, which in retrospect had no other explanation than that of a paraneoplastic syndrome. Eighteen years following craniotomy, a small intracranial tumor rest is under control following two single session radiosurgery treatments. At present, the patient has a multitude of bone and liver metastases, which seem to cause his paraneoplastic symptoms. Conclusion: Screening for malignancies in patients with paraneoplastic symptoms and a history of cranial meningioma should include screening for distant metastases from the meningioma. PMID:28168092

  14. Postoperative Analgesia Using Psoas Sheath Block Versus Three-in-One Block in Anterior Cruciate Ligament Reconstruction

    DTIC Science & Technology

    1999-10-01

    were blocked and two cases where all nerves were blocked. Key Words: Regional Anesthesia; Lumbar Plexus Block, Postoperative Pain Management; Pre...and sciatic(Lumbar4-Sacral3). A "psoas sheath block" and a "three-in-one block" are two techniques used to block the lumbar plexus from which the...nerve blockade Operational definition. A regional anesthetic technique used to block transmission of the nerves of the lumbar plexus including the

  15. Progress on Quantitative Modeling of rf Sheaths

    NASA Astrophysics Data System (ADS)

    D'Ippolito, D. A.; Myra, J. R.; Kohno, H.; Wright, J. C.

    2011-12-01

    A new quantitative approach for computing the rf sheath potential is described, which incorporates plasma dielectric effects and the relative geometry of the magnetic field and the material boundaries. The new approach uses a modified boundary condition ("rf sheath BC") that couples the rf waves and the sheaths at the boundary. It treats the sheath as a thin vacuum region and matches the fields across the plasma-vacuum boundary. When combined with the Child-Langmuir Law (relating the sheath width and sheath potential), the model permits a self-consistent determination of the sheath parameters and the rf electric field at the sheath-plasma boundary. Semi-analytic models using this BC predict a number of general features, including a sheath voltage threshold, a dimensionless parameter characterizing rf sheath effects, and the existence of sheath plasma waves with an associated resonance. Since the sheath BC is nonlinear and dependent on geometry, computing the sheath potential numerically is a challenging computational problem. Numerical results will be presented from a new parallel-processing finite-element rf wave code for the tokamak scrape-off layer (called "rfSOL"). The code has verified the physics predicted by analytic theory in 1D, and extended the solutions into model 2D geometries. The numerical calculations confirm the existence of multiple roots and hysteresis effects, and parameter studies have been carried out. Areas for future work will be discussed.

  16. Uptake of AV-1451 in meningiomas.

    PubMed

    Bruinsma, Tyler J; Johnson, Derek R; Fang, Ping; Senjem, Matthew; Josephs, Keith A; Whitwell, Jennifer L; Boeve, Bradley F; Pandey, Mukesh K; Kantarci, Kejal; Jones, David T; Vemuri, Prashanthi; Murray, Melissa; Graff-Radford, Jonathan; Schwarz, Christopher G; Knopman, David S; Petersen, Ronald C; Jack, Clifford R; Lowe, Val J

    2017-09-08

    AV-1451 is an imaging agent labeled with the positron-emitting radiolabel Fluorine-18. 18F-AV-1451 binds paired helical filament tau (PHF-tau), a pathology related to Alzheimer's disease. In our study of AV-1451 uptake in the brains of cognitively normal subjects, we noted a case of a meningioma with visually significant uptake of AV-1451. We initiated the present retrospective study to further examine cases of meningioma that underwent AV-1451 imaging. We searched the patient records of 650 patients who had undergone AV-1451 at our institution for the keyword "meningioma" to identify potential cases. PET/CT and MRI results were visually reviewed and semi-quantitative analysis of PET was performed. A paired student's t test was run between background and tumor standard uptake values. Fisher's exact test was used to examine the association between AV-1451 uptake and presence of calcifications on CT. We identified 12 cases of meningioma, 58% (7/12) of which demonstrated uptake greater than background using both visual analysis and tumor-to-normal cortex ratios (T/N + 1.90 ± 0.83). The paired student's t test revealed no statistically significant difference between background and tumor standard uptake values (p = 0.09); however, cases with a T/N ratio greater than one showed statistically higher uptake in tumor tissue (p = 0.01). A significant association was noted between AV-1451 uptake and presence of calcifications (p = 0.01). AV-1451 PET imaging should be reviewed concurrently with anatomic imaging to prevent misleading interpretations of PHF-tau distribution due to meningiomas.

  17. Immediate versus delayed primary nerve repair in the rabbit sciatic nerve

    PubMed Central

    Piskin, Ahmet; Altunkaynak, Berrin Zühal; Çιtlak, Atilla; Sezgin, Hicabi; Yazιcι, Ozgür; Kaplan, Süleyman

    2013-01-01

    It is well known that peripheral nerve injury should be treated immediately in the clinic, but in some instances, repair can be delayed. This study investigated the effects of immediate versus delayed (3 days after injury) neurorrhaphy on repair of transected sciatic nerve in New Zealand rabbits using stereological, histomorphological and biomechanical methods. At 8 weeks after immediate and delayed neurorrhaphy, axon number and area in the sciatic nerve, myelin sheath and epineurium thickness, Schwann cell morphology, and the mechanical property of nerve fibers did not differ obviously. These results indicate that delayed neurorrhaphy do not produce any deleterious effect on sciatic nerve repair. PMID:25206663

  18. Meningioma in Breast Cancer Patients: Population-based Analysis of Clinicopathologic Characteristics.

    PubMed

    Milano, Michael T; Grossman, Craig E

    2017-02-01

    Although an association between meningioma and breast cancer (BC) has been postulated, clear mechanisms remain obscure. By conducting population-based analyses in women with both BC and meningioma, hypothesis-generating causal links were pursued. Using the US SEER 18 registry (2004 to 2009), clinicopathologic and demographic characteristics from cohorts of women with only BC (n=279,821) or meningioma (n=19,570) diagnoses were compared with 412 women with both diagnoses (BC-meningioma). BC diagnosis preceded meningioma by >2 months in 48% of women; 20% had synchronous (within 2 mo) disease. Median meningioma size was 1.9 and 2.4 cm in the BC-meningioma and meningioma cohorts, respectively (P=0.0009). Among BC-meningioma patients, meningioma size was similar whether diagnosed >2 months prior, synchronously, or >2 months after BC. Meningioma was pathologically confirmed in 38% of BC-meningioma and 51% of meningioma patients. Distribution of BC histologies was comparable in patients with and without meningioma, with ductal type predominating (80% in BC-meningioma, 83% in BC). Although hormone receptor status of invasive BC was not significantly different between BC-meningioma and BC groups, the BC-meningioma cohort had fewer women with ER+/PR+ in situ disease (P=0.006). BC stage among women with meningioma was more advanced versus women with BC only. Women with BC and meningioma have smaller-sized meningiomas and more advanced BCs compared with women having only 1 diagnosis. As there was no temporal relationship between size and latency between tumor diagnoses, the disparity in meningioma size between BC-meningioma and meningioma cohorts may have BC-associated biological components that warrant further study.

  19. Relationship between myelin sheath diameter and internodal length in axons of the anterior medullary velum of the adult rat.

