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Sample records for nerve sheath meningioma

  1. 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. PMID:27310300

  2. Optic Nerve Sheath Meningioma Masquerading as Optic Neuritis.

    PubMed

    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

  3. 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

  4. 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.

  5. 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.

  6. 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.

  7. Radiation therapy for primary optic nerve meningiomas

    SciTech Connect

    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.

  8. 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.

  9. Malignant Peripheral Nerve Sheath Tumor.

    PubMed

    James, Aaron W; Shurell, Elizabeth; Singh, Arun; Dry, Sarah M; Eilber, Fritz C

    2016-10-01

    Malignant peripheral nerve sheath tumor (MPNST) is the sixth most common type of soft tissue sarcoma. Most MPNSTs arise in association with a peripheral nerve or preexisting neurofibroma. Neurofibromatosis type is the most important risk factor for MPNST. Tumor size and fludeoxyglucose F 18 avidity are among the most helpful parameters to distinguish MPNST from a benign peripheral nerve sheath tumor. The histopathologic diagnosis is predominantly a diagnosis of light microscopy. Immunohistochemical stains are most helpful to distinguish high-grade MPNST from its histologic mimics. Current surgical management of high-grade MPNST is similar to that of other high-grade soft tissue sarcomas. PMID:27591499

  10. 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. PMID:27165368

  11. 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.

  12. Massive exophytic malignant peripheral nerve sheath tumor.

    PubMed

    Khorsand, Derek; Porrino, Jack; Flaherty, Erin; Bandhlish, Anshu; Davidson, Darin

    2016-06-01

    We present a case of a solitary neurofibroma involving the right posterior shoulder of a 69-year-old man with degeneration into a massive, malignant peripheral nerve sheath tumor measuring more than 3 times the average reported size. The radiographic, magnetic resonance imaging, and computed tomographic features are compared with the gross appearance and pathology. PMID:27257459

  13. Remote acute demyelination after focal proton radiation therapy for optic nerve meningioma.

    PubMed

    Redjal, Navid; Agarwalla, Pankaj K; Dietrich, Jorg; Dinevski, Nikolaj; Stemmer-Rachamimov, Anat; Nahed, Brian V; Loeffler, Jay S

    2015-08-01

    We present a unique patient with delayed onset, acute demyelination that occurred distant to the effective field of radiation after proton beam radiotherapy for an optic nerve sheath meningioma. The use of stereotactic radiotherapy as an effective treatment modality for some brain tumors is increasing, given technological advances which allow for improved targeting precision. Proton beam radiotherapy improves the precision further by reducing unnecessary radiation to surrounding tissues. A 42-year-old woman was diagnosed with an optic nerve sheath meningioma after initially presenting with vision loss. After biopsy of the lesion to establish diagnosis, the patient underwent stereotactic proton beam radiotherapy to a small area localized to the tumor. Subsequently, the patient developed a large enhancing mass-like lesion with edema in a region outside of the effective radiation field in the ipsilateral frontal lobe. Given imaging features suggestive of possible primary malignant brain tumor, biopsy of this new lesion was performed and revealed an acute demyelinating process. This patient illustrates the importance of considering delayed onset acute demyelination in the differential diagnosis of enhancing lesions in patients previously treated with radiation. PMID:25937571

  14. 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. PMID:26023558

  15. 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.

  16. Optic Nerve Sheath Melanoma Presenting as a Central Retinal Vein Occlusion.

    PubMed

    Barash, Alexander; Sibony, Patrick A; Stippa, Nigel A; Boyle, Nariman S; Davis, James E

    2016-03-01

    A 64-year-old woman, with a history of diabetes and melanoma, developed a central retinal vein occlusion (CRVO) in her left eye. On exam, she had severe disc edema with retinal nerve fiber layer thickening, and anterior deformation of the peripapillary retinal pigment epithelium (RPE)/Bruch membrane layer (ppRPE/BM) toward the vitreous on spectral domain optical coherence tomography (SD-OCT) suggesting an optic nerve sheath (ONS) meningioma. Magnetic resonance imaging findings and ONS biopsy later confirmed a metastatic melanoma. This case demonstrates that the shape of the RPE/BM on SD-OCT may aid in the decision to consider imaging in patients with isolated CRVO. PMID:26535468

  17. Malignant Peripheral Nerve Sheath Tumor of the Infraorbital Nerve.

    PubMed

    D'Addino, José Luis; Piccoletti, Laura; Pigni, María Mercedes; de Gordon, Maria José Rodriguez Arenas

    2016-06-01

    The objective of this study is to report a large, rare, and ulcerative infiltrated skin lesion. Its diagnosis, therapeutic management, and progress are described. The patient is a 78-year-old white man, who presented with a 12-month ulcerative perforated lesion that had affected and infiltrated the skin, with easy bleeding. He had a history of hypertension, although controlled, was a 40-year smoker, had chronic atrial fibrillation, diabetes, and microangiopathy. During the consultation, the patient also presented with ocular obstruction due to an inability to open the eye. He mentioned having reduced vision. The computed tomography scan showed upper maxilla osteolysis without eye involvement. We underwent a radical resection in which upper maxilla and the anterior orbital margin were included. We used a Becker-type flap that allowed us to rebuild the cheek and to complete a modified neck dissection. Progress was favorable; the patient recovered ocular motility and his vision improved to 20/200. The final biopsy result was "malignant peripheral nerve sheath tumor, malignant schwannoma." Malignant schwannoma of the peripheral nerve is extremely rare. The total resection and reconstruction being completed in one surgery represented a challenge due to the difficulty in obtaining tissues in addition to the necessity of an oncological resection. PMID:27162577

  18. Malignant peripheral nerve sheath tumor of the parotid gland.

    PubMed

    Chis, Octavian; Albu, Silviu

    2014-09-01

    Malignant peripheral nerve sheath tumor (MPNST) refers to spindle cell sarcomas arising from or separating in the direction of cells of the peripheral nerve sheath. The MPNST of the parotid gland is an extremely rare tumor, usually having a poor prognosis, and only a few cases been described in the literature. In this article, we report the diagnostic and therapeutic challenges related to a new case of MPNST of the parotid. Diagnosis was made based on clinical, imaging (computed tomography scan), histologic, and immunohistochemistry findings. Despite comprehensive treatment--complete surgical resection and radiotherapy--the tumor displayed a highly aggressive course. PMID:25153067

  19. 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

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

    PubMed

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

    2016-06-01

    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

  1. 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. PMID:26928128

  2. 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

  3. 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. PMID:25705535

  4. Malignant Peripheral Nerve Sheath Tumour: CT and MRI Findings.

    PubMed

    Sperandio, Massimiliano; Di Poce, Isabelle; Ricci, Aurora; Di Trapano, Roberta; Costanzo, Elisa; Di Cello, Pierfrancesco; Pelle, Fabio; Izzo, Luciano; Simonetti, Giovanni

    2013-01-01

    Malignant peripheral nerve sheath tumour (MPNST) is extremely rare malignancy in the general population, occurring more frequently in patients with Neurofibromatosis type 1 (NF1). In the literature five cases of MPNST arising from the parapharyngeal space (PPS) in patients without neurofibromatosis have been reported. We report imaging techniques in a patient with MPNST in the PPS, who had neither a family history nor sign of NF1. Computed tomography (CT) scan and magnetic resonance imaging (MRI) were performed for a correct therapeutic planning. CT and MRI findings were correlated with hystopathological diagnosis. PMID:23970990

  5. 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. PMID:27338175

  6. Excellent response of malignant peripheral nerve sheath tumour of retroperitoneum to radiation therapy

    PubMed Central

    Akhavan, Ali; Binesh, Fariba; Ghannadi, Fazlollah; Navabii, Hossein

    2012-01-01

    Malignant peripheral nerve sheath tumours are high-grade sarcomas originating from Schwann cells or nerve sheath cells. Most of these tumours are associated with major nerves of the body wall and extremities. The lower extremity and the retroperitoneum are the most common sites. Surgery is the cornerstone of treatment, however, radiation therapy is usually used as an adjuvant treatment. In this paper we present a 57-year-old Iranian woman with malignant peripheral nerve sheath tumour of retroperitoneum who was operated subtotally and then underwent radiation therapy which led to disappearance of all gross residual disease. PMID:23257269

  7. 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

    2015-01-01

    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. PMID:26407677

  8. Conventional and Functional MR Imaging of Peripheral Nerve Sheath Tumors: Initial Experience

    PubMed Central

    Demehri, S.; Belzberg, A.; Blakeley, J.; Fayad, L.M.

    2015-01-01

    BACKGROUND AND PURPOSE Differentiating benign from malignant peripheral nerve sheath tumors can be very challenging using conventional MR imaging. Our aim was to test the hypothesis that conventional and functional MR imaging can accurately diagnose malignancy in patients with indeterminate peripheral nerve sheath tumors. MATERIALS AND METHODS This institutional review board–approved, Health Insurance Portability and Accountability Act–compliant study retrospectively reviewed 61 consecutive patients with 80 indeterminate peripheral nerve sheath tumors. Of these, 31 histologically proved peripheral nerve sheath tumors imaged with conventional (unenhanced T1, fluid-sensitive, contrast-enhanced T1-weighted sequences) and functional MR imaging (DWI/apparent diffusion coefficient mapping, dynamic contrast-enhanced MR imaging) were included. Two observers independently assessed anatomic (size, morphology, signal) and functional (ADC values, early arterial enhancement by dynamic contrast-enhanced MR) features to determine interobserver agreement. The accuracy of MR imaging for differentiating malignant from benign was also determined by receiver operating characteristic analysis. RESULTS Of 31 peripheral nerve sheath tumors, there were 9 malignant (9%) and 22 benign ones (81%). With anatomic sequences, average tumor diameter (6.3 ± 1.8 versus 3.9 ± 2.3 mm, P = .009), ill-defined/infiltrative margins (77% versus 32%; P = .04), and the presence of peritumoral edema (66% versus 23%, P = .01) were different for malignant peripheral nerve sheath tumors and benign peripheral nerve sheath tumors. With functional sequences, minimum ADC (0.47 ± 0.32 × 10−3 mm2/s versus 1.08 ± 0.26 × 10−3 mm2/s; P [H11021] .0001) and the presence of early arterial enhancement (50% versus 11%; P = .03) were different for malignant peripheral nerve sheath tumors and benign peripheral nerve sheath tumors. The minimum ADC (area under receiver operating characteristic curve was 0.89; 95

  9. Sciatica due to malignant nerve sheath tumour of sciatic nerve in the thigh.

    PubMed

    Sharma, R R; Pawar, S J; Mahapatra, A K; Doctor, M; Musa, M M

    2001-06-01

    Malignant peripheral nerve sheath tumour (MPNST) is a rare malignant neoplasm arising from the supportive non-neural component of the peripheral nerves. An unusual case of pain and weakness of the foot and calf muscles due to a giant MPNST of the sciatic nerve in the posterior compartment of the thigh is presented. The patient was already investigated as a case of sciatica due to a lumbar disc disease with a negative magnetic resonance imaging and then unsuccessfully operated elsewhere twice, with a misdiagnosis of tarsal tunnel syndrome. Neurosurgical referral prompted a diagnostic magnetic resonance study of the thigh, revealing the lesion, which was completely excised microsurgically with total relief in the pain and partial improvement in the weakness and sensations in the sole of the foot. PMID:11447444

  10. 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

  11. 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

  12. 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

  13. Benign Nerve Sheath Myxoma in an Infant Misdiagnosed as Infantile Digital Fibromatosis.

    PubMed

    Güngör, Şule; Şişman, Servet; Kocaturk, Emek; Oguz Topal, Ilteris; Yıldırım, Selda

    2016-07-01

    Herein we present the case of a 16-month boy, clinically diagnosed with infantile digital fibromatosis, but 9 months after continued growth, the mass was excised and the histopathologic diagnosis was that of a benign nerve sheath myxoma. We present this case to emphasize that nerve sheath myxomas (also known as myxoid neurothekeoma) should be included in the differential diagnosis of dermal nodules in infants. PMID:27196676

  14. [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. PMID:26934901

  15. Optical coherence tomography imaging of the optic nerve head pre optic and post optic nerve sheath fenestration.

    PubMed

    Chowdhury, Haziq Raees; Rajak, Saul; Heath, Dominic; Brittain, Paul

    2015-01-01

    Idiopathic intracranial hypertension (IIH) is a disorder of unknown aetiology, and causes elevated intracranial pressures. This is often associated with papilloedema with subsequent degrees of optic atrophy if the intracranial pressures are not controlled in a timely manner. Optical coherence tomography imaging is widely recognised for its use in the monitoring of optic nerves in glaucoma, and this report is the first to describe its use to monitor the optic nerve head pre optic and post optic nerve sheath fenestration. PMID:26150609

  16. Malignant Nerve Sheath Tumor of the Spinal Accessory Nerve: A Unique Presentation of a Rare Tumor

    PubMed Central

    Sheikh, Omair A.; Reaves, Ann; Kralick, Francis A.; Brooks, Ari; Musial, Rachel E.

    2012-01-01

    Background Malignant peripheral nerve sheath tumors (MPNSTs), sarcomas originating from tissues of mesenchymal origin, are rare in patients without a history of neurofibromatosis. Case Report We report a case of an MPNST of the spinal accessory nerve, unassociated with neurofibromatosis, which metastasized to the brain. The tumor, originating in the intrasternomastoid segment of the spinal accessory nerve, was removed. Two years later, the patient presented with focal neurological deficits. Radiographic findings revealed a well-defined 2.2×2.2×2.2 cm, homogeneously enhancing mass in the left parieto-occipital region of the brain surrounded by significant vasogenic edema and mass effect, culminating in a 1-cm midline shift to the right. The mass was surgically removed. The patient had nearly complete recovery of vision, speech, and memory. Conclusions To our knowledge, this is the first documented case of an MPNST arising from an extracranial segment of the spinal accessory nerve and metastasizing to the brain. PMID:22523517

  17. Genetic Profiling by Single-Nucleotide Polymorphism-Based Array Analysis Defines Three Distinct Subtypes of Orbital Meningioma

    PubMed Central

    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.

    2015-01-01

    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. PMID:24773246

  18. Pathology in practice: Peripheral nerve sheath tumor in a Shubunkin goldfish

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Peripheral nerve sheath tumors (PNSTs) have been detected in many fish species, including goldfish, several species of snapper, coho salmon, the bicolor damselfish, and rainbow smelt. They originate from neural crest cells and generally occur along the subcutaneous nerves. A viral etiology has bee...

  19. Effect of scuba-diving on optic nerve and sheath diameters

    PubMed Central

    Mehrpour, Masoud; Shams-Hosseini, Narges Sadat; Rezaali, Saeed

    2014-01-01

    Background: There is not any data available about the effect of high bar pressure condition on intracranial pressure. In this study, the effect of diving on the optic nerve and sheath diameters as non-invasive markers of intracranial pressure has been investigated. Methods: Twenty professional male divers from twenty one volunteers were chosen for this cross-sectional study. Only one person who had a history of barotraumas was excluded. Each diver then completed a questionnaire on demographic data, medical and diving history. Nineteen other volunteers were selected to represent a control group. A 10-MHz linear ultrasonic probe was used to measure the optic nerve sheath diameters of both eyes in closed and supine position and its relationship with diving history of divers was determined. Results: It was found that divers have a higher mean optic nerve sheath diameter compared to the normal population as previously reported by other studies. The mean diameter of the left and right optic nerve sheaths were 6.4±0.7, 6.5±0.9 mm respectively and a significant relationship between optic nerve sheath diameter and diving history was found. Conclusion: Results showed that divers have a higher optic nerve diameter than the general population. However, our result cannot yet be considered as a marker of intracranial pressure in divers as it was conducted on an limited number of subjects and so a bigger study should be undertaken for this purpose. PMID:25664290

  20. Malignant Peripheral Nerve Sheath Tumor in Neurofibromatosis Type I : Unusual Presentation of Intraabdominal or Intrathoracic Mass

    PubMed Central

    Kim, Jong Gwang; Sung, Woo Jin; Kim, Dong Hwan; Kim, Young Hwan; Lee, Kyu Bo

    2005-01-01

    A malignant peripheral nerve sheath tumor (MPNST) is an extremely rare soft tissue tumor in the general population. On the other hand, there is a higher incidence of MPNST in patients with neurofibromatosis type I (von Recklinghausen's disease). The common sites are the extremities, trunk, head and neck. However, an intraabdominal or intrathoracic manifestation is uncommon. This paper reports two patients, a 31 year-old woman with multiple neurofibromatosis presenting as an intraabdominal malignant peripheral nerve sheath tumor, and a 33 year-old woman with an intrathoracic malignant peripheral nerve sheath tumor. The patients were treated with chemotherapy followed by radiotherapy. However, one patient died as a result of disease progression 21 months after the diagnosis and the other patient is currently being treated with radiotherapy. PMID:15906964

  1. A Case of Malignant Peripheral Nerve Sheath Tumor of the Hypoglossal Nerve after Stereotactic Radiosurgery Treatment

    PubMed Central

    Yang, Tong; Juric-Sekhar, Gordana; Born, Donald; Sekhar, Laligam N.

    2014-01-01

    Objectives Hypoglossal schwannomas are rare. Surgical resection has been the standard treatment modality. Radiosurgery has been increasingly used for treatment. Radiation-associated secondary malignancy/malignant transformation has not been documented in the literature for the treatment of nonvestibular schwannomas. Setting The patient was a 52-year-old man with an enlarging high cervical/skull base lesion 8.5 years after CyberKnife treatment of a presumed vagal schwannoma. A decision was made for surgical resection, and the tumor was found to originate from the hypoglossal nerve intraoperatively. Final pathology diagnosis was malignant peripheral nerve sheath tumor. Results Patient had a gross total resection. Three months after resection, he received fractionated radiation of 50 Gy in 25 fractions and a boost gamma knife radiosurgery of 10 Gy to the 50% isodose surface. He remained tumor free on repeat magnetic resonance imaging 9 months after the resection. Conclusion Although extremely rare, radiation treatment of nonvestibular schwannomas can potentially cause malignant transformation. PMID:25083387

  2. The coexistence of peripheral nerve sheath tumors and vitiligo: more than coincidence?

    PubMed

    Elsherif, Mohamed A; Spinner, Robert J; Miest, Rachel Y

    2016-01-01

    Neurocristopathies arise from abnormal migration, differentiation, or proliferation of neural crest derivatives, leading to diverse clinical and pathological features. They are classified into dysgenetic or neoplastic, and can affect single or multiple sites (simple versus complex). Examples include congenital melanocytic nevi, neuroblastoma, Hirshsprung's disease, Waardenburg's syndrome, neurofibromatosis (NF) 1 and multiple endocrine neoplasia (MEN) 2A and 2B. We report two cases of peripheral nerve sheath tumors associated with vitiligo and discuss the possible implicated embryologic, genetic and molecular mechanisms. To our knowledge, we also report the first case of de novo malignant peripheral nerve sheath tumor (MPNST) associated with vitiligo. PMID:26607956

  3. Loss of H3K27 trimethylation distinguishes malignant peripheral nerve sheath tumors from histologic mimics.

    PubMed

    Schaefer, Inga-Marie; Fletcher, Christopher Dm; Hornick, Jason L

    2016-01-01

    The diagnosis of malignant peripheral nerve sheath tumor is challenging, particularly in the sporadic setting. Inactivation of the polycomb repressive complex 2 (PRC2), resulting from inactivating mutations of its constituents SUZ12 or EED1, has recently been identified in 70-90% of malignant peripheral nerve sheath tumors. Homozygous PRC2 inactivation results in loss of histone H3K27 trimethylation (H3K27me3). PRC2 inactivation promotes tumor progression and may render patients sensitive to epigenetic-based targeted therapies. H3K27me3 loss has not yet been validated as a diagnostic marker. We evaluated immunohistochemistry for H3K27me3 in 100 malignant peripheral nerve sheath tumors (70 sporadic, 10 neurofibromatosis type 1-associated, 10 radiation-associated, 10 epithelioid) and 200 other spindle cell neoplasms representing potential mimics (20 each monophasic synovial sarcoma, leiomyosarcoma, dedifferentiated liposarcoma, malignant solitary fibrous tumor, low-grade fibromyxoid sarcoma, cellular schwannoma, spindle cell melanoma, unclassified postradiation sarcoma; 10 each atypical neurofibroma, spindle cell rhabdomyosarcoma, gastrointestinal stromal tumor, fibrosarcomatous dermatofibrosarcoma protuberans). In total, 51 (51%) malignant peripheral nerve sheath tumors, including 34 (49%) sporadic, 7 (70%) neurofibromatosis type 1-associated, and 10 (100%) radiation-associated, but no epithelioid malignant peripheral nerve sheath tumors, were negative for H3K27me3. An additional 6 (6%) tumors showed heterogeneous H3K27me3 expression. Among the 90 sporadic, neurofibromatosis type 1-associated, and radiation-associated malignant peripheral nerve sheath tumors, complete H3K27me3 loss was observed in 29% of low-grade, 59% of intermediate-grade, and 83% of high-grade tumors (low vs intermediate/high grade, P=0.0003). Among other tumor types, 4 (20%) unclassified postradiation sarcomas were negative for H3K27me3, whereas all other neoplasms were positive. Loss of H3K27me

  4. Magnetic resonance imaging of the optic nerves and chiasm

    SciTech Connect

    Daniels, D.L.; Herfkins, R.; Gager, W.E.; Meyer, G.A.; Koehler, P.R.; Williams, A.L.; Haughton, V.M.

    1984-07-01

    Magnetic resonance imaging (MR) of the optic nerves and chiasm was compared with computed tomography (CT) in 4 healthy volunteers, 4 patients without orbital or chiasmal abnormalities, and 4 patients with tumor (anterior clinoid meningioma in 2, optic nerve glioma in 1, and optic nerve sheath meningioma in 1). MR was found to be effective in demonstrating the optic nerves and related structures, particularly the intracanalicular portion of the nerve which is difficult to see with CT. Best results were achieved with partial saturation recovery (SR) images. As axial views cannot always distinguish the ethmoid sinus tissue from the optic nerve, it may be necessary to employ both axial and coronal images.

  5. 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

  6. Acute optic nerve sheath fenestration with the free-electron laser

    NASA Astrophysics Data System (ADS)

    Shen, Jin-Hui; Casagrande, Vivien A.; Joos, Karen M.; Shetlar, Debra J.; Robinson, Richard D.; Head, William S.; Mavity-Hudson, Julia A.; Nunnally, Amy H.

    1999-06-01

    Purpose: To determine if the free electron laser (FEL) energy can be delivered to a small space to perform optic nerve sheath fenestration with minimal acute nerve damage. Methods: A 530 mm hollow waveguide probe was designed. Optic nerve sheath fenestration (1.0 mm diameter) was performed in 8 rabbits using either the FEL (4 eyes, 6.45mm, 10 Hz, 2 mJ) or a knife (4 eyes). Within 2 hours following surgery, the animals were perfused with aldehyde fixative. The integrity of the optic nerve and glial response at the site of fenestration were evaluated on tissue selections with H&E, and antibodies to S100β or GFAP. Results: Surgery using the FEL probe was found to be technically superior to the knife. The glial reaction was limited to a zone adjacent to the fenestration and was similar in both the FEL and knife incisions. Conclusions: The FEL appears capable of efficiently performing an optic nerve sheath fenestration in a small space with minimal acute damage. Both the FEL and knife incisions result in a rapid glial response at the site of fenestration even when optic nerve integrity is not compromised.

  7. 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

  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. 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

  10. Morphometric analysis of connective tissue sheaths of sural nerve in diabetic and nondiabetic patients.

    PubMed

    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

  11. Pediatric and adult vision restoration after optic nerve sheath decompression for idiopathic intracranial hypertension.

    PubMed

    Bersani, Thomas A; Meeker, Austin R; Sismanis, Dimitrios N; Carruth, Bryant P

    2016-06-01

    To compare presentations of idiopathic intracranial hypertension and efficacy of optic nerve sheath decompression between adult and pediatric patients, a retrospective cohort study was completed All idiopathic intracranial hypertension patients undergoing optic nerve sheath decompression by one surgeon between 1991 and 2012 were included. Pre-operative and post-operative visual fields, visual acuity, color vision, and optic nerve appearance were compared between adult and pediatric (<18 years) populations. Outcome measures included percentage of patients with complications or requiring subsequent interventions. Thirty-one adults (46 eyes) and eleven pediatric patients (18 eyes) underwent optic nerve sheath decompression for vision loss from idiopathic intracranial hypertension. Mean deviation on visual field, visual acuity, color vision, and optic nerve appearance significantly improved across all subjects. Pre-operative mean deviation was significantly worse in children compared to adults (p=0.043); there was no difference in mean deviation post-operatively (p=0.838). Significantly more pediatric eyes (6) presented with light perception only or no light perception than adult eyes (0) (p=0.001). Pre-operative color vision performance in children (19%) was significantly worse than in adults (46%) (p=0.026). Percentage of patients with complications or requiring subsequent interventions did not differ between groups. The consistent improvement after surgery and low rate of complications suggest optic nerve sheath decompression is safe and effective in managing vision loss due to adult and pediatric idiopathic intracranial hypertension. Given the advanced pre-operative visual deficits seen in children, one might consider a higher index of suspicion in diagnosing, and earlier surgical intervention in treating pediatric idiopathic intracranial hypertension. PMID:27163674

  12. Support of Nerve Conduction by Respiring Myelin Sheath: Role of Connexons.

    PubMed

    Ravera, Silvia; Bartolucci, Martina; Adriano, Enrico; Garbati, Patrizia; Ferrando, Sara; Ramoino, Paola; Calzia, Daniela; Morelli, Alessandro; Balestrino, Maurizio; Panfoli, Isabella

    2016-05-01

    Recently, we have demonstrated that myelin conducts an extramitochondrial oxidative phosphorylation, hypothesizing a novel supportive role for myelin in favor of the axon. We have also hypothesized that the ATP produced in myelin could be transferred thought gap junctions. In this work, by biochemical, immunohistochemical, and electrophysiological techniques, the existence of a connection among myelin to the axon was evaluated, to understand how ATP could be transferred from sheath to the axoplasm. Data confirm a functional expression of oxidative phosphorylation in isolated myelin. Moreover, WB and immunohistochemistry on optic nerve slices show that connexins 32 and 43 are present in myelin and colocalize with myelin basic protein. Interestingly, addition of carbenoxolone or oleamide, two gap junction blockers, causes a decrease in oxidative metabolism in purified myelin, but not in mitochondria. Similar effects were observed on conduction speed in hippocampal Schaffer collateral, in the presence of oleamide. Confocal analysis of optic nerve slices showed that lucifer yellow (that only passes through aqueous pores) signal was found in both the sheath layers and the axoplasma. In the presence of oleamide, but not with oleic acid, signal significantly decreased in the sheath and was lost inside the axon. This suggests the existence of a link among myelin and axons. These results, while supporting the idea that ATP aerobically synthesized in myelin sheath could be transferred to the axoplasm through gap junctions, shed new light on the function of the sheath. PMID:26033217

  13. Lectin histochemistry of normal and neoplastic peripheral nerve sheath. 1. Lectin binding pattern of normal peripheral nerve in man.

    PubMed

    Matsumura, K; Nakasu, S; Nioka, H; Handa, J

    1993-01-01

    The binding patterns of lectins to normal peripheral nerves were examined. Twelve biotinylated lectins were used in this study; Canavalia ensiformis (Con A), Pisum sativum (PSA), Lens culinaris (LCA), Ricinus communis 1 (RCA-1), Arachis hypogaea (PNA), Glycine max (SBA), Sophora japonica (SJA), Bandeiraea simplicifolia 1 (BSL-1), Triticum vulgaris (WGA), succinylated WGA (s-WGA), Ulex europaeus 1 (UEA-1) and Helix pomatia (HPA). Cytoplasm of Schwann cells and perineurial cells was stained by Con A, PSA, LCA, s-WGA and WGA. PNA showed specific binding to perineurial cells, while after neuraminidase treatment stain with this lectin was demonstrated also in Schwann cells. Myelin sheaths were stained with fewer lectins. SBA and HPA with sialic acid removal rarely showed reactivity to the peripheral nerve structure in surgical specimens, in contrast to clear staining of Schwann cells, perineurial cells and myelin sheaths in autopsy specimens. The present study shows distinct lectin stainings of specific structures of the normal human peripheral nerves, and provides important basic information on the alterations of lectin binding patterns during pathological processes in the peripheral nerves. PMID:8310810

  14. Cellular neurothekeoma: case report and its (un) relation with nerve sheath myxoma.

    PubMed

    Navarrete-Dechent, Cristián; Curi-Tuma, Maximiliano; Marín, Celeste; González, Sergio; Sandoval-Osses, Mauricio

    2015-01-01

    Neurothekeoma is an uncommon, benign neoplasm presenting in young adults, primarily on the head and neck. It was initially related to nerve sheath myxoma but with the advent of immunohistochemistry, new insights into its cellular differentiation and origin have emerged, unlinking Neurothekeoma and nerve sheath myxoma. Herein we describe a 19-year-old male who had had a frontal, flesh-colored, asymptomatic papule for 2 years. Histology showed a dermal fusocellular-spindle cell tumor, including an eosinophilic cytoplasm with mild cellular pleomorphism and moderately dense fibrous stroma. IHQ was positive for CD10 and negative for S100 and Claudin-1. These findings were compatible with cellular Neurothekeoma. The lesion was completely extirpated and at the 6-month follow-up, the patient was asymptomatic and had experienced no recurrences. PMID:26312702

  15. Malignant peripheral nerve sheath tumor in a cat with nodal and pulmonary metastases.

    PubMed

    Buza, Elizabeth L; Menzies, Robert A; Goldschmidt, Michael H; Durham, Amy C

    2012-07-01

    Peripheral nerve sheath tumors in domestic cats are infrequently reported and are often locally invasive. An 11-year-old Domestic Shorthair cat was originally diagnosed with a right maxillary benign peripheral nerve sheath tumor at incisional biopsy. At necropsy, the neoplasm had features of malignancy including metastases to the regional lymph nodes and lung. Histologically, the neoplasm contained 2 distinct regions: spindle cells arranged in dense interwoven bundles with Antoni A areas and Verocay bodies and Antoni B regions with loosely arranged spindle cells separated by a mucinous matrix. Immunohistochemically, the neoplastic cells in the primary mass and right mandibular lymph node were strongly positive for vimentin, S-100, and glial fibrillar acidic protein. The neoplastic cells within the lung were strongly positive for vimentin and weakly positive for S-100 and glial fibrillar acidic protein. PMID:22604770

  16. Lost in Translation: Ambiguity in Nerve Sheath Tumor Nomenclature and Its Resultant Treatment Effect

    PubMed Central

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

    2013-01-01

    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. PMID:24216989

  17. Ethanol Ablation of a Peripheral Nerve Sheath Tumor Presenting as a Small Bowel Obstruction.

    PubMed

    Chin, Matthew; Chen, Chien-Lin; Chang, Kenneth; Lee, John; Samarasena, Jason

    2015-10-01

    Ethanol has historically been used as an ablative agent for a variety of lesions. One of the more common applications of this technique is celiac plexus neurolysis; however, recent reports have suggested a role for the endoscopic alcohol ablation of a variety of solid and cystic lesions. We report a novel case of endoscopic ethanol ablation of a peripheral nerve sheath tumor presenting as a small bowel obstruction. PMID:26504873

  18. 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

  19. 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. PMID:26278348

  20. Malignant peripheral nerve sheath tumor of the orbit: case report and literature review.

    PubMed

    Aydin, Mehmet D; Yildirim, Umran; Gundogdu, Cemal; Dursun, Osman; Uysal, Hasan H; Ozdikici, Mete

    2004-05-01

    A 68-year-old woman with progressive visual loss and exophthalmos in her right eye had been operated on for a mass in her right calf 3 years earlier. Imaging showed a huge mass invading the orbital structures and temporal pole. The presumptive diagnosis was a malignant orbital tumor. The tumor was resected totally and eroded tissues such as the lateral rectus muscle and dural compartments were repaired. The histological diagnosis was a malignant peripheral nerve sheath tumor (MPNST). The patient recovered uneventfully and was discharged 8 days after surgery. Two years later she died from a liver tumor. Few MPNSTs involving the orbit have been reported. PMID:16145592

  1. [Malignant peripheral sheath nerve tumor: An exceptional mass of the anterior and middle mediastinum].

    PubMed

    Bacha, S; Chaouch, N; Ayadi, A; Zidi, A; Cheikhrouhou, S; Racil, H; Chabbou, A

    2015-12-01

    Malignant peripheral nerve sheath tumors (MPNST) are rare nervous tumors usually located in the posterior mediastinum in the paravertebral gutters. We report the case of a non-smoking 62-year-old man who was admitted with a 4 months history of cough, hoarseness and shortness of breath. Physical examination noted a superior vena cava syndrome. CT scan of the chest revealed a right anterior and middle mediastinal mass compressing the superior vena cava, the ascending aorta, the right pulmonary artery, invading the superior root of the pulmonary vein and the right auricle. Flexible bronchoscopy showed extrinsic compression of the right main bronchus, the right upper lobe bronchus and intermedius bronchus. The patient underwent surgical biopsy of the mass by mediastinoscopy. Histological examination revealed a malignant peripheral nerve sheath tumor. The patient received a single cycle of chemotherapy (ifosfamid-adriamycin). Clinical course was marked by the fast worsening of the dyspnea and the general state. Patient died three weeks after the cure of the chemotherapy. This case is original by the exceptional clinical presentation of MPSNT with a superior vena cava syndrome and the very rare location of this tumor in the anterior and middle mediastinum. PMID:26190334

  2. Peripheral nerve sheath tumors arising in salivary glands: A clinicopathologic study.

    PubMed

    Guraya, Sahejmeet S; Prayson, Richard A

    2016-08-01

    Primary salivary gland peripheral nerve sheath tumors (PNST) are uncommon. This study is a retrospective, clinicopathologic review of 9 cases of PNST (5 neurofibromas, 3 schwannomas and 1 malignant peripheral nerve sheath tumor (MPNST)) arising from the salivary glands, encountered between 1990 and 2015. All patients with neurofibromas were male (ages 1-62 years) and had a single parotid lesion of which 2 were diffuse, 2 plexiform and one mixed diffuse/plexiform. Four had a history of neurofibromatosis I. Four of 5 presented with symptoms related to mass effect including facial swelling, facial drooping, and dysphagia. All underwent de-bulking surgery and recurred due to continued growth. Of the 3 patients with schwannomas, 1 was male and 2 were female (ages 19, 44 and 56 years). One tumor each arose in the sublingual, submandibular, and parotid glands. Two of 3 presented with soreness and swelling local to the affected gland, especially while chewing. There was no recurrence of these tumors after resection. An MPNST in a male presented as a tender mass in the patient's left parotid; the tumor was resected. There was no evidence of tumor elsewhere in the body. The tumor did not recur in 12 years of follow-up. The most common tumor type in the current series was neurofibroma; most arose in the background of neurofibromatosis type I and all of which recurred after initial subtotal resection. Most PNST arose in the parotid gland. PMID:27402223

  3. 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

  4. Meningiomas of the Pediatric Skull Base: A Review

    PubMed Central

    Gump, William C.

    2014-01-01

    Pediatric skull base meningiomas are rare and complex clinical entities. Meningioma is a relatively uncommon brain tumor in children, and only ∼ 27% involve the skull base. Some evidence suggests that these tumors are more likely to be atypical or malignant in children than adults. The absence of female preponderance in pediatric meningiomas is reflected in the skull base subpopulation. Skull base meningiomas in children are most likely to be found in the anterior or middle fossa base, or involving the orbit and optic nerve sheath. Petroclival, suprasellar/parasellar, cerebellopontine angle, cavernous sinus, and foramen magnum tumors are very rare. Meningiomas constitute a small proportion of reported cases of pediatric skull base pathology, and they are entirely absent from many case series. Initial gross total resection is consistently associated with superior outcomes. Surgical approaches to the pediatric skull base must take additional factors into consideration including relatively smaller anatomy, immature dentition, incompletely aerated sinuses and air cells, and altered configurations of structures such as the pterional bony complex. Multidisciplinary expertise is essential to optimizing treatment outcomes. PMID:25685652

  5. 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

  6. Squamous cell carcinoma and suspect peripheral nerve sheath tumor in a 10-year-old Paint horse

    PubMed Central

    Reid, Natalie

    2009-01-01

    A round mass 4 cm in diameter was present on the proximal rostro-lateral border of the right pinna of a 10-year-old, gelded, Paint horse. A preliminary histopathological diagnosis of a potential squamous cell carcinoma and peripheral nerve sheath tumor was made, and the lesion was resected at the base of the lateral edge of the ear. PMID:20119546

  7. Malignant peripheral nerve sheath tumour of the renal parenchyma presenting as a fast growing atypical renal cyst.

    PubMed

    Ouellet, Simon; Doueik, Alexandre; Sabbagh, Robert

    2013-01-01

    Malignant peripheral nerve sheath tumours (MPNST) of the kidney are very rare, with only 3 cases reported in the English and French literature. However, we report the first case of fast growing atypical renal cyst where a magnetic resonance imaging was an interesting adjunct to the computed tomography scan in imaging this rare tumour. PMID:24069105

  8. 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

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

    PubMed

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

    2015-01-01

    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. PMID:25762575

  10. 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.

  11. EGFR-STAT3 signaling promotes formation of malignant peripheral nerve sheath tumors

    PubMed Central

    Wu, Jianqiang; Patmore, Deanna M.; Jousma, Edwin; Eaves, David W.; Breving, Kimberly; Patel, Ami V.; Schwartz, Eric B.; Fuchs, James R.; Cripe, Timothy P.; Stemmer-Rachamimov, Anat O.; Ratner, Nancy

    2014-01-01

    Malignant peripheral nerve sheath tumors (MPNSTs) develop sporadically or in the context of neurofibromatosis type 1 (NF1). EGFR overexpression has been implicated in MPNST formation, but its precise role and relevant signaling pathways remain unknown. We found that EGFR overexpression promotes mouse neurofibroma transformation to aggressive MPNST (GEM-PNST). Immunohistochemistry demonstrated phosphorylated STAT3 (Tyr705) in both human MPNST and mouse GEM-PNST. A specific JAK2/STAT3 inhibitor FLLL32 delayed MPNST formation in an MPNST xenograft nude mouse model. STAT3 knockdown by shRNA prevented MPNST formation in vivo. Finally, reducing EGFR activity strongly reduced pSTAT3 in vivo. Thus, an EGFR-STAT3 pathway is necessary for MPNST transformation and establishment of MPNST xenografts growth but not for tumor maintenance. Efficacy of the FLLL32 pharmacological inhibitor in delaying MPNST growth suggests that combination therapies targeting JAK/STAT3 might be useful therapeutics. PMID:23318430

  12. EGFR-STAT3 signaling promotes formation of malignant peripheral nerve sheath tumors.

    PubMed

    Wu, J; Patmore, D M; Jousma, E; Eaves, D W; Breving, K; Patel, A V; Schwartz, E B; Fuchs, J R; Cripe, T P; Stemmer-Rachamimov, A O; Ratner, N

    2014-01-01

    Malignant peripheral nerve sheath tumors (MPNSTs) develop sporadically or in the context of neurofibromatosis type 1. Epidermal growth factor receptor (EGFR) overexpression has been implicated in MPNST formation, but its precise role and relevant signaling pathways remain unknown. We found that EGFR overexpression promotes mouse neurofibroma transformation to aggressive MPNST (GEM-PNST). Immunohistochemistry demonstrated phosphorylated STAT3 (Tyr705) in both human MPNST and mouse GEM-PNST. A specific JAK2/STAT3 inhibitor FLLL32 delayed MPNST formation in an MPNST xenograft nude mouse model. STAT3 knockdown by shRNA prevented MPNST formation in vivo. Finally, reducing EGFR activity strongly reduced pSTAT3 in vivo. Thus, an EGFR-STAT3 pathway is necessary for MPNST transformation and establishment of MPNST xenografts growth but not for tumor maintenance. Efficacy of the FLLL32 pharmacological inhibitor in delaying MPNST growth suggests that combination therapies targeting JAK/STAT3 might be useful therapeutics. PMID:23318430

  13. Unusual brachial plexus lesion: Hematoma masquerading as a peripheral nerve sheath tumor

    PubMed Central

    Krisht, Khaled M.; Karsy, Michael; Shah, Lubdha M.; Schmidt, Meic H.; Dailey, Andrew T.

    2016-01-01

    Background: Malignant peripheral nerve sheath tumors (MPNSTs) of the brachial plexus have unique radiographic and clinical findings. Patients often present with progressive upper extremity paresthesias, weakness, and pain. On magnetic resonance (MR) imaging, lesions are isointense on T1-weighted and hyperintense on T2-weighted sequences, while also demonstrating marked enhancement on MR studies with gadolinium diethylenetriamine pentaacetic acid. On the basis of their characteristic MR imaging features and rapid clinical progression, two brachial plexus lesions proved to be organizing hematomas rather than MPNST. Methods: A 51-year-old male and a 31-year-old female were both assessed for persistent and worsened left-sided upper extremity pain, paresthesias, and weakness. In both cases, the MR imaging of the brachial plexus demonstrated an extraspinal enhancing lesion located within the left C7–T1 neuroforamina. Results: Although the clinical and radiographic MR features for these 2 patients were consistent with MPNSTs, both lesions proved to be benign organizing hematomas. Conclusions: These two case studies emphasize that brachial plexus hematomas may mimic MPNSTs on MR studies. Accurate diagnosis of these lesions is critical for determining the appropriate management options and treatment plans. Delaying the treatment of a highly aggressive nerve sheath tumor can have devastating consequences, whereas many hematomas resolve without surgery. Therefore, if the patient has stable findings on neurological examination and a history of trauma, surgical intervention may be delayed in favor of repeat MR imaging in 2–3 months to re-evaluate the size of the mass. PMID:26904368

  14. Functional Expression of Electron Transport Chain and FoF1-ATP Synthase in Optic Nerve Myelin Sheath.

    PubMed

    Bartolucci, Martina; Ravera, Silvia; Garbarino, Greta; Ramoino, Paola; Ferrando, Sara; Calzia, Daniela; Candiani, Simona; Morelli, Alessandro; Panfoli, Isabella

    2015-11-01

    Our previous studies reported evidence for aerobic ATP synthesis by myelin from both bovine brainstem and rat sciatic nerve. Considering that the optic nerve displays a high oxygen demand, here we evaluated the expression and activity of the five Respiratory Complexes in myelin purified from either bovine or murine optic nerves. Western blot analyses on isolated myelin confirmed the expression of ND4L (subunit of Complex I), COX IV (subunit of Complex IV) and β subunit of F1Fo-ATP synthase. Moreover, spectrophotometric and in-gel activity assays on isolated myelin, as well as histochemical activity assays on both bovine and murine transversal optic nerve sections showed that the respiratory Complexes are functional in myelin and are organized in a supercomplex. Expression of oxidative phosphorylation proteins was also evaluated on bovine optic nerve sections by confocal and transmission electron microscopy. Having excluded a mitochondrial contamination of isolated myelin and considering the results form in situ analyses, it is proposed that the oxidative phosphorylation machinery is truly resident in optic myelin sheath. Data may shed a new light on the unknown trophic role of myelin sheath. It may be energy supplier for the axon, explaining why in demyelinating diseases and neuropathies, myelin sheath loss is associated with axonal degeneration. PMID:26334391

  15. 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.