    PubMed

    Ibrahim, M; Butt, A M; Berry, M

    1995-11-01

    Relations between myelin sheath diameters and internodal lengths were measured in whole mounts of osmium stained intact anterior medullary velum (AMV) from glutaraldehyde perfused adult rats. The AMV is a sheet of CNS tissue which roofs the IVth ventricle and contains fascicles of myelinated fibres which arise mainly from the nucleus of the IVth cranial nerve. These fibers displayed a broad range of myelin sheath external diameters and internodal lengths, from < 1-12 microns and 50-750 microns, respectively. Myelin sheath external diameter was a measurement of the axonal diameter plus the thickness of its myelin sheath, while internodal length was measured as the distance between consecutive nodes. There was a broadly linear relationship between myelin sheath diameters and internodal lengths, with the smaller diameter sheaths tending to have shorter internodes than the larger. However, the correlation was weak and for any given diameter myelin sheaths displayed considerable variation in their internodal lengths. The smallest diameter myelin sheaths, < 4 microns, consistently had shorter internodes than predicted by a linear regression and, in an analysis of consecutive internodes in single fibres, the slope was flattened in fibres with a diameter > 4 microns. Our results indicated that small and large calibre fibres may have different myelin sheath diameter-internodal length interrelations.

  20. 5-Aminolevulinic acid fluorescence-guided surgery for spinal meningioma.

    PubMed

    Muroi, Carl; Fandino, Javier; Coluccia, Daniel; Berkmann, Sven; Fathi, Ali-Reza; Landolt, Hans

    2013-01-01

    Fluorescence-guided surgery for cranial meningioma has been reported to be useful. There are no reports about spinal cases using this technique. We report on a meningioma of the cervical spine for which fluorescence-guided surgery was used. A 78-year-old female patient with a meningioma located in the cervical spine underwent surgery using fluorescence guidance. After complete removal of the meningioma and removal and coagulation of the dural attachment (equal to Simpson grade II resection), a fluorescence-positive remnant could be identified and successfully removed. The remnant was found to harbor meningioma tissue on histological examination. Fluorescence-guided microsurgery is helpful in achieving a total resection of spinal meningiomas, and might therefore reduce the risk of recurrence. Copyright © 2013 Elsevier Inc. All rights reserved.

  1. Pathology and Molecular Genetics of Meningioma: Recent Advances

    PubMed Central

    SHIBUYA, Makoto

    2015-01-01

    Meningiomas are the most common intracranial primary neoplasm in adults. Although the spectrum of clinical and molecular genetic issues regarding meningiomas remains undefined, novel genetic alterations that are associated with tumor morphology, malignancy, or location have recently been discovered. This review focuses on recent advances in understanding of the heterogenous pathology of meningiomas, particularly on associations between the clinical, histological, etiological, epidemiological, and molecular genetical aspects of the neoplasm. PMID:25744347

  2. [Primary osteolytic intraosseous meningioma of the occipital bone].

    PubMed

    Bernal-García, Luis Miguel; Cabezudo-Artero, José Manuel; Marcelo-Zamorano, María Bella; Fernández-Alarcón, Luis; Gilete-Tejero, Ignacio

    2015-01-01

    Primary intraosseous meningiomas are considered extradural meningiomas when no dural attachment is present. Most of them arise from the cranial bones and can present either as an osteoblastic or an osteolytic lesion. Osteolytic intraosseous meningiomas are the rarest and very few cases have been reported. Given that many of these may develop signs of malignancy, early histological confirmation is important in order to ensure appropriate treatment. The recommended therapy is surgery, with complete resection whenever possible. We present the case of a large primary intraosseous osteolytic meningioma within the occipital bone, which was completely excised five years ago, currently presenting no signs of recurrence.

  3. Fatty acid synthase as a novel target for meningioma therapy

    PubMed Central

    Haase, Daniela; Schmidl, Stefan; Ewald, Christian; Kalff, Rolf; Huebner, Christian; Firsching, Raimund; Keilhoff, Gerburg; Evert, Matthias; Paulus, Werner; Gutmann, David H.; Lal, Anita; Mawrin, Christian

    2010-01-01

    High levels of fatty acid synthase (FAS) expression have been reported in hormone receptor-positive tumors, including prostate, breast, and ovarian cancers, and its inhibition reduces tumor growth in vitro and in vivo. Similar to other hormone receptor-positive tumor types, meningiomas are progesterone receptor- and estrogen receptor-immunoreactive brain tumors. To define the role of FAS in human meningioma growth control, we first analyzed the FAS expression using a tissue microarray containing 38 meningiomas and showed increased FAS expression in 70% of atypical WHO grade II and anaplastic WHO grade III meningiomas compared with 10% of benign WHO grade I tumors. We next confirmed this finding by real-time PCR and Western blotting. Second, we demonstrated that treatment with the FAS inhibitor, cerulenin (Cer), significantly decreased meningioma cell survival in vitro. Third, we showed that Cer treatment reduced FAS expression by modulating Akt phosphorylation (activation). Fourth, we demonstrated that Cer treatment of mice bearing meningioma xenografts resulted in significantly reduced tumor volumes associated with increased meningioma cell death. Collectively, our data suggest that the increased FAS expression in human meningiomas represents a novel therapeutic target for the treatment of unresectable or malignant meningioma. PMID:20511185

  4. Review of controversies in management of non-benign meningioma.

    PubMed

    Paldor, Iddo; Awad, Mohammed; Sufaro, Yuval Z; Kaye, Andrew H; Shoshan, Yigal

    2016-09-01

    Meningiomas are one of the most common brain tumors. World Health Organisation (WHO) Grade II and Grade III meningiomas are grouped together as non-benign meningioma (NBM). There are several controversies surrounding NBM management, including the significance of extent of resection and the efficacy of post-operative radiation and drug treatment. We reviewed the literature to develop recommendations for management of NBM. The questions we sought to answer were: Does gross total resection (GTR) improve patient outcome? Is radiation therapy (RT) warranted after complete or after incomplete resection of NBM? What drug therapies have been proven to improve outcome in patients with NBM? We found that GTR improves outcome in WHO Grade II meningioma, and should be attempted whenever considered safe. GTR correlates less closely to outcome in Grade III meningioma compared to subtotal resection (STR). Extreme measures to completely resect Grade III meningioma are not warranted. RT following GTR of Grade II meningioma does not improve patient outcome, and may be reserved for recurrence. RT improves outcome following STR of Grade II meningioma. RT improves outcome after resection of Grade III meningioma. No drug therapy has been shown to improve outcome in NBM. This review elucidates recommendations for some of the controversies involving NBM. Copyright © 2016 Elsevier Ltd. All rights reserved.

  5. Clear cell meningioma: case report and review of the literature.

    PubMed

    Oviedo, Angelica; Pang, Dachling; Zovickian, John; Smith, Matthew

    2005-01-01

    Clear cell meningioma (CCM) is a rare variant of meningioma. Only 17 cases have been previously reported in children. Although it has bland cytologic features, it has a higher rate of recurrence than does conventional meningioma. This variant has been reported in sites such as spinal/intradural (lumbar and thoracic), cerebellopontine angle, and supratentorial. The differential diagnosis of CCM includes microcystic meningioma, hemangioblastoma, and clear cell ependymoma. The characteristic histology and immunohistochemistry leads to the diagnosis. We present a case of a 7-year-old boy with a CCM of the cauda equina and a review of pediatric CCM.

  6. Results of stereotactic radiosurgery for patients with imaging defined cavernous sinus meningiomas

    SciTech Connect

    Pollock, Bruce E. . E-mail: pollock.bruce@mayo.edu; Stafford, Scott L.

    2005-08-01

    Introduction: The purpose of this study was to evaluate the efficacy and safety of stereotactic radiosurgery as primary management for patients with imaging defined cavernous sinus meningiomas. Methods: Between 1992 and 2001, 49 patients had radiosurgery for dural-based masses of the cavernous sinus presumed to be meningiomas. The mean patient age was 55.5 years. The mean tumor volume was 10.2 mL; the mean tumor margin dose was 15.9 Gy. The mean follow-up was 58 months (range, 16-144 months). Results: No tumor enlarged after radiosurgery. Twelve of 38 patients (26%) with preexisting diplopia or facial numbness/pain had improvement in cranial nerve function. Five patients (10%) had new (n = 3) or worsened (n = 2) trigeminal dysfunction; 2 of these patients (4%) underwent surgery at 20 and 25 months after radiosurgery despite no evidence of tumor progression. Neither patient improved after partial tumor resection. One patient (2%) developed an oculomotor nerve injury. One patient (2%) had an ischemic stroke related to occlusion of the cavernous segment of the internal carotid artery. Event-free survival was 98%, 85%, and 80% at 1, 3, and 7 years after radiosurgery, respectively. Univariate analysis of patient and dosimetric factors found no analyzed factor correlated with postradiosurgical morbidity. Conclusions: Radiosurgery was an effective primary management strategy for patients with an imaging defined cavernous sinus meningioma. Except in situations of symptomatic mass effect, unusual clinical presentation, or atypical imaging features, surgery to confirm the histologic diagnosis is unlikely to provide clinical benefit.

  7. Open Transcranial Resection of Small (<35 mm) Meningiomas of the Anterior Midline Skull Base in Current Microsurgical Practice.

    PubMed

    Della Puppa, Alessandro; d'Avella, Elena; Rossetto, Marta; Volpin, Francesco; Rustemi, Oriela; Gioffrè, Giorgio; Lombardi, Giuseppe; Rolma, Giuseppe; Scienza, Renato

    2015-09-01

    Despite technical surgical advance, the ultimate management of midline anterior skull base meningiomas remains to be defined. Open transcranial surgery is usually the first treatment option for large meningiomas, while less invasive techniques such as endoscopic surgery or radiosurgery might represent an alternative to open microsurgery for smaller lesions. The aim of our study is to investigate the outcome of open transcranial microsurgery in the resection of small (<35 mm) meningiomas of the midline anterior cranial base. Clinical and surgical data from 43 patients affected by small midline anterior skull base meningiomas operated via an open transcranial approach were retrospectively reviewed. The tumor diameter on its major axis ranged from 12 to 35 mm, with a mean diameter of 28 mm. Gross total resection (Simpson grades I-II) was achieved in 100% of cases through a pterional approach. Postoperative overall morbidity was 9%. It was 3% among patients <70 years. No mortality was reported. Postoperative visual outcome was significantly associated with preoperative visual performance (P = 0.02), but not with preoperative optic nerve compression as detected by magnetic resonance imaging (P = 0.116). Age >70 years was associated with postoperative visual impairment, although not significantly (P = 0.06). Visual function was preserved or improved in 95% of cases, in 100% of patients <70 years, and in 71% of patients with preoperative visual impairment. In our experience, open transcranial surgery proved safe and effective for midline anterior skull base meningiomas smaller than 35 mm in all patients <70 years and in patients >70 years without preoperative visual deficit. Our data are consistent with the literature. Conversely, the standard of treatment for the subgroup of patients >70 years with preoperative visual deficit has not yet been defined. This specific subgroup of patients offers a topic for further investigation. Copyright © 2015 Elsevier Inc. All rights

  8. Osteolytic clear cell meningioma of the petrous bone occurring 36 years after posterior cranial fossa irradiation: Case report.

    PubMed

    Ben Nsir, A; Ben Hamouda, K; Hammedi, F; Kilani, M; Hattab, N

    2016-01-01

    While bone invasion and hyperostosis are frequent phenomena in meningiomas, primary intraosseous meningiomas are rare and their occurrence in the skull base is an extraordinary exception. Moreover, radiation-induced meningiomas represent a unique clinical dilemma given the fact that patients with these tumors had often received a prior full course of radiotherapy. A 42-year-old man presented with a 3-month history of progressively worsening facial asymmetry. His medical history was consistent for a posterior cranial fossa irradiation at the age of 6 years for a non-confirmed brain stem tumor. On admission his Karnofsky performance status was graded as 50% and his neurological examination showed a complete right facial nerve paralysis and hearing impairment. Computed tomography and magnetic resonance imaging demonstrated an osteolytic tumor invading the whole right petrous bone without intracranial involvement. As the tumor reached the external auditory canal, a tissue sample was obtained locally. Pathological examination of the lesion identified a grade II clear cell meningioma and the patient was consequently addressed for an intensity modulated radiation therapy. His condition remained unchanged till the most recent follow-up examination, 8 months later. To the best of our knowledge, a radiation induced osteolytic clear cell meningioma of the petrous bone has not been previously reported. As little literature exists regarding the use of adjuvant therapies for these tumors, intensity modulated radiation therapy remains an attractive treatment option in case of pervious irradiation and general status alteration. Copyright © 2016 Polish Neurological Society. Published by Elsevier Urban & Partner Sp. z o.o. All rights reserved.

  9. Stereotactic radiotherapy of meningiomas compressing optical pathways

    SciTech Connect

    Hamm, Klaus-Detlef . E-mail: khamm@erfurt.helios-kliniken.de; Henzel, Martin; Gross, Markus W.; Surber, Gunnar; Kleinert, Gabriele; Engenhart-Cabillic, Rita

    2006-11-15

    Purpose: Microsurgical resection is usually the treatment of choice for meningiomas, especially for those that compress the optical pathways. However, in many cases of skull-base meningiomas a high risk of neurological deficits and recurrences exist in cases where the complete tumor removal was not possible. In such cases (fractionated) stereotactic radiotherapy (SRT) can offer an alternative treatment option. We evaluated the local control rate, symptomatology, and toxicity. Patients and Methods: Between 1997 and 2003, 183 patients with skull-base meningiomas were treated with SRT, among them were 65 patients with meningiomas that compressed optical pathways (64 benign, 1 atypical). Of these 65 cases, 20 were treated with SRT only, 27 were subtotally resected before SRT, and 18 underwent multiple tumor resections before SRT. We investigated the results until 2005, with a median follow-up of 45 months (range, 22-83 months). The tumor volume (TV = gross tumor volume) ranged from 0.61 to 90.20 cc (mean, 18.9 cc). Because of the risk of new visual disturbances, the dose per fraction was either 2 or 1.8 Gy for all patients, to a total dose of 50 to 60 Gy. Results: The overall survival and the progression-free survival rates for 5 years were assessed to 100% in this patient group. To date, no progression for these meningiomas have been observed. Quantitatively, tumor shrinkage of more than 20%, or more than 2 mm in diameter, was proved in 35 of the 65 cases after SRT. In 29 of the 65 patients, at least 1 of the symptoms improved. On application of the Common Toxicity Criteria (CTC), acute toxicity (Grade 3) was seen in 1 case (worsening of conjunctivitis). Another 2 patients developed late toxicity by LENT-SOMA score, 1 x Grade 1 and 1 x Grade 3 (field of vision loss). Conclusion: As a low-risk and effective treatment option for tumor control, SRT with 1.8 to 2.0 Gy per fraction can also be recommended in case of meningiomas that compress optical pathways. An

  10. Nerve Regeneration in the Peripheral Nervous System versus the Central Nervous System and the Relevance to Speech and Hearing after Nerve Injuries

    ERIC Educational Resources Information Center

    Gordon, Tessa; Gordon, Karen

    2010-01-01

    Schwann cells normally form myelin sheaths around axons in the peripheral nervous system (PNS) and support nerve regeneration after nerve injury. In contrast, nerve regeneration in the central nervous system (CNS) is not supported by the myelinating cells known as oligodendrocytes. We have found that: 1) low frequency electrical stimulation can be…

  11. Nerve Regeneration in the Peripheral Nervous System versus the Central Nervous System and the Relevance to Speech and Hearing after Nerve Injuries

    ERIC Educational Resources Information Center

    Gordon, Tessa; Gordon, Karen

    2010-01-01

    Schwann cells normally form myelin sheaths around axons in the peripheral nervous system (PNS) and support nerve regeneration after nerve injury. In contrast, nerve regeneration in the central nervous system (CNS) is not supported by the myelinating cells known as oligodendrocytes. We have found that: 1) low frequency electrical stimulation can be…

  12. Sheath-in-Sheath Technique for Exteriorization of Body Floss Wire.

    PubMed

    Pua, Uei

    2017-05-01

    In this technique, we describe the insertion of a second sheath into the primary sheath containing a guidewire that is meant to be exteriorized. The second sheath serves to open the valve of the primary sheath and creates a water-tight chamber for the guidewire to enter. The second sheath is then removed, exposing the successfully exteriorized guidewire. This technique is an useful adjunct to conventional guidewire exteriorization techniques during body floss procedures. Copyright © 2017 Elsevier Inc. All rights reserved.