  16. Malignant peripheral nerve sheath tumors of the orbit: a clinicopathologic study of eight cases.

    PubMed Central

    Jakobiec, F A; Font, R L; Zimmerman, L E

    1985-01-01

    Eight adult patients with malignant peripheral nerve sheath tumors of the orbit are described. Only two of the patients were known to have von Recklinghausen's neurofibromatosis. The typical clinical history included the development of a mass in the superonasal quadrant of the orbit, which was palpable immediately beneath the skin of the lid. There was a definite tendency for the lesions to arise in, or grow along, the supraorbital nerve--including posteriorly through the superior orbital fissure to the Gasserian ganglion, and even as far posteriorly along the trigeminal rootlets to the pons. Delays in pathologic diagnosis, which beclouded the true nature of the process, led to multiple recurrences, eventuating in five known fatalities out of the eight patients. In addition to intracranial extension, pulmonary metastases and regional cervical metastases were encountered. Once recognized for their diagnostic value, the histopathologic patterns are highly distinctive: biphasic populations of spindled and epithelioid cells; sheets of epithelioid cells or clusters demarcated by delicate reticulin fibers or thicker collagenous trabeculae; malignant plexiform patterns; and neurotubular patterns. Pure spindle cell populations were encountered only in the two patients with von Recklinghausen's disease, and in each, either a pre-existent benign neurofibroma or a coexistent plexiform neurofibroma was found in the pathology specimens. The best management of this condition depends upon early clinical and pathological recognition, leading to radical surgery, which usually consists of orbital exenteration combined with intracranial extirpation of as much of the trigeminal nerve as possible. Postoperative radiotherapy and chemotherapy after radical surgery might also be advisable. Images FIGURE 16 FIGURE 11 FIGURE 15 FIGURE 14 FIGURE 1 FIGURE 2 FIGURE 3 FIGURE 4 FIGURE 5 FIGURE 6 FIGURE 7 FIGURE 8 FIGURE 9 FIGURE 10 FIGURE 12 FIGURE 13 PMID:3938581

  17. Canine cutaneous spindle cell tumours with features of peripheral nerve sheath tumours: a histopathological and immunohistochemical study.

    PubMed

    Gaitero, L; Añor, S; Fondevila, D; Pumarola, M

    2008-07-01

    In veterinary medicine, the term peripheral nerve sheath tumour is usually restricted to neoplasms that are closely associated with an identified nerve. Thirty-three cases of canine cutaneous tumours previously classified as spindle cell tumours with features resembling peripheral nerve sheath tumours were examined. Two histological patterns were identified: dense areas of spindle shaped cells resembling the Antoni A pattern and less cellular areas with more pleomorphic cells resembling the Antoni B pattern. Immunohistochemically, all tumours uniformly expressed vimentin and 15/33 (45.4%) had scattered and patchy expression of S-100. Laminin expression was found in 25/33 (75.7%) tumours and collagen IV labelling occurred in 14/33 (42.4%). Expression of protein gene product 9.5 was detected in 31/33 (93.9%) of tumours and neuron specific enolase labelling was present in 27/33 (81.8%). Glial fibrillary acidic protein was only expressed within the cytoplasm of some large multinucleated cells in one tumour. These findings suggest that any cutaneous tumour with one of the two histopathological patterns described above should be described as a cutaneous peripheral nerve sheath tumour and that expression of S-100, laminin and collagen IV may be used to define a schwannoma. PMID:18514218

  18. Diagnostic value of the optic nerve sheath diameter in pseudotumor cerebri.

    PubMed

    Bekerman, Inessa; Sigal, Tal; Kimiagar, Itzhak; Almer, Zina Evy; Vaiman, Michael

    2016-08-01

    If persistent severe headache remains the only complaint of a patient, then the diagnosis of pseudotumor cerebri (PTC) can be delayed because in such cases practitioners are hesitant to immediately apply invasive intracranial pressure (ICP) measurement. Our purpose was to apply the technique of measuring diameters of the optic nerve sheath (ONSD) as a diagnostic tool in cases of PTC. Our aim was to provide practitioners with an additional sign to speed up their decision making about implementation of the lumbar puncture. In a retrospective study, CT scan data of 35 consecutive adult patients with PTC were collected and analyzed. ONSD were measured at the point where the ophthalmic artery crosses the optic nerve (anatomical landmark). The correlation analysis was performed with sex, age, and neuro-ophthalmological findings. We found that the ONSD was enlarged in 94.3% of patients with PTC. The enlarged ONSD were 6.2±1.2mm for the right and 6.3±0.9mm for the left (cut-off value >5.5mm). The enlargement was bilateral, and no correlation with age or sex was found (p=0.67 and p=0.76, respectively). Presence of papilledema was detected in 91.4% of patients (32/35) presenting as a slightly less valuable diagnostic sign compared with ONSD. We conclude that in the majority of cases of PTC the ONSD is significantly enlarged, indicating elevated ICP even if CT scans are negative. Implementing this ONSD method as a diagnostic tool in cases of suspected PTC may help in early accurate diagnosis, avoiding misdiagnosis, and providing appropriate early treatment. PMID:27168453

  19. Primary malignant peripheral nerve sheath tumor of the lung in a young child without neurofibromatosis type 1.

    PubMed

    Muwakkit, Samar A; Rodriguez-Galindo, Carlos; El Samra, Ahmad I; Khoury, Ruby; Akel, Samir R; Mroueh, Salman; Razzouk, Bassem; Abboud, Miguel R

    2006-10-15

    Malignant peripheral nerve sheath tumors (MPNST) are uncommon in children and almost half of the cases occur in patients with neurofibromatosis 1 (NF1). We report a child with a primary MPNST of the lung without NF1. MPNST of the lung has similar clinical and radiologic characteristics as pleuropulmonary blastoma. We suggest to include MPNST of the lung in the differential diagnosis of intrapulmonary masses in children. PMID:16544294

  20. Extending the Convergence of Canonical WNT Signaling and Classic Cancer Pathways for Treatment of Malignant Peripheral Nerve Sheath Tumors

    PubMed Central

    Reilly, Karlyne M.

    2016-01-01

    Summary Malignant peripheral nerve sheath tumors (MPNSTs) are incurable tumors of the Schwann cell lineage that progress unpredictably from benign plexiform neurofibromas (PNFs). In this issue of Cancer Discovery Watson and colleagues (1) use an insertional mutagenesis screen combined with network analysis to identify the canonical Wnt signaling pathway as an important potential biomarker of tumor progression and target for combination therapy in MPNSTs. PMID:23749527

  1. 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

  2. Chromosomal imbalances in malignant peripheral nerve sheath tumor detected by metaphase and microarray comparative genomic hybridization.

    PubMed

    Nakagawa, Yasuko; Yoshida, Aki; Numoto, Kunihiko; Kunisada, Toshiyuki; Wai, Daniel; Ohata, Norihide; Takeda, Ken; Kawai, Akira; Ozaki, Toshifumi

    2006-02-01

    Malignant peripheral nerve sheath tumors (MPNSTs) are highly malignant tumors affecting adolescents and adults. There have been a few reports on chromosomal aberrations of MPNSTs; however, the tumor-specific alteration remains unknown. We characterized the genomic alterations in 8 MPNSTs and 8 schwannomas by metaphase comparative genomic hybridization (CGH). In 5 of 8 MPNSTs, microarray CGH was added for more detailed analyses. Frequent gains were identified on 3q13-26, 5p13-14, and 12q11-23 and frequent losses were at 1p31, 10p, 11q24-qter, 16, and 17. Microarray CGH revealed frequent gains of EGFR, DAB2, MSH2, KCNK12, DDX15, CDK6, and LAMA3, and losses of CDH1, GLTSCR2, EGR1, CTSB, GATA3, and SULT2A1. These genes seem to be responsible for developing MPNSTs. The concordance rate between metaphase CGH and microarray CGH was 66%. Metaphase CGH was useful for identifying chromosomal alterations before applying microarray CGH. PMID:16391845

  3. Inhibition of Eyes Absent Homolog 4 expression induces malignant peripheral nerve sheath tumor necrosis.

    PubMed

    Miller, S J; Lan, Z D; Hardiman, A; Wu, J; Kordich, J J; Patmore, D M; Hegde, R S; Cripe, T P; Cancelas, J A; Collins, M H; Ratner, N

    2010-01-21

    Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive sarcomas without effective therapeutics. Bioinformatics was used to identify potential therapeutic targets. Paired Box (PAX), Eyes Absent (EYA), Dachsund (DACH) and Sine Oculis (SIX) genes, which form a regulatory interactive network in Drosophila, were found to be dysregulated in human MPNST cell lines and solid tumors. We identified a decrease in DACH1 expression, and increases in the expressions of PAX6, EYA1, EYA2, EYA4, and SIX1-4 genes. Consistent with the observation that half of MPNSTs develop in neurofibromatosis type 1 (NF1) patients, subsequent to NF1 mutation, we found that exogenous expression of the NF1-GTPase activating protein-related domain normalized DACH1 expression. EYA4 mRNA was elevated more than 100-fold as estimated by quantitative real-time PCR in most MPNST cell lines. In vitro, suppression of EYA4 expression using short hairpin RNA reduced cell adhesion and migration and caused cellular necrosis without affecting cell proliferation or apoptotic cell death. MPNST cells expressing shEYA4 either failed to form tumors in nude mice or formed very small tumors, with extensive necrosis but similar levels of proliferation and apoptosis as control cells. Our findings identify a role of EYA4 and possibly interacting SIX and DACH proteins in MPNSTs and suggest the EYA4 pathway as a rational therapeutic target. PMID:19901965

  4. Optic nerve sheath diameters in healthy adults measured by computer tomography

    PubMed Central

    Vaiman, Michael; Abuita, Rani; Bekerman, Inessa

    2015-01-01

    AIM To measure optic nerve sheath diameters (ONSD) in different locations by computer tomography (CT) and to recommend the best location for cases when ONSD is used for intracranial pressure monitoring. METHODS In a prospective cohort study, CT data of 300 healthy adults were analyzed (600 eyes). In all cases, the CT investigation was performed at the Emergency Department because of the various conditions that proved not to be connected with ophthalmological or neurological pathology. The ONSD were measured at 3 mm and 8 mm distance from the globe, and 3 mm from the anterior opening of the optic canal. The correlation analysis was performed with gender, age, and ethnic background. RESULTS The right/left ONSD are 4.94±1.51/5.17±1.34 mm at 3 mm, 4.35±0.76/4.45±0.62 mm at 8 mm from the globe, and 3.55±0.82/3.65±0.7 mm at 3 mm from the optic canal. No significant differences correlated with gender of the patients, their age, and ethnic background were found. CONCLUSION In healthy persons, the ONSD varies from 5.17±1.34 mm to 3.55±0.82 mm in different locations within the intraorbital space. The most stable results with lesser standard deviation can be obtained if it is measured 8-10 mm from the globe. PMID:26682181

  5. 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. PMID:26650448

  6. 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

  7. Malignant peripheral nerve sheath tumor of kidney—A case report☆

    PubMed Central

    Alharbi, Badr

    2013-01-01

    INTRODUCTION Malignant peripheral nerve sheath tumors (MPNSTs) are very rare soft-tissue tumors. These tumors originate from Schwann cells or pluripotent perineural cells of the neural crest. An isolated MPNST of the kidney is extremely rare and has been reported in only six cases to date. PRESENTATION OF CASE A 30-year-old female with an unremarkable past medical and surgical history presented with an enlarging, right-sided abdominal mass for the last 3 months. A CT scan of the abdomen and pelvis showed a large exophytic mass arising from the right kidney. A metastatic work-up was negative, so a right radical nephrectomy was performed. Histopathological and immunohistochemical studies confirmed the diagnosis of an MPNST. DISCUSSION MPNSTs are rare and aggressive soft-tissue sarcomas that are usually associated with neurofibromatosis type 1 (NF-1) but that also occur post-radiation and sporadically. These tumors are highly malignant neoplasms with a high recurrence rate and distant metastases. Complete surgical excision remains the mainstay of treatment due to the limited response of MPNSTs to both chemotherapy and radiotherapy. CONCLUSION MPNSTs arising from the kidney are very rare. Complete surgical resection provides the only hope for cure. Postoperative chemotherapy and radiotherapy are still controversial, although postoperative radiotherapy is currently recommended by an oncology consensus group. PMID:23995478

  8. Malignant Peripheral Nerve Sheath Tumors in Children with Neurofibromatosis Type 1

    PubMed Central

    Pourtsidis, Apostolos; Baka, Margarita; Bouhoutsou, Despina; Varvoutsi, Maria; Synodinou, Maria; Giamarelou, Panagiota; Kosmidis, Helen

    2014-01-01

    Purpose. Malignant peripheral nerve sheath tumors (MPNSTs) are rare in children and account for approximately 5–10% of all soft tissue sarcomas in adults. MPNSTs may occur independently but individuals with neurofibromatosis type 1 (NF1) have a significantly increased risk. Our aim is to present patients with MPNST treated in our department. Cases and Results. In this report we present 4 cases of MPNSTs (3 females: 13, 12, and 13 years old and 1 male: 10 years old) arising in patients with NF1. All of them presented with an enlarging mass and pain at diagnosis. Tumor was located in the buttock, the spinal cord, the trunk, and the left leg proximal to the heel. Wide excision of the tumor and radiotherapy were applied to all and adjuvant chemotherapy was given to three of them after the disease was progressed. All four died 32, 18, 10, and 22 months after diagnosis with progressive disease locally and pulmonary metastases in two of them. Conclusions. In conclusion, MPNSTs arising in patients with NF1 are high grade sarcomas with short survival. Individuals with NF1 should be followed closely in order to identify early the development of MPNSTs. Aggressive surgery and complete excision significantly improves disease-free survival. The usefulness of radiation therapy in MPNSTs is not determined although all patients will receive radiation therapy at some stage of the disease. The role of chemotherapy is unclear. PMID:25317349

  9. Malignant peripheral nerve sheath tumors in children with neurofibromatosis type 1.

    PubMed

    Pourtsidis, Apostolos; Doganis, Dimitrios; Baka, Margarita; Bouhoutsou, Despina; Varvoutsi, Maria; Synodinou, Maria; Giamarelou, Panagiota; Kosmidis, Helen

    2014-01-01

    Purpose. Malignant peripheral nerve sheath tumors (MPNSTs) are rare in children and account for approximately 5-10% of all soft tissue sarcomas in adults. MPNSTs may occur independently but individuals with neurofibromatosis type 1 (NF1) have a significantly increased risk. Our aim is to present patients with MPNST treated in our department. Cases and Results. In this report we present 4 cases of MPNSTs (3 females: 13, 12, and 13 years old and 1 male: 10 years old) arising in patients with NF1. All of them presented with an enlarging mass and pain at diagnosis. Tumor was located in the buttock, the spinal cord, the trunk, and the left leg proximal to the heel. Wide excision of the tumor and radiotherapy were applied to all and adjuvant chemotherapy was given to three of them after the disease was progressed. All four died 32, 18, 10, and 22 months after diagnosis with progressive disease locally and pulmonary metastases in two of them. Conclusions. In conclusion, MPNSTs arising in patients with NF1 are high grade sarcomas with short survival. Individuals with NF1 should be followed closely in order to identify early the development of MPNSTs. Aggressive surgery and complete excision significantly improves disease-free survival. The usefulness of radiation therapy in MPNSTs is not determined although all patients will receive radiation therapy at some stage of the disease. The role of chemotherapy is unclear. PMID:25317349

  10. Controversies: Optic nerve sheath fenestration versus shunt placement for the treatment of idiopathic intracranial hypertension

    PubMed Central

    Spitze, Arielle; Lam, Peter; Al-Zubidi, Nagham; Yalamanchili, Sushma; Lee, Andrew G

    2014-01-01

    Background: Idiopathic intracranial hypertension (IIH) has been increasing in prevalence in the past decade, following the obesity epidemic. When medical treatment fails, surgical treatment options must be considered. However, controversy remains as to which surgical procedure is the preferred surgical option – optic nerve sheath fenestration (ONSF) or cerebrospinal fluid (CSF) shunting – for the long-term treatment of this syndrome. Purpose: To provide a clinical update of the pros and cons of ONSF versus shunt placement for the treatment of IIH. Design: This was a retrospective review of the current literature in the English language indexed in PubMed. Methods: The authors conducted a PubMed search using the following terms: Idiopathic IIH, pseudotumor cerebri, ONSF, CSF shunts, vetriculo-peritoneal shunting, and lumbo-peritoneal shunting. The authors included pertinent and significant original articles, review articles, and case reports, which revealed the new aspects and updates in these topics. Results: The treatment of IIH remains controversial and lacks randomized controlled clinical trial data. Treatment of IIH rests with the determination of the severity of IIH-related visual loss and headache. Conclusion: The decision for ONSF versus shunting is somewhat institution and surgeon dependent. ONSF is preferred for patients with visual symptoms whereas shunting is reserved for patients with headache. There are positive and negative aspects of both procedures, and a prospective, randomized, controlled trial is needed (currently underway). This article will hopefully be helpful in allowing the reader to make a more informed decision until that time. PMID:25449938

  11. Malignant peripheral nerve sheath tumor of the lower labial mucosa: case report and literature review.

    PubMed

    do Amaral, Thiago Lucena; Valiati, Renato; de Andrade, Bruno Augusto Benevenuto; Rumayor Piña, Alicia; Torres, Sandra Regina; Romañach, Mário José; Agostini, Michelle

    2016-08-01

    Malignant peripheral nerve sheath tumor (MPNST) is an uncommon soft tissue sarcoma with a predilection for the extremities and trunk of adults, rarely occurring in the oral cavity. To date, 10 cases of MPNSTs affecting the lower labial mucosa have been reported in the English language literature. We describe an additional case of MPNST in the lower labial mucosa of a 67-year-old male patient, who exhibited a painful swelling with a history of 4 months of evolution. Microscopic examination showed dense plexiform fascicles of spindle cells with wavy atypical nuclei. Mitotic figures and nuclear pleomorphism were evident. Immunohistochemical analysis of tumor cells revealed positivity for S-100 protein, CD56, CD34, and neuron-specific enolase but was negative for neurofilament protein, glut-1, claudin-1, desmin, and smooth muscle actin. Ki-67 labeling was 20%. The final diagnosis was MPNST. The lesion was surgically removed with wide margins, with no signs of recurrence after 4 years of follow-up. PMID:26852824

  12. Correlation of measurement of optic nerve sheath diameter using ultrasound with magnetic resonance imaging

    PubMed Central

    Shirodkar, Chetan G.; Munta, Kartik; Rao, S. Manimala; Mahesh, M. Uma

    2015-01-01

    Background and Aims: Analysis to correlate the measurements of optic nerve sheath diameter (ONSD) obtained by using ultrasound to magnetic resonance imaging (MRI) techniques in order to establish the accuracy of ocular sonography as a noninvasive modality for detecting raised intracranial pressure (ICP). Materials and Methods: A prospective, observational study was performed in 100 cases of adult meningoencephalitis patients admitted to Intensive Care Unit in whom MRI was performed for neurodiagnosis. ONSD was measured in such patients, 3 mm behind the globe in each eye. A mean binocular ONSD >4.6 mm in female and 4.8 mm in male was taken as cut-off values for diagnosing raised ICP. This was compared with ONSD measured on T2-weighted MRI image measured 3 mm behind the globe. The reading obtained from both the methods were compared with Bland–Altman analysis for correlation and the findings were tabulated. Results: The mean ONSD values measured with ultrasonography (USG) and MRI for female were 5.48 ± 0.43 mm and 5.68 ± 0.44 mm and for male were 5.40 ± 0.37 mm and 5.56 ± 0.38 mm, respectively. The mean age of the female and male was 53.90 ± 17.84 and 56.06 ± 15.67 years, respectively. On comparing ultrasound with MRI-derived ONSD values, we found acceptable agreement between both methods for measurements at a depth of 3 mm (r = 0.02, P < 0.001). Conclusion: In our study, we have found a good correlation between ocular USG and MRI of ONSD. The study has shown agreement with the fact that ocular sonography can be used as a noninvasive tool for detecting raised ICP with accuracy. PMID:26321806

  13. Visual outcomes following optic nerve sheath fenestration via the medial transconjunctival approach.

    PubMed

    Vaidya, Neel S; Mahmoud, Ashraf M; Buzzacco, Dominic; Katz, Steven E

    2016-10-01

    This article determines the safety of optic nerve sheath fenestration (ONSF) for the treatment of patients with intracranial hypertension in the immediate 6-month post-operative period and its efficacy in reducing optic disk edema. Retrospective, non-comparative interventional case series. 207 eyes in 104 patients undergoing ONSF between the years 2005 and 2014. Papilledema grade based on modified Frisen scale and mean deviation of Humphrey visual field. 207 eyes of 104 patients (102 IIH, 2 IH due to dural sinus thrombosis) were included in the study. The patients were 96.1% female (N = 100) and 3.9% male (N = 4). The average patient age was 28.8 years (SD ± 9.5 years) and had a mean opening pressure of 39.85 cmH2O (SD ± 8.4 cmH2O). Mean follow-up period was 6.0 months (SD ± 5.9 months). Papilledema resolved in 76.1% of eyes at 1 week (N = 102 eyes), 75% of eyes at 1 month (N = 90 eyes), and 71% of eyes at 6 months (N = 94 eyes). Visual field comparison had a mean of the paired differences in MD at 1 week, 1 month, and 6 months of 1.59dB (P = 0.006), 2.53dB (P < 0.001), and 1.30dB (P = 0.016), respectively. ONSF is effective in reducing optic disk edema and does not cause vision loss in the 6-month post-operative period regardless of severity of IIH (as judged by elevation of opening pressure measured at pre-operative assessment). PMID:27541942

  14. Prognostic roles for fibroblast growth factor receptor family members in malignant peripheral nerve sheath tumor

    PubMed Central

    Song, Fengju; Zheng, Hong; Chen, Kexin; Zhang, Wei; Yang, Jilong

    2016-01-01

    Background Malignant peripheral nerve sheath tumors (MPNST) are rare, highly malignant, and poorly understood sarcomas. The often poor outcome of MPNST highlights the necessity of identifying prognostic predictors for this aggressive sarcoma. Here, we investigate the role of fibroblast growth factor receptor (FGFR) family members in human MPNSTs. Results aCGH and bioinformatics analysis identified frequent amplification of the FGFR1 gene. FISH analysis revealed that 26.9% MPNST samples had amplification of FGFR1, with both focal and polysomy patterns observed. IHC identified that FGFR1 protein expression was positively correlated with FGFR1 gene amplification. High expression of FGFR1 protein was associated with better overall survival (OS) and was an independent prognostic predictor for OS of MPNST patients. Additionally, combined expression of FGFR1 and FGFR2 protein characterized a subtype of MPNST with better OS. FGFR4 protein was expressed 82.3% of MPNST samples, and was associated with poor disease-free survival. Materials and Methods We performed 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 Medical University Cancer Institute and Hospital. Fluorescence in situ hybridization (FISH) was used to validate the gene amplification detected by aCGH analysis. Another cohort of 63 formalin-fixed paraffin-embedded MPNST samples (including 52 samples for FISH assay) was obtained to explore FGFR1, 2, 3, and 4 protein expression by immunohistochemical (IHC) analysis. Conclusions Our integrated genomic and molecular studies provide evidence that FGFRs play different prognostic roles in MPNST. PMID:26993773

  15. HDAC8, A Potential Therapeutic Target for the Treatment of Malignant Peripheral Nerve Sheath Tumors (MPNST)

    PubMed Central

    Lopez, Gonzalo; Bill, Kate Lynn J.; Bid, Hemant Kumar; Braggio, Danielle; Constantino, Dylan; Prudner, Bethany; Zewdu, Abeba; Batte, Kara; Lev, Dina; Pollock, Raphael E.