  13. Concurrent spinal schwannoma and meningioma mimicking a single cervical dumbbell-shaped tumor: case report.

    PubMed

    Oichi, Takeshi; Chikuda, Hirotaka; Morikawa, Teppei; Mori, Harushi; Kitamura, Daisuke; Higuchi, Junya; Taniguchi, Yuki; Matsubayashi, Yoshitaka; Oshima, Yasushi; Tanaka, Sakae

    2015-12-01

    Dumbbell-shaped tumors consisting of 2 different tumors are extremely rare. Herein, the authors present a case of concurrent spinal schwannoma and meningioma mimicking a single cervical dumbbell-shaped tumor. A 64-year-old man presented with a 5-year history of gradually exacerbating left occipital pain without clinical evidence of neurofibromatosis. Magnetic resonance imaging showed an extradural tumor along the left C-2 nerve root with a small intradural component. The tumor was approached via a C-1 hemilaminectomy. The intradural tumor was resected together with the extradural tumor after opening the dura mater. The intradural tumor was attached to the dura mater around the exit point of the C-2 nerve root. Intraoperative biopsy revealed that the extradural tumor was a schwannoma and that the intradural tumor was a meningioma. The dura mater adjacent to the tumor was then coagulated and resected. Postoperative pathological examination confirmed the same diagnoses with no evidence of continuity between the intra- and extradural components. The patient's postoperative clinical course was uneventful. Clinicians should be aware that cervical dumbbell-shaped tumors can consist of 2 different tumors.

  14. Association of Morbidity with Extent of Resection and Cavernous Sinus Invasion in Sphenoid Wing Meningiomas*

    PubMed Central

    Ivan, Michael E.; Cheng, Jason S.; Kaur, Gurvinder; Sughrue, Michael E.; Clark, Aaron; Kane, Ari J.; Aranda, Derick; McDermott, Michael; Barani, Igor J.; Parsa, Andrew T.

    2012-01-01

    Sphenoid wing meningiomas (SWMs) typically are histologically benign, insidious lesions, but the propensity of these tumors for local invasion makes disease control very challenging. In this review, we assess whether the degree of resection and extent of cavernous sinus invasion affects morbidity, mortality, and recurrence in patients with SWM. A comprehensive search of the English-language literature was performed. Patients were stratified according to extent of resection and extent of cavernous sinus invasion, and tumor recurrence rate, morbidity, and mortality were analyzed. A total of 23 studies and 131 patients were included. Overall recurrence and surgical mortality rate were 11% and 2%, respectively (average follow-up = 65 months). Cranial nerve III palsy was significantly associated with incompletely versus completely resected SWMs (7 to 0%) as well as meningiomas with cavernous sinus invasion versus no sinus invasion (14 vs. 0%). No significant difference in tumor recurrence rate was noted between these groups. In conclusion, complete excision of SWMs is always recommended whenever possible, but surgeons should acknowledge that there is nonetheless a chance of recurrence and should weigh this against the risk of causing cranial nerve injuries. PMID:23372999

  15. Nerve conduction and electromyography studies.

    PubMed

    Kane, N M; Oware, A

    2012-07-01

    Nerve conduction studies (NCS) and electromyography (EMG), often shortened to 'EMGs', are a useful adjunct to clinical examination of the peripheral nervous system and striated skeletal muscle. NCS provide an efficient and rapid method of quantifying nerve conduction velocity (CV) and the amplitude of both sensory nerve action potentials (SNAPs) and compound motor action potentials (cMAPs). The CV reflects speed of propagation of action potentials, by saltatory conduction, along large myelinated axons in a peripheral nerve. The amplitude of SNAPs is in part determined by the number of axons in a sensory nerve, whilst amplitude of cMAPs reflects integrated function of the motor axons, neuromuscular junction and striated muscle. Repetitive nerve stimulation (RNS) can identify defects of neuromuscular junction (NMJ) transmission, pre- or post-synaptic. Needle EMG examination can detect myopathic changes in muscle and signs of denervation. Combinations of these procedures can establish if motor and/or sensory nerve cell bodies or peripheral nerves are damaged (e.g. motor neuronopathy, sensory ganglionopathy or neuropathy), and also indicate if the primary target is the axon or the myelin sheath (i.e. axonal or demyelinating neuropathies). The distribution of nerve damage can be determined as either generalised, multifocal (mononeuropathy multiplex) or focal. The latter often due to compression at the common entrapment sites (such as the carpal tunnel, Guyon's canal, cubital tunnel, radial groove, fibular head and tarsal tunnel, to name but a few of the reported hundred or so 'entrapment neuropathies').

  16. Occipital Neuralgia as the Only Presenting Symptom of Foramen Magnum Meningioma

    PubMed Central

    Yang, Seung-Yeob; Koo, Joon-Bum; Jeong, Sang-Wuk

    2009-01-01

    Background Occipital neuralgia (ON) is a condition characterized by a paroxysmal stabbing pain in the area of the greater or lesser occipital nerves; it is usually regarded by clinicians as idiopathic. Some have suggested that ON can be induced by trauma or injury of the occipital nerves or their roots, but tumor has rarely been reported as a cause of ON. Case Report We report herein a case of foramen magnum meningioma in a 55-year-old woman who presented with ON triggered by head motion as the only symptom without any signs of myelopathy. Conclusions This case indicates that it is important to consider the underlying causes of ON. Precise neurologic and radiological evaluations such as cervical spine magnetic resonance imaging are needed. PMID:20076803

  17. Preoperative Visualization of Cranial Nerves in Skull Base Tumor Surgery Using Diffusion Tensor Imaging Technology.

    PubMed

    Ma, Jun; Su, Shaobo; Yue, Shuyuan; Zhao, Yan; Li, Yonggang; Chen, Xiaochen; Ma, Hui

    2016-01-01

    To visualize cranial nerves (CNs) using diffusion tensor imaging (DTI) with special parameters. This study also involved the evaluation of preoperative estimates and intraoperative confirmation of the relationship between nerves and tumor by verifying the accuracy of visualization. 3T magnetic resonance imaging scans including 3D-FSPGR, FIESTA, and DTI were used to collect information from 18 patients with skull base tumor. DTI data were integrated into the 3D slicer for fiber tracking and overlapped anatomic images to determine course of nerves. 3D reconstruction of tumors was achieved to perform neighboring, encasing, and invading relationship between lesion and nerves. Optic pathway including the optic chiasm could be traced in cases of tuberculum sellae meningioma and hypophysoma (pituitary tumor). The oculomotor nerve, from the interpeduncular fossa out of the brain stem to supraorbital fissure, was clearly visible in parasellar meningioma cases. Meanwhile, cisternal parts of trigeminal nerve and abducens nerve, facial nerve were also imaged well in vestibular schwannomas and petroclival meningioma cases. The 3D-spatial relationship between CNs and skull base tumor estimated preoperatively by tumor modeling and tractography corresponded to the results determined during surgery. Supported by DTI and 3D slicer, preoperative 3D reconstruction of most CNs related to skull base tumor is feasible in pathological circumstances. We consider DTI Technology to be a useful tool for predicting the course and location of most CNs, and syntopy between them and skull base tumor.