    2015-01-01

    Introduction HDAC isoform-specific inhibitors may improve the therapeutic window while limiting toxicities. Developing inhibitors against class I isoforms poses difficulties as they share high homology among their catalytic sites; however, HDAC8 is structurally unique compared to other class I isoforms. HDAC8 inhibitors are novel compounds and have affinity for class I HDAC isoforms demonstrating anti-cancer effects; little is known about their activity in malignant peripheral nerve sheath tumors (MPNST). Recently, we demonstrated anti-MPNST efficacy of HDAC8i in human and murine-derived MPNST pre-clinical models; we now seek to consider the potential therapeutic inhibition of HDAC8 in MPNST. Methods Four Human MPNST cell lines, a murine-derived MPNST cell line, and two HDAC8 inhibitors (PCI-34051, PCI-48012; Pharmacyclics, Inc. Sunnyvale, CA) were studied. Proliferation was determined using MTS and clonogenic assays. Effects on cell cycle were determined via PI FACS analysis; effects on apoptosis were determined using Annexin V-PI FACS analysis and cleaved caspase 3 expression. In vivo growth effects of HDAC8i were evaluated using MPNST xenograft models. 2D gel electrophoresis and mass spectrometry were used to identify potential HDAC8 deacetylation substrates. Results HDAC8i induced cell growth inhibition and marked S-phase cell cycle arrest in human and murine-derived MPNST cells. Relative to control, HDAC8i induced apoptosis in both human and murine-derived MPNST cells. HDAC8i exhibited significant effects on MPNST xenograft growth (p=0.001) and tumor weight (p=0.02). Four potential HDAC8 substrate targets were identified using a proteomic approach: PARK7, HMGB1, PGAM1, PRDX6. Conclusions MPNST is an aggressive sarcoma that is notoriously therapy-resistant, hence the urgent need for improved anti-MPNST therapies. HDAC8 inhibition may be useful for MPNST by improving efficacy while limiting toxicities as compared to pan-HDACis. PMID:26200462

  16. Optic nerve sheath diameter is not related to high altitude headache: a randomized controlled trial.

    PubMed

    Lawley, Justin Stevan; Oliver, Samuel James; Mullins, Paul; Morris, Daniel; Junglee, Naushad Ali; Jelleyman, Charlotte; Macdonald, Jamie Hugo

    2012-09-01

    The mechanism of high altitude headache (HAH) remains unknown. The aim of this study was to determine experimentally whether optic nerve sheath diameter (ONSD), as an indicator of intracranial pressure, is related to HAH. Following sea level measurements at 3 and 12 hours (SL), 23 subjects were passively transported to high altitude (3777 m, HA) via cable car. HAH, ONSD, arterial oxygen saturation (Spo(2)), and fluid balance were determined at 3, 12, 24, and 36 hours. After 12 hours exposure to HA, subjects were classified by visual analogue scale (VAS) as either HAH positive (HAH+) or HAH negative (HAH-). Acetazolamide (250 mg) or placebo was then randomly prescribed at 15, 20, and 28 hours. Outcome means were compared via analysis of variance, and relationships between variables were analyzed by longitudinal regression. Acetazolamide had no statistically significant effect on HAH (p=0.63) or ONSD (p=0.98), but produced a negative fluid balance (p<0.01) (and also increased Spo(2) in exploratory analyses). Spo(2) was lower in HAH+ than HAH- [85 (3)% versus 88 (2)%, p=0.03). Nevertheless, ONSD increased similarly in HAH+ and HAH- (interaction p=0.90). ONSD also remained significantly elevated above SL values for the entire HA period [SL, 5.2 (0.5) versus HA, 5.6 (0.5) mm, p<0.01], despite headache resolving with acclimatization [VAS: SL, 1/100 (3) mm versus HA 3 h, 9/100 (13); 12 h, 10/100 (14); 24 h, 8/100 (12); 36 h, 1/100 (4) mm, p<0.01]. Furthermore, HAH was significantly correlated with Spo(2) (β=-1.39, p<0.01) but not with ONSD (β=0.59, p=0.57). These data do not support that intracranial pressure is associated with the development or amelioration of mild HAH. Clinical trial registration NCT01288781. PMID:22994519

  17. 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

  18. Comparative methylome analysis of benign and malignant peripheral nerve sheath tumors.

    PubMed

    Feber, Andrew; Wilson, Gareth A; Zhang, Lu; Presneau, Nadege; Idowu, Bernadine; Down, Thomas A; Rakyan, Vardhman K; Noon, Luke A; Lloyd, Alison C; Stupka, Elia; Schiza, Vassia; Teschendorff, Andrew E; Schroth, Gary P; Flanagan, Adrienne; Beck, Stephan

    2011-04-01

    Aberrant DNA methylation (DNAm) was first linked to cancer over 25 yr ago. Since then, many studies have associated hypermethylation of tumor suppressor genes and hypomethylation of oncogenes to the tumorigenic process. However, most of these studies have been limited to the analysis of promoters and CpG islands (CGIs). Recently, new technologies for whole-genome DNAm (methylome) analysis have been developed, enabling unbiased analysis of cancer methylomes. By using MeDIP-seq, we report a sequencing-based comparative methylome analysis of malignant peripheral nerve sheath tumors (MPNSTs), benign neurofibromas, and normal Schwann cells. Analysis of these methylomes revealed a complex landscape of DNAm alterations. In contrast to what has been reported for other tumor types, no significant global hypomethylation was observed in MPNSTs using methylome analysis by MeDIP-seq. However, a highly significant (P < 10(-100)) directional difference in DNAm was found in satellite repeats, suggesting these repeats to be the main target for hypomethylation in MPNSTs. Comparative analysis of the MPNST and Schwann cell methylomes identified 101,466 cancer-associated differentially methylated regions (cDMRs). Analysis showed these cDMRs to be significantly enriched for two satellite repeat types (SATR1 and ARLα) and suggests an association between aberrant DNAm of these sequences and transition from healthy cells to malignant disease. Significant enrichment of hypermethylated cDMRs in CGI shores (P < 10(-60)), non-CGI-associated promoters (P < 10(-4)) and hypomethylated cDMRs in SINE repeats (P < 10(-100)) was also identified. Integration of DNAm and gene expression data showed that the expression pattern of genes associated with CGI shore cDMRs was able to discriminate between disease phenotypes. This study establishes MeDIP-seq as an effective method to analyze cancer methylomes. PMID:21324880

  19. 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.

  20. 18F-fluorodeoxyglucose positron emission tomography/computed tomography in a case of malignant peripheral nerve sheath tumor: An unusual presentation

    PubMed Central

    D’souza, Maria M; Jaimini, Abhinav; Kumar Dhali, Tapan; D'souza, Paschal; Saw, Sanjiv; Sharma, Rajnish; Mondal, Anupam

    2013-01-01

    Malignant peripheral nerve sheath tumors (MPNSTs) are rare tumors, with an estimated incidence of 0.1/100,000/year. They are regarded as a rare variety of soft-tissue sarcomas that derive from peripheral nerves or from cells associated with the nerve sheath. Until 50% of observed MPNSTs occur in patients with neurofibromatosis 1 (NF1). The typical presenting signs and symptoms of a PNST are a palpable mass involving a peripheral nerve, loss of nerve function and/or pain. Recently, positron emission tomography (PET) has been used to detect 18F-fluorodeoxyglucose uptake in these tumors. Most of the PET studies have been reported in patients with NF1. We report a case of sporadic MPNST masquerading as infectious dermatoses, with an unusual PET/computed tomography presentation. PMID:24250026

  1. [Changes in the sensitivity of the cornea in patients with intracerebral tumors. Studies of patients with meningioma of the cranial base and neurinoma of the trigeminal nerve and the vestibulocochlear nerve using the Draeger esthesiometer].

    PubMed

    Heller, H; Koniszewski, G

    1988-10-01

    The center of the cornea was measured preoperatively in 29 patients suffering from intracranial tumors (acoustic neuroma, trigeminus neuroma, basal meningioma). These measurements were made with the Draeger electronic-optical esthesiometer; the results were supplemented by an analysis of tumor diameters determined by computer tomography. In each case one-half of the patients with acoustic neuromas and medial meningiomas of the wing of the sphenoid bone manifested a reduction in sensitivity at the center of the ipsilateral cornea (normal: 0.8 to 1.7 x 10(-5) N). The diameters of the sensitivity-reducing acoustic neuromas ranged from 15 mm to 45 mm. It may be deduced both from the topographic conditions at the skull base in the vicinity of the porus acusticus internus and from the conditions associated with a pressure-induced lesion of a peripheral nerve that medial acoustic neuromas as small as 10.1 mm in diameter can lead to a reduction in the conductivity of the ipsilateral trigeminal nerve. Only when they attain a diameter of 28.4 mm and when the proportions of the skull base are equally spacious do the acoustic neuromas regularly cause an ipsilateral corneal hypesthesia. PMID:3236740

  2. An intraosseous malignant peripheral nerve sheath tumor of the lumbar spine without neurofibromatosis: Case report and review of the literature.

    PubMed

    Suzuki, Kayo; Yasuda, Taketoshi; Hori, Takeshi; Watanabe, Kenta; Kanamori, Masahiko; Kimura, Tomoatsu

    2014-06-01

    A malignant peripheral nerve sheath tumor (MPNST) is defined as any malignant tumor that develops or differentiates from cells in the peripheral nerve sheath. This tumor is commonly associated with neurofibromatosis type 1 (NF1) and previous radiotherapy treatment. Primary intraosseous MPNSTs are extremely rare and a case of the lumbar spine in a patient without NF1 is reported in the present study, with a review of the intraosseous MPNST literature. A 45-year-old female presented with a 1-month history of severe lower back pain and pain radiating to the left leg. A total en bloc spondylectomy of L4 was performed. The postoperative histopathological diagnosis was MPNST with deletion of NF1, confirmed by dual-color fluorescence in situ hybridization (FISH) analysis. The tumor recurred 1 month following the surgery. Although adjuvant chemotherapy was administered, the patient succumbed due to intramedullary dissemination and carcinomatous meningitis 8 months following the initial consultation. NF1 deletion by FISH analysis may be particularly useful in distinguishing MPNST from other high-grade malignancies with overlapping morphological features. PMID:24932270

  3. Fenestration in the myelin sheath of nerve fibers of the shrimp: a novel node of excitation for saltatory conduction.

    PubMed

    Hsu, K; Terakawa, S

    1996-07-01

    Giant nerve fibers of the shrimp family Penaeidae conduct impulses at the velocity highest among all animal species (approximately 210 m/s; highest in mammals = 120 m/s). We examined these giant and other small nerve fibers morphologically using a differential interference contrast microscope as well as an electron microscope, and found a very specialized form of excitable membrane that functions as a node for saltatory conduction of the impulse. This node appeared under the light microscope as a characteristic pattern of concentrically aligned rings in a very small spot of the myelin sheath. The diameter of the innermost ring of the node was about 5 microns, and the distance between these nodes was as long as 12 mm. Via an electron microscope, these nodes were characterized by a complete lack of the myelin sheath, forming a fenestration that has a tight junction with an axonal membrane. Voltage clamp measurements by a sucrose gap technique demonstrated that the axonal membrane at these fenestration nodes is exclusively excitable and that the large submyelinic space is a unique conductive pathway for loop currents for saltatory conduction through such fenestration nodes. PMID:8807532

  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. PMID:27234913

  5. Malignant peripheral nerve sheath tumor of the colon in a patient with von Recklinghausen's disease: report of a case.

    PubMed

    Marwah, Sanjay; Gurawalia, Jai Prakash; Sheoran, Kapil Dev; Marwah, Nisha; Gupta, Sumiti; Ranga, Hansraj

    2013-12-01

    Malignant peripheral nerve sheath tumor (MPNST) arising from the colon is an extremely rare clinical entity. We report one such case of an adult female with neurofibromatosis type I who presented with pain and a lump in her abdomen. A computed tomographic scan revealed a 10 × 8-cm mass in the splenic flexure of her colon that was compressing its lumen, but the results of a colonoscopy were normal. Segmental resection of the left colon was performed based on the clinical possibility of gastrointestinal stromal tumor. However, on histopathological examination and immunohistochemical staining, the final diagnosis came out to be MPNST. This case highlights that, although rare, the possibility of MPNST should be considered when dealing with extramucosal colonic wall tumors. PMID:26182132

  6. 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.

  7. Imaging of the malignant peripheral nerve sheath tumour with emphasis οn ultrasonography: correlation with MRI.

    PubMed

    Rafailidis, Vasileios; Kaziani, Theodora; Theocharides, Costas; Papanikolaou, Athanasios; Rafailidis, Dimitrios

    2014-09-01

    Malignant peripheral nerve sheath tumours affect 0.001 % of the population. The epithelioid subtype accounts for only 5 % of them. We herein present a patient with a palpable mass on the left calf, which was investigated by ultrasonography, ultrasonographically guided fine-needle biopsy and MRI. Ultrasonography estimated the size of the tumour and evaluated its echotexture. Doppler technique detected characteristic corkscrew-type blood vessel entering in the upper pole of the mass. T1-weighted, fat-suppression and gadolinium-enhanced MRI sequences confirmed ultrasonographic findings. In conclusion, although final diagnosis was established by biopsy, ultrasonography and MRI proved complementary in the characterization of the lesion and planning of surgery. PMID:25177396

  8. 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

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

    PubMed Central

    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

    2015-01-01

    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

  10. Genomic and molecular aberrations in malignant peripheral nerve sheath tumor and their roles in personalized target therapy.

    PubMed

    Yang, Jilong; Du, Xiaoling

    2013-09-01

    Malignant peripheral nerve sheath tumors (MPNSTs) are malignant tumors with a high rate of local recurrence and a significant tendency to metastasize. Its dismal outcome points to the urgent need to establish better therapeutic strategies for patients harboring MPNSTs. The investigations of genomic and molecular aberrations in MPNSTs which detect many chromosomal aberrations, pathway abnormalities, and specific molecular aberrant events would supply multiple potential therapy targets and contribute to achievement of personalized medicine. The involved genes in the significant gains aberrations include BIRC5, CCNE2, DAB2, DDX15, EGFR, DAB2, MSH2, CDK6, HGF, ITGB4, KCNK12, LAMA3, LOXL2, MET, and PDGFRA. The involved genes in the significant deletion aberrations include CDH1, GLTSCR2, EGR1, CTSB, GATA3, SULT2A1, GLTSCR2, HMMR/RHAMM, LICAM2, MMP13, p16/INK4a, RASSF2, NM-23H1, and TP53. These genetic aberrations involve in several important signaling pathways such as TFF, EGFR, ARF, IGF1R signaling pathways. The genomic and molecular aberrations of EGFR, IGF1R, SOX9, EYA4, TOP2A, ETV4, and BIRC5 exhibit great promise as personalized therapeutic targets for MPNST patients. PMID:23830351

  11. A retroperitoneal NF1-independent malignant peripheral nerve sheath tumor with elevated serum CA125: case report and discussion.

    PubMed

    Yan, Bing; Meng, Xianze; Shi, Bian; Shi, Jun; Qin, Zhifeng; Wei, Pinkang

    2012-08-01

    Malignant peripheral nerve sheath tumors (MPNSTs) are usually located in the trunk, extremities, head, or neck, and most occur with neurofibromatosis type 1 (NF1; von Recklinghausen's disease). No biomarkers have previously been found to be associated with their progression. Retroperitoneal NF1-independent MPNSTs are rare; they are considered to be less aggressive and to have better prognoses compared to NF1-related tumors. Currently, en bloc excision is the only consensus treatment approach. In a 27-year-old male with a giant retroperitoneal MPNST and no stigmata or family history of neurofibromatosis type-1 (NF1), a remarkable elevation of serum CA125 was detected. The high-grade tumor displayed a striking progression: the primary lesion, 25 cm in diameter, recurred in its previous site as a 17-cm MPNST less than 50 days after total excision. Subsequent treatment with microwave ablation and huachansu, a traditional Chinese medication, proved ineffective, and the patient died within 3 months. Our case suggests that retroperitoneal MPNSTs can deteriorate rapidly even if NF1 independent, that aggressive treatment may not benefit large high-grade MPNSTs, and that novel and effective treatment is urgently needed. Our case also suggests the possibility of using serum tumor markers in the early detection and monitoring of MPNSTs. PMID:22528792

  12. Combinatorial Therapy with Tamoxifen And Trifluoperazine Effectively Inhibits Malignant Peripheral Nerve Sheath Tumor Growth by Targeting Complementary Signaling Cascades

    PubMed Central

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

    2014-01-01

    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 NF1-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. Additionally, 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. PMID:25289889

  13. Canine cutaneous peripheral nerve sheath tumours versus fibrosarcomas can be differentiated by neuroectodermal marker genes in their transcriptome.

    PubMed

    Klopfleisch, R; Meyer, A; Lenze, D; Hummel, M; Gruber, A D

    2013-02-01

    The diagnostic differentiation between canine fibrosarcomas and peripheral nerve sheath tumours (PNSTs) is based on histopathological phenotype. Histological differentiation of these tumours can, however, be challenging and there is a lack of immunohistochemical markers to prove their histogenic origin. To identify possible PNST markers and to further characterize their histogenic origin we compared histologically well-defined canine fibrosarcomas and PNSTs by cDNA microarray analysis. Forty-five annotated gene products were significantly differentially expressed between both tumour types. Seven of these gene products, known to be specifically expressed in neuroectodermal tissues, had higher expression levels in PNSTs: FMN2, KIF1B, GLI1, ROBO1, NMUR2, DOK4 and HMG20B. Conversely, eight genes associated with carcinogenesis had higher expression in fibrosarcomas: FHL2, PLAGL1, FNBP1L, BAG2, HK1, CSK and Cox5A. Comparison of the fibrosarcoma and PNST transcriptome therefore identified PNST phenotype-associated genes involved in neuroectodermal differentiation, which may be useful as diagnostic markers. Furthermore, the genes associated with the fibrosarcoma phenotype may serve as markers to differentiate fibrosarcomas from other tumour types. PMID:22818216

  14. Effect of acute high-intensity resistance exercise on optic nerve sheath diameter and ophthalmic artery blood flow pulsatility.

    PubMed

    Lefferts, W K; Hughes, W E; Heffernan, K S

    2015-12-01

    Exertional hypertension associated with acute high-intensity resistance exercise (RE) increases both intravascular and intracranial pressure (ICP), maintaining cerebrovascular transmural pressure. Carotid intravascular pressure pulsatility remains elevated after RE. Whether ICP also remains elevated after acute RE in an attempt to maintain the vessel wall transmural pressure is unknown. Optic nerve sheath diameter (ONSD), a valid proxy of ICP, was measured in 20 participants (6 female; 24 ± 4 yr, 24.2 ± 3.9 kg m(-)(2)) at rest (baseline), following a time-control condition, and following RE (5 sets, 5 repetition maximum bench press, 5 sets 10 repetition maximum biceps curls) using ultrasound. Additionally, intracranial hemodynamic pulsatility index (PI) was assessed in the ophthalmic artery (OA) by using Doppler. Aortic pulse wave velocity (PWV) was obtained from synthesized aortic pressure waveforms obtained via a brachial oscillometric cuff and carotid pulse pressure was measured by using applanation tonometry. Aortic PWV (5.2 ± 0.5-6.0 ± 0.7 m s(-1), P < 0.05) and carotid pulse pressure (45 ± 17-59 ± 19 mm Hg, P < 0.05) were significantly elevated post RE compared with baseline. There were no significant changes in ONSD (5.09 ± 0.7-5.09 ± 0.7 mm, P > 0.05) or OA flow PI (1.35 ± 0.2-1.38 ± 0.3, P > 0.05) following acute RE. In conclusion, during recovery from acute high-intensity RE, there are increases in aortic stiffness and extracranial pressure pulsatility in the absence of changes in ICP and flow pulsatility. These findings may have implications for alterations in cerebral transmural pressure and cerebral aneurysmal wall stress following RE. PMID:25739332

  15. Optic nerve sheath diameter on initial brain CT, raised intracranial pressure and mortality after severe TBI: an interesting link needing confirmation.

    PubMed

    Masquère, Pierre; Bonneville, Fabrice; Geeraerts, Thomas

    2013-01-01

    Optic nerve sheath diameter (ONSD) enlargement on initial computed tomography (CT) scan has been found to be associated with increased mortality after severe traumatic brain injury. This could offer the possibility to detect patients with raised intracranial pressure requiring urgent therapeutic interventions and/or invasive intracranial monitoring to guide the treatment. The method to measure ONSD using CT scan, however, needs further confirmation. Moreover, the link between ONSD enlargement on initial CT scan and raised intracranial pressure also needs to be confirmed by further studies. PMID:23751121

  16. Diagnostic Accuracy of PET/CT-Guided Percutaneous Biopsies for Malignant Peripheral Nerve Sheath Tumors in Neurofibromatosis Type 1 Patients

    PubMed Central

    Brahmi, Mehdi; Combemale, Patrick

    2015-01-01

    Background Malignant peripheral nerve sheath tumors (MPNST) are one of the most frequent causes of death in patients with neurofibromatosis type 1 (NF1). Early detection is crucial because complete surgical resection is the only curative treatment. It has been previously reported that an 18F-fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) image with a T/L (Tumor/Liver) SUVmax ratio > 1.5 provides a high negative predictive value; however, it is not specific enough to make a NF1-related MPNST diagnosis. A formal proof of malignant transformation from a histological analysis is necessary before surgical excision because the procedure can cause mutilation. The objective of the present work was to investigate the effectiveness of and complications associated with PET/CT-guided percutaneous biopsies for an NF1-related MPNST diagnosis. Methods PET/CT-guided percutaneous biopsy procedures performed on 26 NF1 patients with a clinical suspicion of MPNST and a suspect lesion from a PET/CT scan (T/L SUVmax ratio > 1.5) were retrospectively evaluated. The localization of the suspected malignant site was determined using PET/CT. A stereotactic (ultrasonic and CT control) core biopsy technique was used with a local anesthesia. Results The first PET/CT-guided percutaneous biopsies enabled a pathological diagnosis for all of the patients (no "inconclusive " results were obtained), and no secondary procedures were needed. Among the 26 patients, the histopathological results from the biopsy were malignant in 17 cases and benign (BPNST with atypical cells) in nine cases. No complications from the diagnostic procedure were observed. A surgical resection was performed in 18 patients (seven benign and 11 malignant biopsies), removing the fine needle biopsy scar. In addition, six locally advanced/metastatic MPNST were treated with chemo/radiotherapy, and two BPNST had no progression after a follow-up of 14 and 39 months, respectively. The PET

  17. 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

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

    PubMed

    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

  19. 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 Central

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

    2016-01-01

    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; Nf1flox/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; Nf1flox/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. PMID:26859681

  20. Surgical Management of Solitary Nerve Sheath Tumors of the Cervical Spine: A Retrospective Case Analysis Based on Tumor Location and Extension

    PubMed Central

    ABE, Junya; TAKAMI, Toshihiro; NAITO, Kentaro; YAMAGATA, Toru; ARIMA, Hironori; OHATA, Kenji

    2014-01-01

    Complete resection of spinal nerve sheath tumors (NSTs) does not always result in significant neurological deficit. The purpose of this retrospective case analysis was to discuss the optimal surgical strategy for spinal NST of the cervical spine. Twenty-four patients who underwent surgery for solitary cervical NST over the past decade were included in this retrospective study. Patients with neurofibromatosis or schwannomatosis were excluded. Seventeen of the 24 cases (70.8%) showed extradural dumbbell extension, most frequently at the C1 or C2 vertebral level. Neurological condition was assessed using the modified McCormick functional schema and sensory pain scale. Total removal of the tumor was achieved in 20 of 24 cases (83.3%). Staged surgery using combined anterior and posterior approaches was applied for 2 of 17 cases with extradural dumbbell extension. Tumor involvement with nerve root fibers critical for upper extremity function (C5–C8) was recognized in 6 of 24 cases (25.0%), with complete resection in all 6 cases. Final assessment of neurological function revealed satisfactory or acceptable recovery in all 6 patients. Spinal NSTs with extradural dumbbell extension are a common condition in the cervical spine. Complete removal of spinal NST of the cervical spine may carry a risk of permanent neurological deficit, but such sequelae appeared to be the exception in the present case analysis. A radical and safe surgical strategy, including staged surgery combining anterior and posterior approaches, should be tailored to the individual case. PMID:25367583

  1. Surgical management of solitary nerve sheath tumors of the cervical spine: a retrospective case analysis based on tumor location and extension.

    PubMed

    Abe, Junya; Takami, Toshihiro; Naito, Kentaro; Yamagata, Toru; Arima, Hironori; Ohata, Kenji

    2014-01-01

    Complete resection of spinal nerve sheath tumors (NSTs) does not always result in significant neurological deficit. The purpose of this retrospective case analysis was to discuss the optimal surgical strategy for spinal NST of the cervical spine. Twenty-four patients who underwent surgery for solitary cervical NST over the past decade were included in this retrospective study. Patients with neurofibromatosis or schwannomatosis were excluded. Seventeen of the 24 cases (70.8%) showed extradural dumbbell extension, most frequently at the C1 or C2 vertebral level. Neurological condition was assessed using the modified McCormick functional schema and sensory pain scale. Total removal of the tumor was achieved in 20 of 24 cases (83.3%). Staged surgery using combined anterior and posterior approaches was applied for 2 of 17 cases with extradural dumbbell extension. Tumor involvement with nerve root fibers critical for upper extremity function (C5-C8) was recognized in 6 of 24 cases (25.0%), with complete resection in all 6 cases. Final assessment of neurological function revealed satisfactory or acceptable recovery in all 6 patients. Spinal NSTs with extradural dumbbell extension are a common condition in the cervical spine. Complete removal of spinal NST of the cervical spine may carry a risk of permanent neurological deficit, but such sequelae appeared to be the exception in the present case analysis. A radical and safe surgical strategy, including staged surgery combining anterior and posterior approaches, should be tailored to the individual case. PMID:25367583

  2. Angiomatous meningioma in Sturge-Weber syndrome.

    PubMed

    Ahmed, Zubair; Prayson, Richard A

    2015-06-01

    We report a case of an intraventricular angiomatous meningioma arising in a 3-year-old boy diagnosed with Sturge-Weber syndrome (SWS) who presented with intractable epilepsy and right-sided hemiparesis. He underwent surgical resection of the epileptogenic focus. Histologic sections showed the typical findings of SWS accompanied by adjacent mild focal cortical dysplasia (International League Against Epilepsy Type Ib pattern; Palmini et al. Type IA). A small intraventricular mass, which was incidentally noted on imaging studies, was also excised. The mass showed a prominent venous vasculature with intermixed meningothelial cells, consistent with an angiomatous meningioma World Health Organization Grade I. SWS is a rare, sporadically occurring disorder marked by a port wine stain (hemangioma of the skin) arising in the distribution of the trigeminal nerve accompanied by an angiomatous proliferation in the leptomeninges. The underlying cortex often shows prominent dystrophic mineralization and gliosis. Patients often present with seizures and may require surgical resection when seizures prove to be pharmacoresistant. Meningiomas in SWS are a rare occurrence (only one known previously reported case) and angiomatous meningioma in SWS has never been described. The literature is briefly reviewed and the pathogenesis of hemangiomas in SWS and its implication in angiomatous meningioma is discussed. PMID:25766367

  3. Checkpoint inhibition in meningiomas.

    PubMed

    Bi, Wenya Linda; Wu, Winona W; Santagata, Sandro; Reardon, David A; Dunn, Ian F

    2016-06-01

    Meningiomas are increasingly appreciated to share similar features with other intra-axial central nervous system neoplasms as well as systemic cancers. Immune checkpoint inhibition has emerged as a promising therapy in a number of cancers, with durable responses of years in a subset of patients. Several lines of evidence support a role for immune-based therapeutic strategies in the management of meningiomas, especially high-grade subtypes. Meningiomas frequently originate juxtaposed to venous sinuses, where an anatomic conduit for lymphatic drainage resides. Multiple populations of immune cells have been observed in meningiomas. PD-1/PD-L1 mediated immunosuppression has been implicated in high-grade meningiomas, with association between PD-L1 expression with negative prognostic outcome. These data point to the promise of future combinatorial therapeutic strategies in meningioma. PMID:27197540

  4. Descriptive epidemiology of primary tumors of the brain, cranial nerves and cranial meninges in Los Angeles County.

    PubMed

    Preston-Martin, S

    1989-01-01

    This report presents data on the distribution of 8,612 cases of primary tumors of the brain, cranial nerves and cranial meninges (both benign and malignant) diagnosed among residents of Los Angeles County from 1972 to 1985. Incidence rates of gliomas, meningiomas, nerve sheath tumors and all histologic types combined are presented for specific age, sex and ethnic groups. At all ages, the highest incidence is seen for gliomas among men. Meningioma rates are higher among women than men in every ethnic group. In both sexes, glioma rates are highest among whites, and meningioma rates are highest among blacks. Asians have the lowest rates of both types of tumors. Proportional incidence ratios are elevated among those born in Eastern Europe, Southern Europe and the Middle East and among Jewish residents of Los Angeles County. A clear trend of increasing glioma incidence with increasing social class is seen among males. An analysis among white men aged 25-64 by occupation and industry at the time of diagnosis supports several previously published findings. A glioma excess is evident among workers in the aircraft industry. Workers in the petroleum industry and the rubber and plastics industry have an excess of meningiomas. Occupational groups at excess risk include dentists who have an increased risk of all types of brain tumors and electricians whose excess risk is limited to gliomas. PMID:2586698

  5. Lectin histochemistry of normal and neoplastic peripheral nerve sheath. 2. Lectin binding patterns of schwannoma and neurofibroma.

    PubMed

    Matsumura, K; Nakasu, S; Nioka, H; Handa, J

    1993-01-01

    Lectin binding patterns of 31 schwannomas and 6 neurofibromas were examined using 12 lectins, and the results were compared with those of normal peripheral nerves. Tumors obtained from 10 cases of neurofibromatosis and 4 recurrent schwannomas were included. Changes of glycoconjugates were observed in association with a neoplastic transformation of Schwann cells; Arachis hypogaea (PNA) staining after neuraminidase treatment seen in normal Schwann cells was reduced in schwannoma of Antoni type A, and bindings with Glycine max (SBA) and Helix pomatia (HPA) after sialic acid removal, which were not seen in normal Schwann cells, appeared in schwannoma cells. Intensities of staining of tumor cells with each lectin were higher in Antoni type B than those in Antoni type A. No differences in lectin binding patterns were observed between schwannomas in patients with neurofibromatosis or recurrent schwannomas and ordinary, primary schwannomas in patients without stigmata of neurofibromatosis. Lectin binding patterns of Schwann cells and perineurial cells in neurofibroma were almost similar to those in normal peripheral nerves with an exception of faint stain of Schwann cells with HPA after neuraminidase pretreatment. This result suggests differences in extent of differentiation between schwannoma cells and neoplastic Schwann cells in neurofibroma. Specific PNA binding to perineurial cells in neurofibroma indicates the significance of this lectin as a marker of these cells. PMID:8310811

  6. Intracerebral malignant peripheral nerve sheath tumor in a child with neurofibromatosis Type 1 and middle cerebral artery aneurysm treated with endovascular coil embolization.

    PubMed

    Ellis, Michael J; Cheshier, Samuel; Sharma, Sunjay; Armstrong, Derek; Hawkins, Cynthia; Bouffet, Eric; Rutka, James T; Taylor, Michael D

    2011-10-01

    Among the neoplastic conditions that affect patients with neurofibromatosis Type 1 (NF1) are malignant peripheral nerve sheath tumors (MPNSTs), which typically arise from peripheral nerves of the limbs, trunk, and lumbar and brachial plexuses. Ionizing radiation is an established risk factor for MPNST development, especially in susceptible patients such as those with NF1. Patients with NF1 are also at risk for intracranial aneurysms, which are increasingly being successfully managed with endovascular therapies. The authors describe the case of a 9-year-old, previously healthy girl who presented in extremis with a right frontal intracerebral hemorrhage resulting from a ruptured right middle cerebral artery (MCA) trifurcation aneurysm. Following urgent decompressive craniectomy, the patient underwent endovascular coil embolization of the MCA aneurysm without complication. Given her mother's history of NF1, the child underwent genetic testing, which disclosed signs positive for NF1. The patient recovered well, but follow-up MR imaging and MR angiography performed at 14 months demonstrated a large frontotemporal mass encasing the right MCA trifurcation. The patient underwent frontotemporal craniotomy and subtotal resection of the mass, which was histologically found to be an intracranial MPNST. The patient received chemotherapy and focal radiation therapy and remains alive at 6 months postresection. To the authors' knowledge, this represents the only known case of intracranial neoplasm arising in the region of an intracranial aneurysm repaired by endovascular coil embolization. While patients with NF1 represent a population with genetic susceptibility to radiation-induced tumors, the pathogenesis of intracerebral MPNSTs remains poorly understood. PMID:21961539

  7. Histological and immunohistochemical study of 30 cases of canine meningioma.

    PubMed

    Montoliu, P; Añor, S; Vidal, E; Pumarola, M

    2006-11-01

    This report describes the incidence, location and histopathological and immunohistochemical features of 30 canine meningiomas, of which 22 were intracranial, three were retrobulbar and five were located in the spinal canal. Nine types of meningioma were diagnosed: transitional (9), meningothelial (5), psammomatous (3), anaplastic (3), fibroblastic (2), angioblastic (2), papillary (2), microcystic (1) and meningiomas arising from the optic nerve (3). One of the optic nerve tumours had a granular cell component. All tumours were examined immunohistochemically with antibodies against vimentin, S100, neuron-specific enolase (NSE), cytokeratin (CK) and glial fibrillary acidic protein (GFAP). Vimentin was demonstrated in all tumours, and concentric whorls of cells showed more intense labelling than did bundles of fibroblastic cells. S100 labelling was detected in all tumours except a single angioblastic meningioma. The intensity of labelling for S100 was lower than that for vimentin, and bundles of fibroblastic cells showed particularly strong positivity. NSE labelling was highly variable, but most tumours displayed moderate positivity. CK expression was observed in five of the 30 meningiomas, and was stronger in areas of microcystic differentiation. Most of the tumours were GFAP-negative, but two fibroblastic meningiomas were strongly positive. PMID:17049358

  8. Malignant Peripheral Nerve Sheath Tumor Invasion Requires Aberrantly Expressed Epidermal Growth Factor (EGF) Receptors and is Variably Enhanced by Multiple EGF Family Ligands

    PubMed Central

    Byer, Stephanie J.; Brossier, Nicole M.; Peavler, Lafe T.; Eckert, Jenell M.; Watkins, Stacey; Roth, Kevin A.; Carroll, Steven L.

    2013-01-01

    Aberrant epidermal growth factor receptor (EGFR) expression promotes the pathogenesis of malignant peripheral nerve sheath tumors (MPNSTs), the most common malignancy associated with neurofibromatosis type 1, but the mechanisms by which EGFR expression promotes MPNST pathogenesis are poorly understood. We hypothesized that inappropriately expressed EGFRs promote MPNST invasion and found that these kinases are concentrated in MPNST invadopodia in vitro. EGFR knockdown inhibited the migration of unstimulated MPNST cells in vitro and exogenous EGF further enhanced MPNST migration in a substrate-specific manner, promoting migration on laminin and, to a lesser extent, collagen. Thus, in this setting, EGF acts as a chemotactic factor. We also found that the 7 known EGFR ligands (EGF, betacellulin, epiregulin, heparin-binding EGF, transforming growth factor α [TGFα], amphiregulin, and epigen) variably enhanced MPNST migration in a concentration-dependent manner, with TGFα being particularly potent. With the exception of epigen, these factors similarly promoted the migration of non-neoplastic Schwann cells. Although transcripts encoding all 7 EGFR ligands were detected in human MPNST cells and tumor tissues, only TGFα was consistently overexpressed and was found to colocalize with EGFR in situ. These data indicate that constitutive EGFR activation, potentially driven by autocrine or paracrine TGFα signaling, promotes the aggressive invasive behavior characteristic of MPNSTs. PMID:23399900

  9. Induction of apoptosis in Neurofibromatosis Type 1 malignant peripheral nerve sheath tumor cell lines by a combination of novel farnesyl transferase inhibitors and lovastatin

    PubMed Central

    Wojtkowiak, Jonathan W.; Fouad, Farid; LaLonde, Daniel T.; Kleinman, Miriam D.; Gibbs, Richard A.; Reiners, John J.; Borch, Richard F.; Mattingly, Raymond R.

    2013-01-01

    Neurofibromatosis Type 1 (NF1) is a genetic disorder that is driven by the loss of neurofibromin (Nf) protein function. Nf contains a Ras GTPase activating domain (Ras-GAP), which directly regulates Ras signaling. Numerous clinical manifestations are associated with the loss of Nf and increased Ras activity. Ras proteins must be prenylated in order to traffic and functionally localize with target membranes. Hence, Ras is a potential therapeutic target for treating NF1. We have tested the efficacy of two novel farnesyl transferase inhibitors (FTI), 1 and 2, alone or in combination with lovastatin, on two NF1 malignant peripheral nerve sheath tumor (MPSNT) cell lines, NF90-8 and ST88-14. Single treatments of 1, 2, or lovastatin had no effect on MPNST cell proliferation. However, low micromolar combinations of 1 or 2 with lovastatin (FTI/lovastatin) reduced Ras prenylation in both MPNST cell lines. Further, this FTI/lovastatin combination treatment reduced cell proliferation and induced an apoptotic response as shown by morphological analysis, pro-caspase-3/-7 activation, loss of mitochondrial membrane potential, and accumulation of cells with sub G1 DNA content. Little to no detectable toxicity was observed in normal rat Schwann cells following FTI/lovastatin combination treatment. These data support the hypothesis that combination FTI plus lovastatin therapy may be a potential treatment for NF1 MPNSTs. PMID:18367665

  10. Whole-exome sequencing of breast cancer, malignant peripheral nerve sheath tumor and neurofibroma from a patient with neurofibromatosis type 1.

    PubMed

    McPherson, John Richard; Ong, Choon-Kiat; Ng, Cedric Chuan-Young; Rajasegaran, Vikneswari; Heng, Hong-Lee; Yu, Willie Shun-Shing; Tan, Benita Kiat-Tee; Madhukumar, Preetha; Teo, Melissa Ching-Ching; Ngeow, Joanne; Thike, Aye-Aye; Rozen, Steven George; Tan, Puay-Hoon; Lee, Ann Siew-Gek; Teh, Bin-Tean; Yap, Yoon-Sim

    2015-12-01

    Neurofibromatosis type 1 (NF1) is a genetic disorder characterized by the development of multiple neurofibromas, cafe-au-lait spots, and Lisch nodules. Individuals with NF1 are at increased risk of developing various tumors, such as malignant peripheral nerve sheath tumor (MPNST), pheochromocytoma, leukemia, glioma, rhabdomyosarcoma, and breast cancer. Here, we describe the exome sequencing of breast cancer, MPNST, and neurofibroma from a patient with NF1. We identified a germline mutation in the NF1 gene which resulted in conversion of leucine to proline at amino acid position 847. In addition, we showed independent somatic NF1 mutations in all the three tumors (frameshift insertion in breast cancer (p.A985fs), missense mutation in MPNST (p.G23R), and inframe deletion in dermal neurofibroma (p.L1876del-Inf)), indicating that a second hit in NF1 resulting in the loss of function could be important for tumor formation. Each tumor had a distinct genomic profile with mutually exclusive mutations in different genes. Copy number analysis revealed multiple copy number alterations in the breast cancer and the MPNST, but not the benign neurofibroma. Germline loss of chromosome 6q22.33, which harbors two potential tumor suppressor genes, PTPRK and LAMA2, was also identified; this may increase tumor predisposition further. In the background of NF1 syndrome, although second-hit NF1 mutation is critical in tumorigenesis, different additional mutations are required to drive the formation of different tumors. PMID:26432421

  11. Wilms’ Tumor Gene 1 (WT1) Silencing Inhibits Proliferation of Malignant Peripheral Nerve Sheath Tumor sNF96.2 Cell Line

    PubMed Central

    Parenti, Rosalba; Cardile, Venera; Graziano, Adriana Carol Eleonora; Parenti, Carmela; Venuti, Assunta; Bertuccio, Maria Paola; Furno, Debora Lo; Magro, Gaetano

    2014-01-01

    Wilms’ tumor gene 1 (WT1) plays complex roles in tumorigenesis, acting as tumor suppressor gene or an oncogene depending on the cellular context. WT1 expression has been variably reported in both benign and malignant peripheral nerve sheath tumors (MPNSTs) by means of immunohistochemistry. The aim of the present study was to characterize its potential pathogenetic role in these relatively uncommon malignant tumors. Firstly, immunohistochemical analyses in MPNST sNF96.2 cell line showed strong WT1 staining in nuclear and perinuclear areas of neoplastic cells. Thus, we investigated the effects of silencing WT1 by RNA interference. Through Western Blot analysis and proliferation assay we found that WT1 knockdown leads to the reduction of cell growth in a time- and dose-dependent manner. siWT1 inhibited proliferation of sNF96.2 cell lines likely by influencing cell cycle progression through a decrease in the protein levels of cyclin D1 and inhibition of Akt phosphorylation compared to the control cells. These results indicate that WT1 knockdown attenuates the biological behavior of MPNST cells by decreasing Akt activity, demonstrating that WT1 is involved in the development and progression of MPNSTs. Thus, WT1 is suggested to serve as a potential therapeutic target for MPNSTs. PMID:25474318

  12. 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

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

    PubMed

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

    2016-08-01

    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

  14. 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. PMID:19333184

  15. Nerve Sheath Tumors in Neurofibromatosis Type 1: Assessment of Whole-Body Metabolic Tumor Burden Using F-18-FDG PET/CT

    PubMed Central

    Salamon, Johannes; Papp, László; Tóth, Zoltán; Laqmani, Azien; Apostolova, Ivayla; Adam, Gerhard; Mautner, Victor F.; Derlin, Thorsten

    2015-01-01

    Purpose To determine the metabolically active whole-body tumor volume (WB-MTV) on F-18-fluorodeoxyglucose positron emission tomography/computed tomography (F-18-FDG PET/CT) in individuals with neurofibromatosis type 1 (NF1) using a three-dimensional (3D) segmentation and computerized volumetry technique, and to compare PET WB-MTV between patients with benign and malignant peripheral nerve sheath tumors (PNSTs). Patients and Methods Thirty-six NF1 patients (18 patients with malignant PNSTs and 18 age- and sex-matched controls with benign PNSTs) were examined by F-18-FDG PET/CT. WB-MTV, whole-body total lesion glycolysis (WB-TLG) and a set of semi-quantitative imaging-based parameters were analyzed both on a per-patient and a per-lesion basis. Results On a per-lesion basis, malignant PNSTs demonstrated both a significantly higher MTV and TLG than benign PNSTs (p < 0.0001). On a per-patient basis, WB-MTV and WB-TLG were significantly higher in patients with malignant PNSTs compared to patients with benign PNSTs (p < 0.001). ROC analysis showed that MTV and TLG could be used to differentiate between benign and malignant tumors. Conclusions WB-MTV and WB-TLG may identify malignant change and may have the potential to provide a basis for investigating molecular biomarkers that correlate with metabolically active disease manifestations. Further evaluation will determine the potential clinical impact of these PET-based parameters in NF1. PMID:26625155

  16. Malignant Peripheral Nerve Sheath Tumor Is a Challenging Diagnosis: A Systematic Pathology Review, Immunohistochemistry, and Molecular Analysis in 160 Patients From the French Sarcoma Group Database.