  18. Olfactory groove meningiomas: approaches and complications.

    PubMed

    Aguiar, Paulo Henrique Pires de; Tahara, Adriana; Almeida, Antonio Nogueira; Simm, Renata; Silva, Arnaldo Neves da; Maldaun, Marcos Vinicius Calfatt; Panagopoulos, Alexandros Theodoros; Zicarelli, Carlos Alexandre; Silva, Pedro Gabriel

    2009-09-01

    Olfactory groove meningiomas (OGM) account for 4.5% of all intracranial meningiomas. We report 21 patients with OGMs. Tumors were operated on using three surgical approaches: bifrontal (7 patients), fronto-pterional (11 patients) and fronto-orbital (3 patients). Total tumor removal (Simpson Grade 1) was achieved in 13 patients and Simpson II in 8 patients. Perioperative mortality was 4.76%. The average size of the OGM was 4.3+/-1.1cm. The overall recurrence rate was 19%. We preferred to use the pterional approach, which provides quick access to the tumor with less brain exposure. It also allows complete drainage of cisternal cerebrospinal fluid, providing a good level of brain relaxation during surgery. However, for long, thin tumors, hemostasis can be difficult using this approach.

  19. Epidural Cystic Spinal Meningioma: A Case Report.

    PubMed

    Zhang, Ji; Chen, Zheng-he; Wang, Zi-feng; Sun, Peng; Jin, Jie-tian; Zhang, Xiang-heng; Zhao, Yi-ying; Wang, Jian; Mou, Yong-gao; Chen, Zhong-ping

    2016-03-01

    Cystic spinal meningioma (CSM) is an uncommon meningioma variant. Extradural CSMs are particularly rare and difficult to distinguish from other intraaxial tumors. This study presents a case of a 36-year-old woman with intraspinal extradual CSM at the thoracolumbar spine. She experienced persistent weakness, progressive numbness, and sensory disturbance in the right lower limb. Magnetic resonance imaging (MRI) of the patient revealed an irregular cystic mass at the thoracic 11 to lumbar 3 levels dorsally. This case was misdiagnosed as other neoplasms prior to surgery because of the atypical radiographic features and location of the tumor. Extradural CSMs should be considered in the differential diagnosis of intraspinal extradural cystic neoplasms. Complete removal of cystic wall provides an optimal outcome, rendering the lesion curable.

  20. Treatment recommendations for primary extradural meningiomas.

    PubMed

    Mattox, Austin; Hughes, Betsy; Oleson, James; Reardon, David; McLendon, Roger; Adamson, Cory

    2011-01-01

    Primary extradural meningiomas (PEMs) represent about 2% of all meningiomas and are often encountered by non-neurosurgeons. These lesions typically present as enlarging, painless, benign masses that can be surgically cured. Imaging is critical for defining involvement of adjacent structures; however, diagnosis depends on classic histologic patterns. Treatment for benign PEMs (WHO I) consists of resection with wide margins, whereas adjuvant therapy after resection of atypical (WHO II) or malignant (WHO III) PEMs should be considered. By using the collective experience from our comprehensive cancer center, including neuro-oncologists, neuroradiologists, and neurosurgeons, in addition to a complete literature review, the authors have established treatment guidelines not previously reported. This manuscript describes key features of these challenging tumors to aid in diagnosis, presents the largest published review of all reported PEMs (n = 163), and provides salient treatment guidelines to surgeons unfamiliar with these challenging tumors.

  1. Gamma Knife radiosurgery for sellar and parasellar meningiomas: a multicenter study.

    PubMed

    Sheehan, Jason P; Starke, Robert M; Kano, Hideyuki; Kaufmann, Anthony M; Mathieu, David; Zeiler, Fred A; West, Michael; Chao, Samuel T; Varma, Gandhi; Chiang, Veronica L S; Yu, James B; McBride, Heyoung L; Nakaji, Peter; Youssef, Emad; Honea, Norissa; Rush, Stephen; Kondziolka, Douglas; Lee, John Y K; Bailey, Robert L; Kunwar, Sandeep; Petti, Paula; Lunsford, L Dade

    2014-06-01

    last clinical follow-up, 86.2% of patients demonstrated no change or improvement in their neurological condition, whereas 13.8% of patients experienced symptom progression. New or worsening cranial nerve deficits were seen in 9.6% of patients, with cranial nerve (CN) V being the most adversely affected nerve. Functional improvements in CNs, especially in CNs V and VI, were observed in 34% of patients with preexisting deficits. New or worsened endocrinopathies were demonstrated in 1.6% of patients; hypothyroidism was the most frequent deficiency. Unfavorable outcome with tumor growth and accompanying neurological decline was statistically more likely in patients with larger tumor volumes (p = 0.022) and more than 1 prior surgery (p = 0.021). Gamma Knife radiosurgery provides a high rate of tumor control for patients with parasellar or sellar meningiomas, and tumor control is accompanied by neurological preservation or improvement in most patients.

  2. LINAC radiosurgery in skull base meningiomas.

    PubMed

    Deinsberger, R; Tidstrand, J; Sabitzer, H; Lanner, G

    2004-12-01

    LINAC radiosurgery has provided increasing access and changed treatment strategies in patients with benign skull base meningiomas in recent years. From January 1996 to January 2003, 37 patients with skull base meningiomas were treated with LINAC radiosurgery. A combination of the University of Florida system and the X Knife System, developed by Radionics, was used in all patients. Eight patients were treated by microsurgical resection before radiosurgery, in two patients tumor recurrence was treated and in 29 patients LINAC radiosurgery was the initial treatment procedure. The median treatment volume was 5.9 mL (0.7 to 22 mL) and the median given dose was 14.6 Gy (12.8 to 18 Gy) prescribed to the 80 percent isodose line. The follow-up period is between 12 and 96 months, median 66 months. 12 patients (32.4 %) showed a significant reduction in tumor size, in 11 patients (29.7 %) there was significantly less contrast enhancement and in 13 patients (35.1 %) the skull base meningioma was without any change. In one patient (2.8 %) there was tumor growth 48 months after radiosurgery, so the tumor control rate was 97.2 %. Two patients (5.6 %) showed neurological worsening after radiosurgery, whereas the symptoms were transient in one patient. LINAC radiosurgery offers us an effective treatment modality and changes treatment strategies in skull base meningiomas towards a less aggressive surgical approach. By combining microsurgery and radiosurgery we can achieve high tumor control rates with an acceptable low morbidity and a high level of quality of life.

  3. Generalized sheath criterion for arbitrary degenerate plasmas

    NASA Astrophysics Data System (ADS)

    Akbari-Moghanjoughi, M.

    2017-01-01

    In this research, we study the generalized sheath criterion for plasmas with an arbitrary degree of electron degeneracy and temperature, ranging from the classical dilute regime to the fully degenerate quantum plasmas. The latter may be relevant to warm dense matter and/or laboratory high energy density matter or even astrophysical stellar plasmas. The hydrostatic one dimensional model is used to establish the generalized Bohm's criterion for sheath entrance ion speed limits, and the small amplitude theory of the sheath problem, which accurately describes the sheath parameters for lower ion acoustic Mach numbers, is developed. Our results indicate that the sheath characteristic parameters such as electrostatic potential and density profiles, as well as the wall potential and the sheath length, are significantly affected by plasma parameters such as the ion and electron temperature and number densities in the plasma region. In particular, there are fundamental differences between sheath structures of the dilute classical plasmas and those of dense quantum ones.

  4. Unusual median nerve schwannoma: a case presentation.

    PubMed

    Anghel, Andrea; Tudose, Irina; Terzea, Dana; Răducu, Laura; Sinescu, Ruxandra Diana

    2014-01-01

    Peripheral nerve sheath tumors are common soft tissue neoplasms and their characterization is often challenging. Although the surgical pathology defines some typical entities, some degree of controversy regarding the classification of these tumors still exists. Newer imagistic and histopathological techniques are crucial for their accurate diagnosis and grading. We present an unusual case of median nerve schwannoma in a young patient, discussing the clinical, surgical and pathological elements, including immunohistochemistry.

  5. Foramen Magnum Meningioma: Dysphagia of Atypical Etiology

    PubMed Central

    Tsang, Matthew W.; Mobley, Bret C.; Cheng, Walter W.

    2007-01-01

    INTRODUCTION We present a case of a foramen magnum meningioma that highlights the importance of the neurologic exam when evaluating a patient with dysphagia. A 58-year-old woman presented with an 18-month history of progressive dysphagia, chronic cough and 30-pound weight loss. Prior gastroenterologic and laryngologic workup was unrevealing. Results Her neurologic examination revealed an absent gag reflex, decreased sensation to light touch on bilateral distal extremities, hyperreflexia, and tandem gait instability. Repeat esophagogastroduodenoscopy was normal, whereas laryngoscopy and video fluoroscopy revealed marked hypopharyngeal dysfunction. Brain magnetic resonance imaging demonstrated a 3.1 × 2.7 × 2.9 cm foramen magnum mass consistent with meningioma. The patient underwent neurosurgical resection of her mass with near complete resolution of her neurologic symptoms. Pathology confirmed diagnosis of a WHO grade I meningothelial meningioma. Conclusion CNS pathology is an uncommon but impressive cause of dysphagia. Our case demonstrates the importance of a thorough neurologic survey when evaluating such a patient. PMID:18080720

  6. Chordoid Meningioma: Differentiating a Rare World Health Organization Grade II Tumor from Other Meningioma Histologic Subtypes Using MRI.

    PubMed

    Pond, J B; Morgan, T G; Hatanpaa, K J; Yetkin, Z F; Mickey, B E; Mendelsohn, D B

    2015-07-01

    Meningiomas are very commonly diagnosed intracranial primary neoplasms, of which the chordoid subtype is seldom encountered. Our aim was to retrospectively review preoperative MR imaging of intracranial chordoid meningiomas, a rare WHO grade II variant, in an effort to determine if there exist distinguishing MR imaging characteristics that can aid in differentiating this atypical variety from other meningioma subtypes. Ten cases of WHO grade II chordoid meningioma were diagnosed at our institution over an 11-year span, 8 of which had preoperative MR imaging available for review and were included in our analysis. Chordoid meningioma MR imaging characteristics, including ADC values and normalized ADC ratios, were compared with those of 80 consecutive cases of WHO grade I meningioma, 21 consecutive cases of nonchordoid WHO grade II meningioma, and 1 case of WHO grade III meningioma. Preoperative MR imaging revealed no significant differences in size, location, signal characteristics, or contrast enhancement between chordoid meningiomas and other meningiomas. There were, however, clear differences in the ADC values and normalized ADC ratios, with a mean absolute ADC value of 1.62 ± 0.33 × 10(-3) mm(2)/s and a mean normalized ADC ratio of 2.22 ± 0.47 × 10(-3) mm(2)/s in chordoid meningiomas compared with mean ADC and normalized ADC values, respectively, of 0.88 ± 0.13 × 10(-3) mm(2)/s and 1.17 ± 0.16 × 10(-3) mm(2)/s in benign WHO grade I meningiomas, 0.84 ± 0.11 × 10(-3) mm(2)/s and 1.11 ± 0.15 × 10(-3) mm(2)/s in nonchordoid WHO grade II meningiomas, and 0.57 × 10(-3) mm(2)/s and 0.75 × 10(-3) mm(2)/s in the 1 WHO grade III meningioma. Chordoid meningiomas have statistically significant elevations of ADC and normalized ADC values when compared with all other WHO grade I, II, and III subtypes, which enables reliable preoperative prediction of this atypical histopathologic diagnosis. © 2015 by American Journal of Neuroradiology.

  7. Chemically extracted acellular allogeneic nerve graft combined with ciliary neurotrophic factor promotes sciatic nerve repair

    PubMed Central

    Zhang, Yanru; Zhang, Hui; Katiella, Kaka; Huang, Wenhua

    2014-01-01

    A chemically extracted acellular allogeneic nerve graft can reduce postoperative immune rejection, similar to an autologous nerve graft, and can guide neural regeneration. However, it remains poorly understood whether a chemically extracted acellular allogeneic nerve graft combined with neurotrophic factors provides a good local environment for neural regeneration. This study investigated the repair of injured rat sciatic nerve using a chemically extracted acellular allogeneic nerve graft combined with ciliary neurotrophic factor. An autologous nerve anastomosis group and a chemical acellular allogeneic nerve bridging group were prepared as controls. At 8 weeks after repair, sciatic functional index, evoked potential amplitude of the soleus muscle, triceps wet weight recovery rate, total number of myelinated nerve fibers and myelin sheath thickness were measured. For these indices, values in the three groups showed the autologous nerve anastomosis group > chemically extracted acellular nerve graft + ciliary neurotrophic factor group > chemical acellular allogeneic nerve bridging group. These results suggest that chemically extracted acellular nerve grafts combined with ciliary neurotrophic factor can repair sciatic nerve defects, and that this repair is inferior to autologous nerve anastomosis, but superior to chemically extracted acellular allogeneic nerve bridging alone. PMID:25221592

  8. Ultrasound-guided rectus sheath block in children with umbilical hernia: Case series.

    PubMed

    Alsaeed, Abdul Hamid; Thallaj, Ahmed; Khalil, Nancy; Almutaq, Nada; Aljazaeri, Ayman

    2013-10-01

    Umbilical hernia repair, a common day-case surgery procedure in children, is associated with a significant postoperative pain. The most popular peripheral nerve blocks used in umbilical hernia repair are rectus sheath infiltration and caudal block. The rectus sheath block may offer improved pain relief following umbilical hernia repair with no undesired effects such as lower limb motor weakness or urinary retention seen with caudal block which might delay discharge from the hospital. Ultrasound guidance of peripheral nerve blocks has reduced the number of complications and improved the quality of blocks. The aim of this case series is to assess the post rectus sheath block pain relief in pediatric patients coming for umbilical surgery. Twenty two (22) children (age range: 1.5-8 years) scheduled for umbilical hernia repair were included in the study. Following the induction of general anesthesia, the ultrasonographic anatomy of the umbilical region was studied with a 5-16 MHz 50 mm linear probe. An ultrasound-guided posterior rectus sheath block of both rectus abdominis muscles (RMs) was performed (total of 44 punctures). An in-plain technique using Stimuplex A insulated facet tip needle 22G 50mm. Surgical conditions, intraoperative hemodynamic parameters, and postoperative analgesia by means of the modified CHEOPS scale were evaluated. ultrasonograghic visualization of the posterior sheath was possible in all patients. The ultrasound guided rectus sheath blockade provided sufficient analgesia in all children with no need for additional analgesia except for one patient who postoperatively required morphine 0.1 mg/kg intravenously. There were no complications. Ultrasound guidance enables performances of an effective rectus sheath block for umbilical hernia. Use of the Stimuplex A insulated facet tip needle 22G 50mm provides easy, less traumatic skin and rectus muscle penetration and satisfactory needle visualiza.