    PubMed

    Le Guellec, Sophie; Decouvelaere, Anne-Valérie; Filleron, Thomas; Valo, Isabelle; Charon-Barra, Céline; Robin, Yves-Marie; Terrier, Philippe; Chevreau, Christine; Coindre, Jean-Michel

    2016-07-01

    An accurate histopathologic diagnosis is essential for an adequate treatment of soft tissue sarcomas. The diagnosis of malignant peripheral nerve sheath tumor (MPNST) can be complex, particularly outside the neurofibromatosis type 1 (NF1) context. MPNST is a rare malignancy, and due to the lack of specific histologic criteria, several differential diagnoses must be considered. A total of 350 patients diagnosed with MPNST (from 1990 to 2013) were retrieved from the French sarcoma network (RRePS) and the Conticabase (Connective Tissue Cancer Network database). Tumor samples were available for 160 cases (45.2%). Pathology review, immunohistochemistry (IHC), and molecular analysis (when dealing with a monomorphic sarcoma) were systematically performed. Patient, tumor, and treatment characteristics were evaluated to identify prognostic factors for the definitive primary MPNST (n=106) cohort. Twenty-nine tumors (18.1%) initially diagnosed as MPNST were reclassified on the basis of histologic review, IHC, and molecular analysis. Patients with NF1 disease comprised 64% of the remaining cohort. The 5-year overall survival for patients from the entire cohort was 47%, 34.8% for NF1 patients, and 68.5% for patients without NF1 disease, making NF1 syndrome an independent poor prognostic factor of survival. Positive margins and lack of radiation therapy were independent predictors of local recurrence. The Fédération Nationale des Centres de Lutte Contre le Cancer tumor grade was an independent prognostic indicator of metastasis. Given the therapeutic implications of a misdiagnosis, the systematic pathology review, IHC, and molecular analysis (when dealing with monomorphic sarcoma) strategy allowed reclassification of 20% of cases, mainly the sporadic MPNSTs. PMID:27158754

  17. Sec6/8 regulates Bcl-2 and Mcl-1, but not Bcl-xl, in malignant peripheral nerve sheath tumor cells.

    PubMed

    Tanaka, Toshiaki; Kikuchi, Noriaki; Goto, Kaoru; Iino, Mitsuyoshi

    2016-05-01

    Sec6 and Sec8, which are components of the exocyst complex, has been concerned with various roles independent of its role in secretion, such as cell migration, invadopodia formation, cytokinesis, glucose uptake, and neural development. Given the vital roles of the exocyst complex in cellular and developmental processes, the disruption of its function may be closely related to various diseases such as cancer, diabetes, and neuronal disorders. Malignant peripheral nerve sheath tumors (MPNSTs) have high malignant potential and poor prognosis because of aggressive progression and metastasis. To date, no chemotherapeutic agents have been validated for MPNSTs treatment because how MPNSTs are resistant to chemotherapeutic agents remains unknown. This study demonstrates that combination of doxorubicin and sorafenib induces apoptosis in MPNST cells through downregulation of B cell lymphoma protein 2 (Bcl-2), Bcl-2-related protein long form of Bcl-x (Bcl-xl), and myeloid cell leukemia 1 (Mcl-1). Moreover, both Sec6 and Sec8 levels decreased after treatment with doxorubicin and sorafenib and were found to be associated with Bcl-2 and Mcl-1 expressions, but not Bcl-xl. Although Sec8 was found to be involved in the regulation of both Bcl-2 and Mcl-1 at the mRNA level, Sec6 regulated Bcl-2 at the mRNA level and the binding affinity of F-box and WD repeat domain containing 7 and Mcl-1, thereby controlling Mcl-1 at the protein level. Bcl-2 or Mcl-1 mRNA suppression by Sec6 or Sec8 depletion resulted in significant changes in nuclear factor-kappa B, cAMP response element, and p53 transcriptional activity. These results suggest that Sec6 and Sec8 are therapeutic target molecules in MPNST. PMID:26892009

  18. Giant malignant peripheral nerve sheath tumor with cauda equina syndrome and subarachnoid hemorrhage: Complications in a case of type 1 neurofibromatosis.

    PubMed

    Patil, Tushar B; Singh, Maneesh Kumar; Lalla, Rakesh

    2015-01-01

    Type 1 neurofibromatosis (NF1), which mainly involves ectodermal tissue arising from the neural crest, can increase the risk of developing malignant peripheral nerve sheath tumors (MPNSTs), soft tissue sarcomas and subarachnoid hemorrhage. We describe a patient with neurofibromatosis type 1 who developed soft tissue sarcoma, MPNST, and subarachnoid hemorrhage. A 22-year-old male reported right focal seizures consequence to severe headache. He had a weakness in both legs, could walk only with the support of a stick for the last 3 months and suffered from constipation and intermittent urinary retention for the past 1 week. The patient had a history of swelling in the back of left thigh for which surgical resection was done 6 months back. Cutaneous examination revealed multiple nodules of varying sizes all over the body, along with many café-au-lait spots and Lisch nodule in iris. Patient had weakness in bilateral hip abduction, extension, knee flexion, extension and ankle dorsiflexion and plantiflexion. Bilateral ankle reflexes were absent while other deep tendon reflexes were sub-optimal. A noncontrast computed tomography brain indicated subarachnoid hemorrhage in left perisylvian region. Ultrasound of left thigh showed a hypoechoic solid lesion in the posterior aspect of left thigh in muscle plane. Histopathology of the lesion following resection showed features suggestive of a low-grade pleomorphic rhabdomyosarcoma. Histology of cutaneous nodules was consistent with neurofibroma. Magnetic resonance imaging of the lumbosacral spine demonstrated a tumor arising from cauda equina. Histopathological examination of the tumor suggested high-grade MPNST. Unfortunately, the patient's MPNST was inoperable, and he received palliative radiotherapy for local control of the disease. The care of a patient with neurofibromatosis requires a comprehensive multisystem evaluation. MPNST occurs in 8-13% patients with neurofibromatosis. Early diagnosis and surgical resection are key

  19. 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

  20. Loss of H3K27 tri-methylation is a diagnostic marker for Malignant Peripheral Nerve Sheath Tumors and an indicator for an inferior survival

    PubMed Central

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

    2016-01-01

    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 to 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 favours 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 by

  1. 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

  2. Surgical Treatment for Falcotentorial Meningiomas.

    PubMed

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

    2016-07-01

    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

  3. 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

  4. 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

  5. Natural history of multiple meningiomas

    PubMed Central

    Wong, Ricky H.; Wong, Andrew K.; Vick, Nicholas; Farhat, Hamad I.

    2013-01-01

    Background: Asymptomatic solitary meningiomas are typically managed with clinical and radiographic follow-up. Multiple meningiomas represents a clinical entity distinct from solitary meningiomas and can be sporadic, radiation-induced, associated with neurofibromatosis, or exhibit other familial inheritance. The growth rate for multiple meningiomas is not known and therefore management of these complicated patients can be difficult. Methods: A retrospective chart review was performed on 12 patients with a total of 55 meningiomas. Patients with neurofibromatosis were not included. Serial enhanced magnetic resonance imaging was used to determine tumor growth rates. Treatment history was also reviewed and included for analysis. Results: Analysis of all 55 tumors demonstrated an average rate of growth of 0.46 cm3/year (range: −0.57-2.94 cm3/year). In the 23 tumors that received no treatment, the average rate of growth was 0.34 cm3/year (range: −0.03-1.8 cm3/year). Ten of the 23 tumors that received no treatment had no history of cranial irradiation. This group demonstrated a growth rate of 0.44 cm3/year (range: −0.01-1.8 cm3/year). Linear regression analysis did not yield any significant relationship between tumor burden and rates of growth. Conclusion: Tumor growth rates in patients with multiple meningiomas did not appear to be higher than reported rates for incidentally found solitary meningiomas. As such, asymptomatic multiple meningioma patients should be managed with clinical and radiographic follow-up. PMID:23776757

  6. Rhabdoid Meningioma: Report of Two Cases

    PubMed Central

    Reddy, Ch. Karunakar; Rao, A. Divakar; Ballal, Chandra K.

    2015-01-01

    Rhabdoid meningioma is an uncommon anaplastic variant of meningioma. We describe the clinicoradiological and histomorphological features of two such cases of meningioma, with a brief review of literature. One case in a 9-year-old girl, presented initially as an atypical meningioma in the right fronto-parietal region, which on recurrence 18 months later, evolved into a rhabdoid meningioma. The second case in a 33-year-old male was located in the right parieto-occipital region. PMID:25859490

  7. Ulnar nerve dysfunction

    MedlinePlus

    ... surface of the body where it crosses the elbow. The damage destroys the nerve covering ( myelin sheath) ... be caused by: Long-term pressure on the elbow An elbow fracture or dislocation Temporary pain and ...

  8. Bevacizumab in Treating Patients With Recurrent or Progressive Meningiomas

    ClinicalTrials.gov

    2016-02-26

    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

  9. Unilateral vocal cord palsy and dysphagia: an unusual presentation of cerebellopontine angle meningioma.

    PubMed

    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

  10. 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

  11. Involvement of epimutations in meningioma.

    PubMed

    Venza, Mario; Visalli, Maria; Beninati, Concetta; Catalano, Teresa; Biondo, Carmelo; Teti, Diana; Venza, Isabella

    2015-07-01

    Epimutations are heritable and reversible cell markers, which can influence cell function going beyond the effects of DNA mutations. They result from multiple and coordinated mechanisms able to modulate gene expression. Regarding the significance of epigenetics in meningioma, few and somehow contradictory results are available, although promising information has been obtained. Here we highlight the most recent advances about the impact of DNA methylation, histone modifications, and microRNA regulation on meningioma development as well as the interplay between genetic and epigenetic alterations. Data indicate that epigenetics can help to identify novel candidate genes for the management and treatment of meningioma. PMID:25930103

  12. Gravitoelectromagnetic sheath

    NASA Astrophysics Data System (ADS)

    Goutam, H. P.; Karmakar, P. K.

    2015-06-01

    In this paper we propose a gravito-electro-magnetic sheath (GEMS) model to explore the equilibrium properties of the solar plasma system. It describes the solar interior plasma (SIP) on the bounded scale and the solar wind plasma (SWP) on the unbounded scale from the viewpoint of plasma-based theory. This differs from the previously reported gravito-electrostatic sheath (GES) model employed to precisely define the solar surface boundary (SSB) on the fact that the present investigation incorporates variable temperature, magnetic field and collisional processes on the solar plasma flow dynamics. We show that the included parameters play important roles in the solar plasma dynamics. We demonstrate that the SSB location shifts outward as a result of the magnetic field by 14 % in comparison with that predicted by the GES model. As a consequence of the interaction of the plasma with magnetic field, the width of the sheath broadens by 25 % in comparison with the GES model predicted value. This physically means that the magnetic field decreases the distribution of the tiny (inertialess) electrons relative to the massive (inertial) ions, which in turn increases the confining wall potential value resulting in the increased width. Besides, the sonic point moves inward by 8 % as a result of collisions in the SIP that leads to rapid acceleration. Here, collisional dynamics plays an important role in the conversion process of the electron thermal energy into the bulk plasma flow energy. An interesting feature of continuous and smooth transition of the electric current density from the SIP to the SWP (with finite positive divergence on both the scales) through the SSB under inhomogeneous temperature distribution is also reported. Finally, the analyses may be applied to understand the realistic equilibrium dynamics of stellar plasmas never addressed before within the earlier GES framework like establishment of current-field correlation, properties of the slow solar wind and its

  13. 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

  14. KINOMIC ALTERATIONS IN ATYPICAL MENINGIOMA

    PubMed Central

    Anderson, Joshua C.; Taylor, Robert B.; Fiveash, John B.; de Wijn, Rik; Gillespie, G. Yancey; Willey, Christopher D.

    2015-01-01

    Background We sought to profile Atypical Meningioma in a high-throughput manner to better understand the altered signaling within these tumors and specifically the kinases altered in recurrent atypical meningioma. Kinomic Profiles could be used to identify prognostic biomarkers for responders/non-responders to classify future patients that are unlikely to benefit from current therapies. Directly these results could be used to identify drug-actionable kinase targets as well. Methods Peptide-substrate microarray kinase activity analysis was conducted with a PamStation®12 analyzing the tyrosine kinome in each tumor kinetically against ~144 target peptides. These data were then analyzed relative to clinical outcome (e.g., tumor recurrence). Results 3 major clusters of atypical meningiomas were identified with highly variant peptides primarily being targets of EGFR family, ABL, BRK and BMX kinases. Kinomic analysis of recurrent atypical meningiomas indicated patterns of increased phosphorylation of BMX, TYRO3 and FAK substrates as compared to non-recurrent tumors. Conclusion The atypical meningiomas profiled here exhibited molecular sub-clustering that may have phenotypic corollaries predictive of outcome. Recurrent tumors had increases in kinase activity that may predict resistance to current therapies, and may guide selection of directed therapies. Taken together these data further the understanding of kinomic alteration in atypical meningioma, and the processes that may not only mediate recurrence, but additionally may identify kinase targets for intervention. PMID:27158663

  15. Medical treatment of recurrent meningiomas.

    PubMed

    Chamberlain, Marc C; Barnholtz-Sloan, Jill S

    2011-10-01

    Meningiomas are the second most common primary brain tumor and are primarily treated with surgery (with or without embolization) and radiotherapy. Increasingly today, meningiomas undergo multiple resections and two radiotherapy treatments (either stereotactic or conventional external beam) before consideration for hormonal, chemotherapy or targeted therapy. The failure of hormonal and cytotoxic chemotherapy in the treatment of recurrent meningioma and increasing understanding of potential molecular targets in meningioma has resulted in multiple studies utilizing single-agent targeted therapy directed at biologically relevant signaling pathways, such as somatostatin (Sandostatin(®) LAR, SOM230c), PDGF (imatinib), EGF (erlotinib) and VEGF (sunitinib and vatalanib). Early results using a targeted approach have been modest at best and are often associated with significant toxicity. Consequently and at present, the brain tumor guidelines recognize only three medical therapies for inoperable and radiation-refractory meningiomas: hydroxyurea, IFN-α and Sandostatin LAR, a somatostatin analogue. Clearly, there remains an unmet need in neuro-oncology with respect to the medical treatment of recurrent meningiomas. PMID:21955199

  16. 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

  17. 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. PMID:27068012

  18. Incidental finding of meningioma on bone scintigraphy.

    PubMed

    Thakorlal, A; Wong, D C; Anderson, R J

    2005-06-01

    An incidental finding of an intracranial posterior fossa meningioma detected by bone scintigraphy is presented. Most of the published literature on the diagnosis of meningioma is on the use of CT and MRI. There is limited published literature on the detection of meningioma with bone scintigraphy. PMID:15932468

  19. Chordoid meningioma, part of a multiple intracranial meningioma: a case report & review.

    PubMed

    Sriram, Prabu Rau

    2013-07-01

    Chordoid meningioma, classified as atypical meningioma according to the World Health Organisation (WHO) classification, is a rare subtype, which represents only 0.5% of all meningiomas and is associated with a high incidence of recurrence. Multiple intracranial meningiomas are rare in non-neurofibromatosis patients. We present a female patient with both of these rare types of meningioma. The patient presented with two concurrent intracranial meningiomas, with one a meningotheliomatous subtype and the other a chordoid meningioma. Given the wide array of histological differential diagnoses in chordoid meningioma, immunohistochemistry has a significant role to play in differentiating them. Recurrence in chordoid meningioma can be generally predicted based on the extent of resection, the percentage of chordoid element, and proliferation indices. PMID:24044003

  20. Management of Atypical and Anaplastic Meningiomas.

    PubMed

    Buttrick, Simon; Shah, Ashish H; Komotar, Ricardo J; Ivan, Michael E

    2016-04-01

    Meningiomas are the most prevalent primary tumor of central nervous system origin and, although most neoplasms are benign, a small proportion exemplifies an aggressive profile characterized by high recurrence rates, pleomorphic histology, and overall resistance to standard treatment. Standard initial therapy for malignant meningiomas includes maximal safe surgical resection followed by focal radiation in certain cases. The role for chemotherapy during recurrence of these aggressive meningiomas is less clear. Prognosis is poor and recurrence of malignant meningiomas is high. This article provides an overview of atypical and anaplastic malignant meningiomas, their treatment, and ongoing research for more effective treatments. PMID:27012388

  1. Surgical Resectability of Skull Base Meningiomas.

    PubMed

    Goto, Takeo; Ohata, Kenji

    2016-07-15

    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

  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. [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. PMID:20429360

  4. 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.

  5. Presentation and Patterns of Late Recurrence of Olfactory Groove Meningiomas

    PubMed Central

    Snyder, William E.; Shah, Mitesh V.; Weisberger, Edward C.; Campbell, Robert L.

    2000-01-01

    The objective of this article is to present the recurrence pattern of olfactory groove meningiomas after surgical resection. Four patients, one female and three males, with surgically resected olfactory groove meningiomas presented with tumor recurrence. All patients underwent resection of an olfactory groove meningioma and later presented with recurrent tumors. The mean age at initial diagnosis was 47 years. All presented initially with vision changes, anosmia, memory dysfunction, and personality changes. Three patients had a preoperative MRI scan. All patients had a craniotomy, with gross total resection achieved in three, and 90% tumor removal achieved in the fourth. Involved dura was coagulated, but not resected, in all cases. Three patients were followed with routine head CT scans postoperatively, and none was followed with MRI scan. The mean time to recurrence was 6 years. Three patients presented with recurrent visual deterioration, and one presented with symptoms of nasal obstruction. Postoperative CT scans failed to document early tumor recurrence, whereas MRI documented tumor recurrence in all patients. Tumor resection and optic nerve decompression improved vision in two patients and stabilized vision in two. Complete resection was not possible because of extensive bony involvement around the anterior clinoid and inferior to the anterior cranial fossa in all cases. Evaluation of four patients with recurrent growth of olfactory groove meningiomas showed the epicenter of recurrence to be inferior to the anterior cranial fossa, with posterior extension involving the optic canals, leading to visual deterioration. This location led to a delay in diagnosis in patients who were followed only with routine CT scans. Initial surgical procedures should include removal of involved dura and bone, and follow-up evaluation should include formal ophthalmologic evaluations and routine head MRI scans. ImagesFigure 1Figure 2Figure 3Figure 4Figure 5Figure 6Figure 7 PMID

  6. Cystic Meningioma Masquerading as a Metastatic Tumor: A Case Report.

    PubMed

    Ramanathan, Nithya; Kamaruddin, Khairul Azmi; Othman, Aizzat; Mustafa, Fadhli; Awang, Mohamed Saufi

    2016-05-01

    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

  7. 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.

  8. Recurrence of Skull Base Meningiomas: The Role of Aggressive Removal in Surgical Treatment.

    PubMed

    da Silva, Carlos Eduardo; Peixoto de Freitas, Paulo Eduardo

    2016-06-01

    Objectives The recurrence of meningiomas is a crucial aspect that must be considered during the planning of treatment strategy. The Simpson grade classification is the most relevant surgical aspect to predict the recurrence of meningiomas. We report on a series of patients with recurrent skull base meningiomas who were treated with the goal of radical removal. Design A retrospective study. Setting Hospital Ernesto Dornelles, Porto Alegre, Brazil. Participants Patients with recurrent skull base meningiomas. Main Outcomes Measures The goal of obtaining aggressive resection (i.e., Simpson grades I and II). Results The average age was 54 years, the mean follow-up period was 52.1 months, and Simpson grades I and II were obtained in 82%. The overall mortality was 5.8%. Transient cranial nerve deficits occurred in 11.7%; the definitive morbidity was also 5.8%. A second recurrence occurred in 5.8%. Conclusions Radical removal of recurrent skull base meningiomas is achievable and should be considered an option with a good outcome and an acceptable morbidity. The common surgical finding that was responsible for recurrence in this study was incomplete removal during the first surgery. We recommend extensive dura and bone removal in the surgical treatment of such recurrent lesions. PMID:27175316

  9. 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.

  10. APPARATUS FOR SHEATHING RODS

    DOEpatents

    Ford, W.K.; Wyatt, M.; Plail, S.

    1961-08-01

    An arrangement is described for sealing a solid body of nuclear fuel, such as a uranium metal rod, into a closelyfitting thin metallic sheath with an internal atmosphere of inert gas. The sheathing process consists of subjecting the sheath, loaded with the nuclear fuel body, to the sequential operations of evacuation, gas-filling, drawing (to entrap inert gas and secure close contact between sheath and body), and sealing. (AEC)

  11. 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.

  12. Primary extradural calvarial meningioma: case report.

    PubMed

    Damtie, Zenebe Gedlie

    2004-01-01

    Intracranial meningiomas usually develop intradurally with in the confines of the skull. Meningiomas originating in an extradural location are rare. A woman of 69 years had a hard mass located in the left parietal region. The neurological examination and laboratory data were with in normal limits. Plain skull X-rays revealed hyperostotic changes. The meningioma was removed in bloc. And histologically, it was found to be transitional type of menigioma. PMID:15884277

  13. Atypical and ischemic features of embolized meningiomas.

    PubMed

    Matsuda, Ken; Takeuchi, Hiroaki; Arai, Yoshikazu; Kitai, Ryuhei; Hosoda, Tetsuya; Tsunetoshi, Kenzo; Arishima, Hidetaka; Sato, Kazufumi; Kikuta, Ken-Ichiro

    2012-01-01

    Preoperative embolization (POE) of meningiomas is widely used to facilitate surgical removal and to reduce intraoperative blood loss. The resulting necrosis and enhanced proliferation have been reported to affect subsequent histologic grading. However, there was little concern about ischemic features, for example small cells resembling atypical meningiomas, cytoplasmic vacuoles resembling clear cell meningioma, intercellular discohesion resembling rhabdoid meningioma, and perivascular cuffs resembling papillary meningioma. Therefore, the extent of these ischemic features was scored and Ki-67 staining indices were investigated in a POE group composed of 29 specimens of meningiomas treated with POE and compared with equivalent results for a non-POE group composed of 29 meningiomas that were not treated with POE. Small cells with high N/C ratios, cytoplasmic vacuoles, intercellular discohesion, and perivascular cuffs were significantly increased in the POE group (versus the non-POE group, p < 0.05). There were no significant differences of the Ki-67 index between the POE group (2.2%) and the non-POE group (1.9%) (p = 0.49). Our results suggest that small cell change resulting in necrosis may be followed by POE, and that clear cell-like, rhabdoid cell-like, or pseudopapillary pattern identified in meningiomas may also be induced by POE. Therefore, histological findings and determination of grading should be evaluated cautiously in cases of embolized meningiomas. PMID:21789536

  14. 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. PMID:15669787

  15. Incidental Meningiomas: Management in the Neuroimaging Era.

    PubMed

    Spasic, Marko; Pelargos, Panayiotis E; Barnette, Natalie; Bhatt, Nikhilesh S; Lee, Seung James; Ung, Nolan; Gopen, Quinton; Yang, Isaac

    2016-04-01

    The number of patient imaging studies has increased because of precautious physicians ordering scans when a vague symptom is presented; subsequently, the number of incidental meningiomas detected has increased as well. These brain tumors do not present with related symptoms and are usually small. MRI and computed tomographic scans most frequently capture incidental meningiomas. Incidental meningiomas are managed with observation, radiation, and surgical resection. Ultimately, a conservative approach is recommended, such as observing an incidental meningioma and then only radiating if the tumor displays growth, whereas a surgical approach is to be used only when proven necessary. PMID:27012387

  16. [Ganglia of peripheral nerves].