  9. Allergy reduces the risk of meningioma: a meta-analysis

    PubMed Central

    Wang, Peng-fei; Ji, Wen-Jun; Zhang, Xiao-hui; Li, Shou-wei; Yan, Chang-Xiang

    2017-01-01

    Meningiomas are the most common brain tumours; however, little is known regarding their aetiology. The data are inconsistent concerning atopic disease and the risk of developing meningioma. Thus, we conducted a meta-analysis to investigate the association between allergic conditions and the risk of developing meningioma. A systematic literature search was conducted using PubMed and Web of SCI from Jan 1979 to Feb 2016. Two investigators independently selected the relevant articles according to the inclusion criteria. Eight case-control studies and 2 cohort studies were included in the final analysis, comprising 5,679 meningioma cases and 55,621 control subjects. Compared with no history of allergy, the pooled odds ratio (OR) for allergic conditions was 0.81 (0.70–0.94) for meningioma in a random-effects meta-analysis. Inverse correlations of meningioma occurrence were also identified for asthma and eczema, in which the pooled ORs were 0.78 (0.70–0.86) and 0.78 (0.69–0.87), respectively. A reduced risk of meningioma occurrence was identified in hay fever; however, the association was weak (0.88, 95% CI = 0.78–0.99). The source of this heterogeneity could be the various confounding variables in individual studies. Overall, the current meta-analysis indicated that allergy reduced the risk of developing meningiomas. Large cohort studies are required to investigate this relationship. PMID:28071746

  10. Metastatic meningioma in the sacrum: a case report.

    PubMed

    Lee, Yan-Yaw; Wen-Wei Hsu, Robert; Huang, Tsung-Jen; Hsueh, Swei; Wang, Jeng-Yi

    2002-02-15

    This report describes a 51-year-old woman with a sacral metastatic meningioma that originated from an intracranial meningioma. To describe an unusual presentation of a metastatic meningioma in the sacrum. Extracranial metastases of meningioma are very rare. The phenomenon of metastasis may have more to do with the ability to invade the wall of a blood vessel than with the mitotic activity of a tumor. Therefore, metastases of the meningioma can occur even with a benign histologic picture in the original intracranial meningioma. A 51-year-old woman had experienced low back pain and sciatica of the left leg for several months. Plain radiographs of the lumbosacral spine showed an osteolytic lesion with an irregular margin that occupied the left side of the sacrum. Magnetic resonance imaging revealed a soft mass invading the left sacrum, ilium, and presacral space. Surgical removal of the sacral tumor via an anterior-posterior-anterior approach was done. Histopathologic examination revealed a metastatic meningioma with a meningotheliomatous histologic composition. Sixteen months after excision of the metastatic sacral lesion, the patient was ambulating freely and experiencing mild constipation and urine retention. In this case of metastatic meningioma in the sacrum, which is the first such report to the authors' best knowledge, total excision of the tumor was successful.

  11. Exophytic intramedullary meningioma of the cervical spinal cord.

    PubMed

    Sahni, D; Harrop, J S; Kalfas, I H; Vaccaro, A R; Weingarten, D

    2008-10-01

    Intramedullary spinal cord neoplasms are relatively uncommon. The most common intramedullary tumors are astrocytomas and ependymomas. Meningiomas can occur as an intradural tumor; however, they are typically in the extramedullary compartment. A 42-year-old male presented with progressive sensory loss in the upper extremities and lower extremity weakness. Pre-operative imaging suggested an intramedullary cervical lesion. To treat the progressive neurological abnormality, surgical resection was planned. At surgery, it was noted that the tumor originated in the cervical spinal cord and extended into the extramedullary region. Histology confirmed the lesion to be a meningioma. This meningioma variant has not previously been described. Spinal meningiomas may occur in locations other than intradural, extramedullary locations, and should be included in the differential diagnosis of intramedullary lesions. Intramedullary meningiomas can be successfully treated with surgery.

  12. Fast-Growing Meningioma in a Woman Undergoing Fertility Treatments

    PubMed Central

    Elashaal, Abdurrahim

    2016-01-01

    Meningiomas have long been known to be associated with sexual hormones. We discuss here the case of a woman with a huge meningioma that rapidly grew over the course of a couple years while the patient was simultaneously taking fertility treatments. There is substantial evidence suggesting that fertility treatments can fuel the growth of meningiomas. The potential risks should be considered in women with a previous or family history of meningiomas who plan to undergo fertility treatment. These patients need to be evaluated and a screening imaging of brain MRI (Magnetic Resonant Imaging) should be offered in the middle or toward the end of such a treatment to control and prevent complications of these meningiomas. PMID:28116188

  13. Comparative Oncogenomics for Peripheral Nerve Sheath Cancer Gene Discovery

    DTIC Science & Technology

    2015-06-01

    transcriptional control (Ncoa3, Nr4a3, Zscan22), vesicular trafficking (Napa) and cell motility (Sept6, Vcl). It was also notable that some small CNVs that...metastasis; regulates lysosomal trafficking Nr4a3 (nuclear receptor subfamily 4, group A, member 3) Ligand-dependent transcription factor Aldob...protein attachment protein alpha) Trans-Golgi vesicle trafficking Bbc3 (Bcl binding component 3; PUMA) p53-regulated inducer of apoptosis Mark4

  14. Morphology of human intracardiac nerves: an electron microscope study

    PubMed Central

    PAUZIENE, NERINGA; PAUZA, DAINIUS H.; STROPUS, RIMVYDAS

    2000-01-01

    Since many human heart diseases involve both the intrinsic cardiac neurons and nerves, their detailed normal ultrastructure was examined in material from autopsy cases without cardiac complications obtained no more than 8 h after death. Many intracardiac nerves were covered by epineurium, the thickness of which was related to nerve diameter. The perineurial sheath varied from nerve to nerve and, depending on nerve diameter, contained up to 12 layers of perineurial cells. The sheaths of the intracardiac nerves therefore become progressively attenuated during their course in the heart. The intraneural capillaries of the human heart differ from those in animals in possessing an increased number of endothelial cells. A proportion of the intraneural capillaries were fenestrated. The number of unmyelinated axons within unmyelinated nerve fibres was related to nerve diameter, thin cardiac nerves possessing fewer axons. The most distinctive feature was the presence of stacks of laminated Schwann cell processes unassociated with axons that were more frequent in older subjects. Most unmyelinated and myelinated nerve fibres showed normal ultrastructure, although a number of profiles displayed a variety of different axoplasmic contents. Collectively, the data provide baseline information on the normal structure of intracardiac nerves in healthy humans which may be useful for assessing the degree of nerve damage both in autonomic and sensory neuropathies in the human heart. PMID:11117629

  15. Aluminum Wire Rope Polyethylene Sheathing Failure.

    DTIC Science & Technology

    1997-12-01

    of polyethylene taken from sheathed wire rope samples with and without cracking . Sample Melting Point (°C) Heat of Fusion (J/g) Percent...The cracked polyethylene sheathing was made from a higher density PE than the uncracked sheathing. It failed when it was wound onto a cable reel...12, 512 (1979). 6. A. Lustiger, ’ Environmental Stress Cracking : The Phenomenon and its Utility’, Chapter 16 in Failure of Plastics, edited by W

  16. Endoscopic-Assisted Posterior Intradural Petrous Apicectomy in Petroclival Meningiomas: A Clinical Series and Assessment of Perioperative Morbidity.

    PubMed

    Tatagiba, Marcos; Rigante, Luigi; Mesquita Filho, Paulo; Ebner, Florian H; Roser, Florian

    2015-12-01

    To describe the clinical feasibility and outcome of the endoscopic-assisted posterior intradural petrous apicectomy approach (PIPA) for petroclival meningiomas extending into the supratentorial space. From 2005-2013, 29 patients with a petroclival meningioma underwent tumor removal through a PIPA approach. The approach consists of a retrosigmoid approach, intradural anterior resection of the petrous apex and microsurgical removal of the tumor, followed by endoscopic-assisted visualization and removal of tumor parts in the middle fossa or anterior to the brainstem. Patients consisted of 7 men and 22 women; the mean age of patients was 52.7 years. In 24 patients, surgery was performed with the patient in a semisitting position; in 5 patients, surgery was performed with the patient in a supine position. A total resection was achieved in 19 patients (66%). A Karnofsky performance scale score >60% was recorded in 27 patients (93%), with surgical complications that involved a cerebrospinal fluid leak in 3 patients, bleeding in the surgical cavity in 2 patients, and pneumocephalus in 1 patient. The most frequent postoperative neurologic deficit was facial palsy (34%), which disappeared or improved consistently in all but 1 patient, who required a cranial nerve VII-cranial nerve XII anastomosis. For petroclival meningiomas extending into the middle fossa, the endoscopic-assisted PIPA approach is safe and straightforward. The principal advantages of the PIPA approach are familiarity with the retrosigmoid route; the absence of temporal lobe retraction; and early control of the cranial nerves, vessels, and brainstem. However, careful patient selection regarding tumor extension is fundamental to obtaining optimal outcomes. Copyright © 2015 Elsevier Inc. All rights reserved.

  17. Vertebral Artery Transposition Via an Extreme-Lateral Approach for Anterior Foramen Magnum Meningioma or Craniocervical Junction Tumors.

    PubMed

    Park, Hun Ho; Lee, Kyu-Sung; Hong, Chang-Ki

    2016-04-01

    Vertebral artery (VA) transposition in the extreme-lateral transcondylar approach can minimize the manipulation of the low cranial nerves and the brain stem. The authors describe the surgical technique of VA transposition. From March 2000 to December 2014, 28 of 48 patients underwent VA transposition for anterior foramen magnum meningioma (16 patients) and craniocervical junction (CCJ) tumors (12 patients). Tumor was resected via an extreme-lateral approach with partial condylectomy to expose the anterior portion of the brain stem. For intradural tumors, the VA was mobilized caudomedially after circumferential dural incision around the VA at the level of the foramen magnum. For extradural tumors involving the CCJ, VA was transposed medially from the transverse foramen of C1 without any dural incision. Gross total resection was achieved in 26 of 28 patients (92.9%) with VA transposition. Histologically, meningioma and schwannoma were most common. The origin of the tumors was foramen magnum (57.1%), C1 nerve root (17.9%), clivus (10.7%), jugular foramen (7.1%), posterior skull base (3.6%), and hypoglossal canal (3.6%). VA transposition was performed intradurally in 19 patients (67.9%) and extradurally in 9 patients (32.1%). Surgical morbidity was 17.9% including 4 patients with hypoglossal nerve palsy and 1 patient with quadriparesis. The mean follow-up duration after surgery was 4.2 years (range, 0.1-14.8 years). VA transposition can provide a wide surgical window for anterior foramen magnum meningioma or tumors involving the CCJ with the least manipulation of the neuraxis. Copyright © 2016 Elsevier Inc. All rights reserved.

  18. Olfactory groove meningioma: discussion of clinical presentation and surgical outcomes following excision via the subcranial approach.

    PubMed

    Pepper, Jon-Paul; Hecht, Sarah L; Gebarski, Stephen S; Lin, Erin M; Sullivan, Stephen E; Marentette, Lawrence J

    2011-11-01

    To describe surgical outcomes and radiographic features of olfactory groove meningiomas treated by excision through the subcranial approach. Special emphasis is placed on paranasal sinus and orbit involvement. Retrospective review of a series of patients. Nineteen patients underwent excision of olfactory groove meningioma (OGM) via the transglabellar/subcranial approach between December 1995 and November 2009. Nine patients had previously undergone prior resection at outside institutions, and four had prior radiotherapy in addition to a prior excision. Transglabellar/subcranial surgical approach to the anterior skull base was performed. Tumor histology included three World Health Organization (WHO) grade III lesions, one WHO grade II lesion, and 15 WHO grade I lesions. Fourteen patients had evidence of extension into the paranasal sinuses, with the ethmoid sinus being most commonly involved. Kaplan-Meier estimates of mean overall and disease-free survival were 121.45 months and 93.03 months, respectively. The mean follow-up interval was 41.0 months, and at the time of data analysis three patients had recurrent tumors. Seven (36.8%) patients experienced a major complication in the perioperative period; there were no perioperative mortalities. Orbit invasion was observed in four patients, with optic nerve impingement in 11 patients. Of these, three patients had long-term diplopia. No patients experienced worsening of preoperative visual acuity. Olfactory groove meningiomas demonstrate a propensity to spread into the paranasal sinuses, particularly in recurrent cases. Given a tendency for infiltrative recurrence along the skull base, this disease represents an important area of collaboration between neurosurgery and otolaryngology. The subcranial approach offers excellent surgical access for excision, particularly for recurrences that involve the paranasal sinuses and optic apparatus. Copyright © 2011 The American Laryngological, Rhinological, and Otological Society

  19. Genomic and Expression Profiling of Benign and Malignant Nerve Sheath Profiling of Benign and Malignant Nerve Sheath

    DTIC Science & Technology

    2007-05-01

    Expression of epidermal growth factor receptor, ERBB2 and KIT in adult soft tissue sarcomas: a clinicopathologic study of 281 cases. Cancer 103: 1881-90...TERMS miRNA microarrays, expression profiling, tissue microarrays Immunohistochemistry 16. SECURITY CLASSIFICATION OF: 17. LIMITATION OF...features of the transition of NF to MPNST (see 3rd annual report). We hypothesize that microRNA ( miRNA ) expression differences, as yet unstudied in

  20. Ureteral access strategies: pro-access sheath.

    PubMed

    Vanlangendonck, Richard; Landman, Jaime

    2004-02-01

    Routine use of the ureteral access sheath during flexible ureteroscopic procedures provides consistent, reliable, and unencumbered access to the upper tracts. The ureteral access sheath can be reliably and easily deployed if used properly and requires no special training. As such, it can be easily adopted into current urologic practice. Not only does the access sheath facilitate rapid, repeated, and atraumatic access to the upper tracts, but it also avoids back-loading over a superstiff guidewire, which may incur costly damage to the ureteroscope. The access sheath reduces overall costs and decreases operative times. Furthermore, application of a ureteral access sheath reduces the frustration associated with complex and some routine ureteroscopic procedures by optimizing irrigant flow to improve the surgeon's endoscopic vision while minimizing the intrarenal pressures that the kidney must tolerate. There is no evidence that the access sheath results in clinically significant ureteral ischemia, and extensive clinical use of the access sheath for long procedures has not resulted in increased stricture formation. Finally, the ureteral access sheath is useful for other procedures, such as PCNL, by improving visualization and minimizing the requirement for multiple percutaneous access sites. Winston Churchill said it best: "Give us the tools and we will finish the job." Industry has provided urologists with the tools in the form of advanced flexible ureteroscopes, the holmium laser, nitinol baskets, and the ureteral access sheath. Now it is up to urologists to finish the job.

  1. Transient sheath overvoltages in armored power cables

    SciTech Connect

    Gustavsen, B.; Sletbak, J.

    1996-07-01

    This paper is concerned with methods of limiting the build-up of transient voltages between sheath and armor in long armored power cables. Calculations by a frequency dependent cable model demonstrate that this voltage can be efficiently limited to an acceptable level by introducing sheath-armor bondings at regular intervals, or by using a semiconductive sheath-armor interlayer. The paper investigates the required minimum length between bondings, as well as the required conductivity of the sheath-armor interlayer if the use of bondings is to be avoided.

  2. Gold ink coating of thermocouple sheaths

    DOEpatents

    Ruhl, H. Kenneth

    1992-01-01

    A method is provided for applying a gold ink coating to a thermocouple sheath which inclu