    PubMed

    Tatagiba, M; Penkert, G; Samii, M

    1993-01-01

    The authors present two different types of ganglion affecting the peripheral nerves: extraneural and intraneural ganglion. Compression of peripheral nerves by articular ganglions is well known. The surgical management involves the complete removal of the lesion with preservation of most nerve fascicles. Intraneural ganglion is an uncommon lesion which affects the nerve diffusely. The nerve fascicles are usually intimately involved between the cysts, making complete removal of all cysts impossible. There is no agreement about the best surgical management to be applied in these cases. Two possibilities are available: opening of the epineural sheath lengthwise and pressing out the lesion; or resection of the affected part of the nerve and performing a nerve reconstruction. While in case of extraneural ganglion the postoperative clinical evolution is very favourable, only long follow up studies will reveal in case of intraneural ganglion the best surgical approach. PMID:8128785

  17. Meningioma as a cause of chronic orofacial pain: case reports.

    PubMed

    Cook, R J; Sharif, I; Escudier, M

    2008-09-01

    We describe two middle-aged men whose chronic orofacial pain was caused by underlying meningiomas. In both cases treatment was delayed because evolving dentoalveolar and possible chronic idiopathic facial pain had been investigated before presentation. Subsequent disturbances of the ipsilateral VII (and later VIII) nerves prompted magnetic resonance imaging (MRI) of one patient, while the possibility of a central lesion was recognised at presentation in the second, whose atypical focus of trigeminal neuralgia was labile within the ipsilateral distribution of the trigeminal nerve. Both cases highlight the importance of considering proximal intracranial lesions as a possible cause of atypical or refractory chronic orofacial pain so unnecessary delay in the diagnosis of otherwise operable tumours can be avoided. PMID:18304709

  18. Germline and somatic mutations in meningiomas.

    PubMed

    Smith, Miriam J

    2015-04-01

    Meningiomas arise from the arachnoid layer of the meninges that surround the brain and spine. They account for over one third of all primary central nervous system tumors in adults and confer a significant risk of location-dependent morbidity due to compression or displacement. A significant increase in risk of meningiomas is associated with neurofibromatosis type 2 (NF2) disease through mutation of the NF2 gene. In addition, approximately 5% of individuals with schwannomatosis disease develop meningiomas, through mutation of the SWI/SNF chromatin remodeling complex subunit, SMARCB1. Recently, a second SWI/SNF complex subunit, SMARCE1, was identified as a cause of clear cell meningiomas, indicating a wider role for this complex in meningioma disease. The sonic hedgehog (SHH)-GLI1 signaling pathway gene, SUFU, has also been identified as the cause of hereditary multiple meningiomas in a large Finnish family. The recent identification of somatic mutations in components of the SHH-GLI1 and AKT1-MTOR signaling pathways indicates the potential for cross talk of these pathways in the development of meningiomas. This review describes the known meningioma predisposition genes and their links to the recently identified somatic mutations. PMID:25857641

  19. Stereotactic radiosurgery for WHO grade I meningiomas.

    PubMed

    Sheehan, Jason P; Williams, Brian J; Yen, Chun Po

    2010-09-01

    Meningiomas represent a common intracranial tumor in the adult population. Although extirpation to achieve a gross total resection or at least decrease mass effect has been the mainstay of treatment, stereotactic radiosurgery has come to play an increasingly important role in the management of patients with meningiomas. Radiosurgery utilizes highly focused, beams of ionizing radiation to inactivate tumor cells. Image guidance and a steep dose fall off are critical features of this approach. The radiobiology of radiosurgery differs in certain advantageous ways from conventional radiotherapy. Radiosurgery initially was utilized to treat recurrent or residual skull base meningiomas. As success was observed in this setting, radiosurgery has gradually expanded its role so as to treat convexity meningiomas; it is also used as an upfront treatment for patients for whom clinical and neuro-imaging findings are consistent with a meningioma. Most large series demonstrate tumor control rates for patients with grade I meningiomas in excess of 85%. Neurological function is generally preserved or improved for patients with meningiomas. However, complications can occur. Longitudinal follow-up including neurologic and radiologic assessment is required. Single and multisession stereotactic radiosurgery will likely play an expanded role in the treatment of patients with meningiomas. PMID:20734218

  20. Metastatic Meningioma Presenting as Cancer of Unknown Primary

    PubMed Central

    Gupta, Vinay; Gonsalves, Wilson I.; Robinson, Steven I.

    2013-01-01

    We describe a case of anaplastic meningioma presenting in an extracranial osseous location, initially diagnosed as cancer of unknown primary. Although anaplastic meningioma comprise 3% of all meningiomas, this subtype is more likely to be associated with metastases. The increased degree of dedifferentiation in anaplastic meningioma makes diagnosis difficult, especially if characteristic imaging findings of meningioma are not identified. Adequate tissue for diagnostic purposes and appropriate imaging studies may help in establishing a definitive diagnosis. PMID:24416493

  1. 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

  2. RF Sheath Models

    NASA Astrophysics Data System (ADS)

    D'Ippolito, D. A.; Myra, J. R.

    2007-11-01

    RF sheath formation on the antennas and walls in ICRF-heated experiments can reduce the heating efficiency, limit the coupled power, and cause damage to plasma-facing structures. The sheaths are driven by a slow wave component of the rf field due to a mismatch between the magnetic field and the boundary (antenna or wall). Quantitative modeling of the highly nonlinear sheaths may now be feasible for the first time in massively-parallel-processing (MPP) codes developed in the RF SciDAC project. Recently, a new approach to sheath modeling was proposed,ootnotetextD.A. D'Ippolito and J.R. Myra, Phys. Plasmas 13, 102508 (2006). in which the sheath physics is incorporated into the RF wave computation by using a modified boundary condition (BC) on the RF fields in both wave propagation and antenna codes. Here, we illustrate the use of the sheath BC for near-field sheaths by a model calculation that includes electromagnetic effects and a simple antenna coupling model. Properties of the model (such as the role of sheath-plasma waves) and implications for antenna codes such as TOPICAootnotetextV. Lancellotti et al., Nucl. Fusion 46, S476 (2006). will be discussed.

  3. 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

  4. Unilateral facial myokymia in a dog with an intracranial meningioma.

    PubMed

    Holland, C T; Holland, J T; Rozmanec, M

    2010-09-01

    A 23-month-old castrated male Cavalier King Charles spaniel was evaluated because of a 6-month history of unusual rippling/undulating movements of the right facial muscles that were continuous and persisted during sleep. Neurological examination revealed narrowing of the right palpebral fissure and unilateral right-sided facial myokymia that was characterised by myokymic, and to a lesser degree, neuromyotonic discharges on concentric needle electromyographic examination. After persisting unchanged for almost 2.5 years from its onset, the facial myokymia gradually disappeared over a 6-month period concomitant with the emergence of a persistent ipsilateral facial paralysis and head tilt. At 5 years and 9 months after the first examination, signs of ipsilateral lacrimal, pharyngeal and laryngeal dysfunction became evident and the dog was euthanased. Postmortem examination identified a malignant (WHO grade III) meningioma in the right cerebellopontomedullary angle that compressed the ventrolateral cranial medulla, effaced the jugular foramen and internal acoustic meatus and extended into the facial canal of the petrous temporal bone. Novel findings were the unique observation of isolated unilateral facial myokymia preceding diagnosis of a meningioma affecting facial nerve function within the caudal cranial fossa and the remarkably long duration of neurological signs (75 months) attributable to the neoplasm. PMID:20726972

  5. Spontaneous Regression of an Incidental Spinal Meningioma

    PubMed Central

    Yilmaz, Ali; Kizilay, Zahir; Sair, Ahmet; Avcil, Mucahit; Ozkul, Ayca

    2016-01-01

    AIM: 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. CASE REPORT: 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. CONCLUSION: Although it is a rare condition, the clinicians should consider that meningiomas especially incidentally diagnosed may be regressed spontaneously. PMID:27275345

  6. Plasma-Sheath Model

    NASA Astrophysics Data System (ADS)

    Riemann, Karl-Ulrich

    2012-10-01

    In typical gas discharges a quasineutral plasma is shielded from a negativ absorbing wall by a thin positive sheath that is nearly planar and collision-free. The subdivision of ``plasma'' and ``sheath'' was introduced by Langmuir and is based on a small ratio of the electron Debye lenghth λD to the dominant competing characteristic plasma length l. Depending on the special conditions, l may represent, e.g., the plasma extension, the ionization length, the ion mean free path, the ion gyro radius, or a geometric length. Strictly speaking, this subdivion is possible only in the asymptotic limit λD/l->0. The asymptotic analysis results in singularities at the ``sheath edge'' closely related to the ``Bohm criterion.'' Due to these singularities a direct smooth matching of the separate plasma and sheath soltions is not possible. To obtain a consistent smooth transition, the singular sheath edge must be bridged by an additinal narrow ``intermediate'' model zone accounting both for plasma processes (e.g., collisions) and for the first build up of space charge. Due to this complexity and to different interpretations of the ``classical'' papers by Langmuir and Bohm, the asymptotic plasma-sheath concept and the definition of the sheath edge were questioned and resulted in controversies during the last two decades. We discuss attempts to re-define the sheath edge, to account for finite values of λD/l in the Bohm criterion, and demonstrate the consistent matching of plasma and sheath. The investigations of the plasma-sheath transition discussed so far are based on a simplified fluid analysis that cannot account for the essential inhomogeneity of the boundary layer and for the dominant role of slow ions in space charge formation. Therefore we give special emphasis to the kinetic theory of the plasma-sheath transition. Unfortunately this approach results in an additional mathematical difficulty caused by ions with zero velocity. We discuss attempts to avoid this singularity by

  7. 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

  8. A retrospective analysis of meningioma in Central Texas.

    PubMed

    Fonkem, Ekokobe; Dandashi, Jad A; Stroberg, Edana; Garrett, David; Harris, Frank S; El Nihum, Ibrahim M; Cooper, James; Dayawansa, Samantha; Huang, Jason H

    2016-06-01

    Documented meningioma cases in Central Texas (USA) from 1976 to 2013 were studied utilizing the Scott & White Brain Tumor Registry. All the cases examined were histologically diagnosed as meningiomas. Of the 372 cases, most were benign tumors (p<0.05). A majority of the patients were females (p<0.05). Elderly individuals (>45years of age) superseded the younger patients in meningioma incidence (p<0.05). Previous data regarding meningioma epidemiology in Texas showed a higher incidence in black patients when compared to white patients. By contrast, this study's findings of Central Texas meningioma demographics show increased incidence of meningiomas in white patients (p<0.05). This interesting find in meningioma prevalence warrants further investigation with a larger sample size, in order to establish validity and further parse out possible causes of meningioma development among white individuals. PMID:26851351

  9. 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

  10. Meningiomas with Rhabdoid or Papillary Components : Prognosis and Comparison with Anaplastic Meningiomas.

    PubMed

    Kim, Jeong-Kwon; Jung, Tae-Young; Jung, Shin; Lee, Kyung-Hwa; Kim, Seul-Kee; Lee, Eun Jung

    2016-07-01

    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

  11. 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

  12. Suprasellar Clear Cell Meningioma in an Infant

    PubMed Central

    Anunobi, Charles C.; Bankole, Olufemi; Ikeri, Nzechukwu Z.; Adeleke, Nurudeen A.

    2016-01-01

    Clear cell meningiomas are an uncommon subtype of meningioma rarely seen in infancy. We report a case of clear cell meningioma in an 8-month-old male infant. He presented at the Lagos University Teaching Hospital, Lagos, Nigeria, in 2015 with persistent vomiting, poor feeding and failure to thrive over a four month period. Generalised hypertonia and hyperreflexia were noted on examination. Computed tomography of the brain revealed a huge largely isodense suprasellar mass with a hypodense core. The tumour, which measured 6 × 5 × 4 cm, enhanced non-uniformly with contrast injection and extended to occlude the third ventricle. The patient underwent a bifrontal craniotomy with subtotal tumour excision. Six hours postoperatively, he went into cardiac arrest and could not be resuscitated. A histological diagnosis of clear cell meningioma was made as the tumour cells were immunoreactive to epithelial membrane antigen, S100 protein and vimentin. This case of clear cell meningioma was unusual due to its early occurrence and supratentorial location. PMID:27606120

  13. Genetic and molecular alterations in meningiomas.

    PubMed

    Alexiou, George A; Markoula, Sofia; Gogou, Pinelopi; Kyritsis, Athanasios P

    2011-05-01

    Meningiomas are the most common benign intracranial tumors in adults arising from the dura matter. The etiology of meningiomas is mostly unknown, although several risk factors have been described, such as ionizing radiation, head injury, hormones and genetic factors. According to WHO they are classified into 3 grades, grade I, grade II and grade III. Meningiomas express various hormonal and growth factor receptors, such as progesterone, estrogen, somatostatin, transforming growth factor alpha (TGF-alpha) and epidermal growth factor (EGF) receptors, which may be related to their biological behavior and response to treatment. Chromosomal abnormalities linked to meningiomas involve chromosomes 22, 1p, 9p, 10p, 11, 14q, 15, 17, and 18q. In addition, genes that may be involved in the formation of meningiomas include NF2, DAL-1, p14 (ARF), p53, MDM2, Rb, p16 and c-myc. It is likely that detailed molecular information will aid in establishing a molecular grading of these tumors and predict response to treatment and survival. PMID:21227570

  14. Suprasellar Clear Cell Meningioma in an Infant.

    PubMed

    Anunobi, Charles C; Bankole, Olufemi; Ikeri, Nzechukwu Z; Adeleke, Nurudeen A

    2016-08-01

    Clear cell meningiomas are an uncommon subtype of meningioma rarely seen in infancy. We report a case of clear cell meningioma in an 8-month-old male infant. He presented at the Lagos University Teaching Hospital, Lagos, Nigeria, in 2015 with persistent vomiting, poor feeding and failure to thrive over a four month period. Generalised hypertonia and hyperreflexia were noted on examination. Computed tomography of the brain revealed a huge largely isodense suprasellar mass with a hypodense core. The tumour, which measured 6 × 5 × 4 cm, enhanced non-uniformly with contrast injection and extended to occlude the third ventricle. The patient underwent a bifrontal craniotomy with subtotal tumour excision. Six hours postoperatively, he went into cardiac arrest and could not be resuscitated. A histological diagnosis of clear cell meningioma was made as the tumour cells were immunoreactive to epithelial membrane antigen, S100 protein and vimentin. This case of clear cell meningioma was unusual due to its early occurrence and supratentorial location. PMID:27606120

  15. Transformation of a meningioma with atypical imaging.

    PubMed

    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

  16. Periostin: a novel prognostic predictor for meningiomas.

    PubMed

    Liu, Yi; Shi, Jin; Chen, Ming; Cao, Yong-fu; Liu, Ya-wei; Pan, Jun; Qi, Song-tao

    2015-02-01

    The expression and role of periostin in meningiomas remains unknown. Tissue specimens of 175 convexity meningiomas were immunohistochemically examined with antibodies against periostin and Ki67. The expression levels of periostin and Ki67 were compared among different WHO groups. The role of periostin and Ki67 in postoperative prognosis of meningiomas was also analyzed. Negative (-) expression of Ki67 was observed in 101 (57.7 %) cases of all the surgical tissue samples. The Ki67 expressions differed significantly among the WHO groups (P < 0.001) and correlated positively with the WHO grade (r = 0.673, P < 0.001). Low/negative staining of periostin was observed in 116 (66.3 %) cases. The periostin expressions differed significantly among the WHO groups (P < 0.001). Periostin expression correlated positively with the WHO grade (r = 0.742, P < 0.001). There was a positive correlation between Ki67 expression and periostin (r = 0.513, P < 0.001). Both Ki67 expression and periostin expression was found statistically different between brain invasion tumor and non-invasion tumor (p < 0.001). The recurrence rate and PFS rate in both varied Ki67 expression groups and periostin expression groups was statistically different (P < 0.001). The survival time and PFS time in both varied Ki67 expression groups and periostin expression groups was also statistically different (P < 0.001). Periostin was expressed in tumor stroma of meningiomas. Both periostin and Ki67 may behave as a maker in predicting the grade and prognosis in meningiomas. Drugs that targets periostin aims at reducing invasion of meningioma patients should be further researched. PMID:25519301

  17. 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. PMID:26191206

  18. 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.

  19. 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

  20. The longitudinal epineural incision and complete nerve transection method for modeling sciatic nerve injury

    PubMed Central

    Cheng, Xing-long; Wang, Pei; Sun, Bo; Liu, Shi-bo; Gao, Yun-feng; He, Xin-ze; Yu, Chang-yu

    2015-01-01

    Injury severity, operative technique and nerve regeneration are important factors to consider when constructing a model of peripheral nerve injury. Here, we present a novel peripheral nerve injury model and compare it with the complete sciatic nerve transection method. In the experimental group, under a microscope, a 3-mm longitudinal incision was made in the epineurium of the sciatic nerve to reveal the nerve fibers, which were then transected. The small, longitudinal incision in the epineurium was then sutured closed, requiring no stump anastomosis. In the control group, the sciatic nerve was completely transected, and the epineurium was repaired by anastomosis. At 2 and 4 weeks after surgery, Wallerian degeneration was observed in both groups. In the experimental group, at 8 and 12 weeks after surgery, distinct medullary nerve fibers and axons were observed in the injured sciatic nerve. Regular, dense myelin sheaths were visible, as well as some scarring. By 12 weeks, the myelin sheaths were normal and intact, and a tight lamellar structure was observed. Functionally, limb movement and nerve conduction recovered in the injured region between 4 and 12 weeks. The present results demonstrate that longitudinal epineural incision with nerve transection can stably replicate a model of Sunderland grade IV peripheral nerve injury. Compared with the complete sciatic nerve transection model, our method reduced the difficulties of micromanipulation and surgery time, and resulted in good stump restoration, nerve regeneration, and functional recovery. PMID:26692866

  1. 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

  2. Pediatric meningiomas an aggressive subset: A clinicopathological and immunohistochemical study

    PubMed Central

    Hui, M; Uppin, MS; Saradhi, M Vijaya; Sahu, BP; Purohit, AK; Sundaram, C

    2015-01-01

    Background: Meningiomas are uncommon neoplasms in the pediatric age group and differ in various aspects from their adult counterparts. They account for 0.4-4.6% of all primary brain tumors. Aims: To retrospectively analyze the clinicopathological and immunohistochemical features of pediatric meningiomas. Materials and Methods: Meningiomas in patients under 18 years of age diagnosed between January 2001 to December 2011 were analyzed retrospectively. The hematoxylin and eosin stained sections and Ki 67 labelling index (LI) were reviewed for all the cases Results: The pediatric meningiomas accounted for 1.52% of total meningiomas (15/983). The mean age at presentation was 12 years with male to female ratio of 1.5:1. The presenting symptoms were headache, seizures, and motor deficits. The histology included 9 cases (60%) of atypical meningioma (WHO grade II) followed by 4 cases (26.67%) of WHO grade-I and 2 cases (13.33%) of anaplastic meningioma (WHO grade III). Five cases had a recurrence. Ki67 LI ranged from 0.5% to 1.5% in grade I, 0.5% to 15% in grade II and 13% to 24% in grade III meningiomas. Conclusion: Meningiomas are rare in children and show a male preponderance. There was a higher incidence of atypical and anaplastic meningiomas in pediatric population. PMID:25511215

  3. 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.

  4. Global expression profile in low grade meningiomas and schwannomas shows upregulation of PDGFD, CDH1 and SLIT2 compared to their healthy tissue.

    PubMed

    Torres-Martin, Miguel; Lassaletta, Luis; Isla, Alberto; De Campos, Jose M; Pinto, Giovanny R; Burbano, Rommel R; Castresana, Javier S; Melendez, Barbara; Rey, Juan A

    2014-12-01

    Schwannomas and grade I meningiomas are non‑metastatic neoplasms that share the common mutation of gene NF2. They usually appear in neurofibromatosis type 2 patients. Currently, there is no drug treatment available for both tumors, thus the use of wide expression technologies is crucial to identify therapeutic targets. Affymetrix Human Gene 1.0 ST was used to test global gene expression in 22 meningiomas, 31 schwannomas and, as non-tumoral controls, 3 healthy meningeal tissues, 8 non-tumoral nerves and 1 primary Schwann cell culture. A non-stringent P-value cut-off and fold change were used to establish deregulated genes. We identified a subset of genes that were upregulated in meningiomas and schwannomas when compared to their respectively healthy tissues, including PDGFD, CDH1 and SLIT2. Thus, these genes should be thoroughly studied as targets in a possible combined treatment. PMID:25333347

  5. Global expression profile in low grade meningiomas and schwannomas shows upregulation of PDGFD, CDH1 and SLIT2 compared to their healthy tissue

    PubMed Central

    TORRES-MARTIN, MIGUEL; LASSALETTA, LUIS; ISLA, ALBERTO; DE CAMPOS, JOSE M.; PINTO, GIOVANNY R.; BURBANO, ROMMEL R.; CASTRESANA, JAVIER S.; MELENDEZ, BARBARA; REY, JUAN A.

    2014-01-01

    Schwannomas and grade I meningiomas are non-metastatic neoplasms that share the common mutation of gene NF2. They usually appear in neurofibromatosis type 2 patients. Currently, there is no drug treatment available for both tumors, thus the use of wide expression technologies is crucial to identify therapeutic targets. Affymetrix Human Gene 1.0 ST was used to test global gene expression in 22 meningiomas, 31 schwannomas and, as non-tumoral controls, 3 healthy meningeal tissues, 8 non-tumoral nerves and 1 primary Schwann cell culture. A non-stringent P-value cut-off and fold change were used to establish deregulated genes. We identified a subset of genes that were upregulated in meningiomas and schwannomas when compared to their respectively healthy tissues, including PDGFD, CDH1 and SLIT2. Thus, these genes should be thoroughly studied as targets in a possible combined treatment. PMID:25333347

  6. 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

  7. Meningioma of Foramen Magnum Causing Drop Attacks

    PubMed Central

    Mahore, Amit; Mavani, Sandip; Rangarajan, Vithal; Patil, Manoj; Sathe, Prashant; Kawale, Juhi; Tikeykar, Vishakha

    2015-01-01

    A 52-year-old female presented with frequent episodes of falls without loss of consciousness. These episodes lasted for brief period followed by full neurological recovery. Magnetic resonance imaging (MRI) of the brain showed foramen magnum meningioma encasing left vertebral artery. The patient had dramatic improvement after excision of the tumor. PMID:25793133

  8. Dilemmas and diagnostic difficulties in meningioma.

    PubMed

    Hallinan, J T P D; Hegde, A N; Lim, W E H

    2013-08-01

    This article will review the uncommon locations and morphological features of meningiomas, which are important to recognize in order to avoid misdiagnosis. Uncommon locations will be demonstrated at the cerebellopontine angle, pineal, optic, intraventricular, and intradiploic regions. Unusual imaging features including cysts, metaplastic changes, and peritumoural oedema will also be discussed. PMID:23623578

  9. Spinal metastases from pituitary hemangiopericytic meningioma

    SciTech Connect

    Kumar, P.P.; Good, R.R.; Skultety, F.M.; Masih, A.S.; McComb, R.D.

    1987-10-01

    A rare, previously irradiated, recurrent malignant angioblastic meningioma of the pituitary, hemangiopericytic type, was locally controlled by a new endocurietherapy technique that allows delivery of very high (10,000 cGy), sharply localized irradiation. Rather than succumbing to the local tumor recurrence, as would otherwise be expected, the patient developed distant spinal metastases several years later.

  10. Meningioma of foramen magnum causing drop attacks.

    PubMed

    Mahore, Amit; Ramdasi, Raghvendra; Mavani, Sandip; Rangarajan, Vithal; Patil, Manoj; Sathe, Prashant; Kawale, Juhi; Tikeykar, Vishakha

    2015-01-01

    A 52-year-old female presented with frequent episodes of falls without loss of consciousness. These episodes lasted for brief period followed by full neurological recovery. Magnetic resonance imaging (MRI) of the brain showed foramen magnum meningioma encasing left vertebral artery. The patient had dramatic improvement after excision of the tumor. PMID:25793133

  11. Mifepristone (RU 486) treatment of meningiomas.

    PubMed Central

    Lamberts, S W; Tanghe, H L; Avezaat, C J; Braakman, R; Wijngaarde, R; Koper, J W; de Jong, H

    1992-01-01

    Meningiomas are common brain tumours which are generally benign, well circumscribed and slow growing. In a minority of patients complete surgical removal is not possible and re-growth of tumour tissue is a major clinical problem. Most meningiomas contain progesterone receptors. The anti-progestational drug mifepristone (RU 486) binds to these receptors. Ten patients were treated with 12 recurrent or primary "inoperable" meningiomas, all of whom had shown recent neuroradiological and/or ophthalmological evidence of tumour growth. They received 200 mg mifepristone daily for 12 months. Most patients initially had complaints of nausea, vomiting and/or tiredness. In four patients prednisone (7.5 mg/day) was given after which these side-effects subsided. CT scan analysis of tumour size, showed progression of growth of five meningiomas in four patients, stable disease in three patients with three tumours and regression of four tumours in three patients. A decrease in the complaints of headache and an improved general well being was observed in five patients. Two patients died during the treatment period from unrelated causes. Mifepristone treatment resulted in control of tumour growth (= stable disease) in six of 10 patients who had shown recent evidence of tumour growth. In three of these six patients consistent tumour shrinkage was observed. PMID:1619417

  12. Chitosan conduits combined with nerve growth factor microspheres repair facial nerve defects

    PubMed Central

    Liu, Huawei; Wen, Weisheng; Hu, Min; Bi, Wenting; Chen, Lijie; Liu, Sanxia; Chen, Peng; Tan, Xinying

    2013-01-01

    Microspheres containing nerve growth factor for sustained release were prepared by a compound method, and implanted into chitosan conduits to repair 10-mm defects on the right buccal branches of the facial nerve in rabbits. In addition, chitosan conduits combined with nerve growth factor or normal saline, as well as autologous nerve, were used as controls. At 90 days post-surgery, the muscular atrophy on the right upper lip was more evident in the nerve growth factor and normal sa-line groups than in the nerve growth factor-microspheres and autologous nerve groups. physiological analysis revealed that the nerve conduction velocity and amplitude were significantly higher in the nerve growth factor-microspheres and autologous nerve groups than in the nerve growth factor and normal saline groups. Moreover, histological observation illustrated that the di-ameter, number, alignment and myelin sheath thickness of myelinated nerves derived from rabbits were higher in the nerve growth factor-microspheres and autologous nerve groups than in the nerve growth factor and normal saline groups. These findings indicate that chitosan nerve conduits bined with microspheres for sustained release of nerve growth factor can significantly improve facial nerve defect repair in rabbits. PMID:25206635

  13. Chitosan conduits combined with nerve growth factor microspheres repair facial nerve defects.

    PubMed

    Liu, Huawei; Wen, Weisheng; Hu, Min; Bi, Wenting; Chen, Lijie; Liu, Sanxia; Chen, Peng; Tan, Xinying

    2013-11-25

    Microspheres containing nerve growth factor for sustained release were prepared by a compound method, and implanted into chitosan conduits to repair 10-mm defects on the right buccal branches of the facial nerve in rabbits. In addition, chitosan conduits combined with nerve growth factor or normal saline, as well as autologous nerve, were used as controls. At 90 days post-surgery, the muscular atrophy on the right upper lip was more evident in the nerve growth factor and normal sa-line groups than in the nerve growth factor-microspheres and autologous nerve groups. physiological analysis revealed that the nerve conduction velocity and amplitude were significantly higher in the nerve growth factor-microspheres and autologous nerve groups than in the nerve growth factor and normal saline groups. Moreover, histological observation illustrated that the di-ameter, number, alignment and myelin sheath thickness of myelinated nerves derived from rabbits were higher in the nerve growth factor-microspheres and autologous nerve groups than in the nerve growth factor and normal saline groups. These findings indicate that chitosan nerve conduits bined with microspheres for sustained release of nerve growth factor can significantly improve facial nerve defect repair in rabbits. PMID:25206635

  14. Recurrent meningioma of the scalp after 13 years.

    PubMed

    Ashmore, Daniel Lee; Clancy, Rachel; Chumas, Paul D

    2016-01-01

    Meningiomas represent some of the most common types of tumour of the central nervous system. They are considered benign, with ∼1 in 1000 known to metastasise. We report a case of recurrent meningioma of the scalp 13 years following craniotomy to treat the primary. The recurrent tumour of the scalp was eventually excised 5 years later and the defect closed with an anterolateral thigh free flap in a joint procedure between the neurosurgeons and plastic surgeons. Histology of the scalp lesion confirmed a WHO grade I meningioma. Although a number of explanations for recurrent scalp meningiomas exist, the precise role of isolated genetic defects in scalp meningiomas is incomplete. The scalp meningioma in this case consisted of a complex karyotype suggestive of more aggressive disease. This case emphasises that cytogenetics may play a greater role in identifying more aggressive tumours than histology alone. PMID:27389725

  15. Multiple Meningiomas in a Patient with Cowden Syndrome

    PubMed Central

    Pain, Margaret; Darbinyan, Armine; Fowkes, Mary; Shrivastava, Raj

    2016-01-01

    Background  Cowden syndrome is a rare, multisystem disease manifesting with increased hamartomas and neoplasms. Though meningioma has been documented in patients with Cowden syndrome, the relationship between these two phenomena is still unclear. Case Description  We report a case of a 43-year-old female patient with a known PTEN mutation and clinical history of Cowden syndrome. A workup of headache demonstrated two skull base meningiomas. At the time of surgery, several additional tiny meningiomas were detected in the same region. Conclusions  The development of multiple meningiomas in a patient with predisposition for tumor is more than coincidental. Though PTEN mutations and deletions have not been shown to be critical for meningioma development, this case challenges that conclusion. In light of recent genetic advances in meningioma molecular pathogenesis, the role of the PTEN/AKT/PI3K pathway is discussed. PMID:27563534

  16. 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

  17. Analytic Model of Antenna Sheaths

    NASA Astrophysics Data System (ADS)

    D'Ippolito, D. A.; Myra, J. R.

    2008-11-01

    RF sheaths are generated on ICRF antennas whenever the launched fast wave also drives a slow wave, e.g. when the magnetic field is tilted (not perpendicular to the current straps). A new approach to sheath modeling was recently proposed in which the RF waves are computed using a modified boundary condition at the sheath surface to describe the plasma-sheath coupling. Here, we illustrate the use of the sheath BC for antenna sheaths by a model electromagnetic perturbation calculation, treating the B field tilt as a small parameter. Analytic expressions are obtained for the sheath voltage and the rf electric field parallel to B in both sheath and plasma regions, including the Child-Langmuir (self-consistency) constraint. It is shown that the plasma corrections to the sheath voltage (which screen the rf field) can be important. The simple vacuum-field sheath-voltage estimate is obtained as a limiting case. Implications for antenna codes such as TOPICA will be discussed. D.A. D'Ippolito and J.R. Myra, Phys. Plasmas 13, 102508 (2006). V. Lancellotti et al., Nucl. Fusion 46, S476 (2006).

  18. Cavernous Hemangioma of the Skull and Meningioma: Association or Coincidence?

    PubMed Central

    Kilani, M.; Darmoul, M.; Hammedi, F.; Ben Nsir, A.; Hattab, M. N.

    2015-01-01

    Intraosseous cavernous hemangiomas of the skull are rare. Meningiomas are quite frequently encountered in a neurosurgical practice. The association between these two entities is nevertheless very uncommon. The authors present a case of a 72-year-old woman suffering from headache. The MRI showed a parietal meningioma with adjacent thick bone. The meningioma and the bone were removed. The histological examination confirmed the diagnosis of meningioma and revealed a cavernoma of the skull. The relationship between the lesions suggests more than a coincidental association. Several hypotheses are proposed to explain common causal connections. PMID:25960899

  19. Loss of SUFU Function in Familial Multiple Meningioma

    PubMed Central

    Aavikko, Mervi; Li, Song-Ping; Saarinen, Silva; Alhopuro, Pia; Kaasinen, Eevi; Morgunova, Ekaterina; Li, Yilong; Vesanen, Kari; Smith, Miriam J.; Evans, D. Gareth R.; Pöyhönen, Minna; Kiuru, Anne; Auvinen, Anssi; Aaltonen, Lauri A.; Taipale, Jussi; Vahteristo, Pia

    2012-01-01

    Meningiomas are the most common primary tumors of the CNS and account for up to 30% of all CNS tumors. An increased risk of meningiomas has been associated with certain tumor-susceptibility syndromes, especially neurofibromatosis type II, but no gene defects predisposing to isolated familial meningiomas have thus far been identified. Here, we report on a family of five meningioma-affected siblings, four of whom have multiple tumors. No NF2 mutations were identified in the germline or tumors. We combined genome-wide linkage analysis and exome sequencing, and we identified in suppressor of fused homolog (Drosophila), SUFU, a c.367C>T (p.Arg123Cys) mutation segregating with the meningiomas in the family. The variation was not present in healthy controls, and all seven meningiomas analyzed displayed loss of the wild-type allele according to the classic two-hit model for tumor-suppressor genes. In silico modeling predicted the variant to affect the tertiary structure of the protein, and functional analyses showed that the activity of the altered SUFU was significantly reduced and therefore led to dysregulated hedgehog (Hh) signaling. SUFU is a known tumor-suppressor gene previously associated with childhood medulloblastoma predisposition. Our genetic and functional analyses indicate that germline mutations in SUFU also predispose to meningiomas, particularly to multiple meningiomas. It is possible that other genic mutations resulting in aberrant activation of the Hh pathway might underlie meningioma predisposition in families with an unknown etiology. PMID:22958902

  20. 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. PMID:27338209

  1. Meningioma and occlusive vasculopathy: coexisting complications of past extracranial radiation

    SciTech Connect

    Montanera, W.; Chui, M.; Hudson, A.

    1985-07-01

    Two cases are reported in which a meningioma and occlusion of the internal carotid artery with development of transdural collateral circulation coexisted following extracranial radiation in childhood.

  2. 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.

  3. 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

  4. Immediate versus delayed primary nerve repair in the rabbit sciatic nerve.

    PubMed

    Piskin, Ahmet; Altunkaynak, Berrin Zühal; Çitlak, Atilla; Sezgin, Hicabi; Yazιcι, Ozgür; Kaplan, Süleyman

    2013-12-25

    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

  5. 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. PMID:19577476

  6. 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. PMID:26986119

  7. Microsurgical resection of giant intraventricular meningioma.

    PubMed

    Liu, James K

    2013-01-01

    Intraventricular meningiomas are rare tumors, accounting for approximately 0.5 to 3% of all intracranial meningiomas. The majority arise in the atrium of the lateral ventricle. The surgical management of these tumors remains a considerable challenge because of their deep location and proximity to critical structures. Complete resection, if safely possible, should be the goal of surgery since this results in the best rates of local control. Although various approaches exist to access the lateral ventricular system, selection of the optimal approach should be individualized to the patient based upon the location of the tumor within the ventricle, the tumor size, the origin of the vascular supply to the tumor, and the relationship to neighboring neurovascular structures at risk. In this operative video manuscript, the author demonstrates an illustrative step-by-step technique for microsurgical resection of a giant intraventricular meningioma of the left atrium via a transcortical parieto-occipital approach. The patient illustrated in this video presented with a large recurrent meningioma (> 5 cm) approximately 10 years after the initial resection. The tumor had grown around a pre-existing shunt catheter and resulted in loculated hydrocephalus. A complete resection and shunt revision were both performed at the same sitting. The operative technique and surgical nuances, including the surgical approach, intradural tumor removal, closure, and management of hydrocephalus are illustrated in this video atlas. The video can be found here: http://youtu.be/vpdmZ1ccWSM. (http://thejns.org/doi/abs/10.3171/2013.V1.FOCUS12352) PMID:23282155

  8. Prostate carcinoma mimicking a sphenoid wing meningioma

    PubMed Central

    Bradley, Lucas H.; Burton, Matthew; Gokden, Murat; Serletis, Demitre

    2015-01-01

    Introduction We report here on a rare case of a large, lateral sphenoid wing tumor with radiographic and intraoperative findings highly suggestive of meningioma, yet pathology was in fact consistent with metastatic prostate adenocarcinoma. Presentation of case An 81 year-old male presented with expressive dysphasia, right-sided weakness and headaches. Imaging revealed a heterogeneously-enhancing lesion based on the left lateral sphenoid wing. The presumed diagnosis was strongly in favor of meningioma, and the patient underwent complete resection of the dural-based lesion. Final pathology confirmed the unexpected finding of a metastatic prostate adenocarcinoma. Although he tolerated surgery well, the patient was subsequently referred for palliative therapy given findings of widespread systemic disease. Discussion Intracranial metastases may involve the dura, at times presenting with rare radiographic features highly suggestive for meningioma, as in our case here. This makes differentiation, at least based on imaging, a challenge. Elderly patients presenting with neurological deficits secondary to a newly-diagnosed, dural-based lesion should thus be considered for metastasis, prompting additional imaging studies (including body CT, MRI or PET) to rule out a primary lesion elsewhere. In some cases, this may affect the overall decision to proceed with surgical resection, or alternatively, to proceed directly to palliative therapy (the latter decision made in the context of widespread metastatic disease). Conclusion We conclude that dural-based metastatic lesions may mimic meningiomas, warranting thorough pre-operative work-up to exclude the possibility of metastasis. In certain cases, identification of widespread disease might preclude surgery and favor palliation, instead. PMID:26318129

  9. Resection of meningiomas with implantable microwave coagulation

    SciTech Connect

    Zhou, X.P.; Xie, Q.L.; Liu, J.M.; Yue, Z.J.; Cai, K.H.

    1996-05-01

    Implantable microwave coagulation was used to perform resection on 62 patients that had intracranial meningiomas. When 20--60 W microwave power was applied for 15 s, the temperature at the center of the tumor tissue was 43--63 C; 30 mm from the center, the temperature was under 40 C. Histological changes in the center of the tumor showed coagulative necrosis, diminished nuclei, and obliterated blood vessels. The changes at 10--20 mm from the center of the tumor showed coagulative necrosis and degeneration and, 30--50 mm from the center of the tumor, showed normal cell morphology after microwave coagulation. The thermal field in brain tumor has an effective diameter of about 40 mm. No side effects on the normal brain tissues were observed. The amount of blood loss during the operation was minimal while the meningioma was coagulated, especially when the meningioma was located at the skull base or in the parasagittal or cerebral convexity region. After microwave coagulation, the entire tumor could easily be removed. Among the 62 surgically treated cases, gross total tumor excision was 85%. No postoperative complications occurred after microwave coagulation, and there was no operative mortality in the series. The authors believe that this new technique has the advantage of simplicity, less blood loss, and smooth postoperative procedures. Hemostatic effects during the operation are satisfactory, and blood transfusion can be reduced by 50--60%.

  10. Meninigiomas of the Craniocervical Junction – A Distinctive Subgroup of Meningiomas

    PubMed Central

    Dührsen, Lasse; Emami, Pedram; Matschke, Jakob; Abboud, Tammam; Westphal, Manfred; Regelsberger, Jan

    2016-01-01

    Objective Meningioma of the cranio-cervical junction is a rare diagnosis and demand a thorough surgical planning as radical excision of these tumors is difficult. In this context recurrence is most likely due to regrowth of residual tumor. The aim of this study was to evaluate the clinical course of patients operated for craniocervical meningioma (CCM) and to investigate the histological features of these tumors and their impact on recurrence rate. Methods All patients who were operated for CCM at our institution between 2003 and 2012 were identified. Presenting symptoms, MRI findings, surgical approaches and recurrence rate were reviewed retrospectively using medical charts. Histological features of the included tumors were studied focusing on subtypes and MIB-1 immunoreactivity and compared with MIB-1 immunoreactivity in an age and gender-matched control group of patients with supratentorial meningioma. Results 18 patients with CCM with a mean age of 56.2 years and median follow-up of 60 months were included in the study. Sensory or motor deficit was the most frequent presenting symptom followed by neck pain and lower cranial nerve palsy. Simpson grade II resection was achieved in 16 patients and Simpson grade III resection in two patients. Mortality, morbidity and recurrence rates were 16.7%, 5.5% and 5.5%, respectively. According to the WHO-grading all were found to be grade I meningiomas. Histological subtypes included meningotheliomatous (10), transitional (2), fibrillar (2), angiomatous (3) and secretory (1) meningioma. The mean MIB-1 labeling index in the study group was significantly higher than in the control group, (7.2% and 3.6%, respectively), p < 0.05. There was no correlation between MIB-1 levels and tumor recurrence. Conclusions CCM seems to have a benign character. Despite a significantly higher MIB-1 index, a high rate of recurrence was not observed. Therefore, approaches with high morbidity are not justified. Nevertheless, in view of the

  11. 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'). PMID:22614870

  12. 21 CFR 884.5320 - Glans sheath.

    Code of Federal Regulations, 2012 CFR

    2012-04-01

    ... 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...

  13. 21 CFR 884.5320 - Glans sheath.

    Code of Federal Regulations, 2014 CFR

    2014-04-01

    ... 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...

  14. 21 CFR 884.5320 - Glans sheath.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

    ... 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...

  15. Challenging diagnosis of peripillous sheaths.

    PubMed

    Gnarra, Maria; Saraceni, Pierluigi; Rossi, Alfredo; Murabit, Amera; Caradonna, Emanuela; Fania, Luca; Feliciani, Claudio

    2014-01-01

    Peripillous sheaths, or hair casts, are asymptomatic, white, cylindrical concretions that encircle the hair without adhering to it. They are infrequently documented in the literature, are often misdiagnosed, and generate avoidable apprehension and expense for parents and caregivers. Dermoscopy is the standard for a rapid, noninvasive, cost-effective diagnosis. We describe a case of peripillous sheaths presenting in a boy. PMID:24846654

  16. Variants of meningiomas: a review of imaging findings and clinical features.

    PubMed

    Kunimatsu, Akira; Kunimatsu, Natsuko; Kamiya, Kouhei; Katsura, Masaki; Mori, Harushi; Ohtomo, Kuni

    2016-07-01

    Meningiomas are common neoplasms that frequently occur in the brain and spine. Among the 15 histological subtypes of meningiomas in the WHO classification, the incidence of meningothelial meningiomas is the highest, followed by fibrous and transitional meningiomas. These three subtypes account for approximately 80 % of all meningiomas, and thus could be regarded as typical meningiomas. For this reason, other uncommon histological subtypes may be considered as imaging variants, and diagnosis is often challenging for radiologists solely based on imaging features of typical meningiomas. In addition to the histological subtypes, meningiomas arising in atypical locations could be easily mistaken for other lesions more commonly observed in those locations. The purpose of this article is to review characteristic clinical and imaging findings of uncommon meningiomas, including histological variants and meningiomas occurring in relatively rare locations. PMID:27138052

  17. Sonography of Common Peripheral Nerve Disorders With Clinical Correlation.

    PubMed

    Jacobson, Jon A; Wilson, Thomas J; Yang, Lynda J-S

    2016-04-01

    Sonography is now considered an effective method to evaluate peripheral nerves. Low cost, high resolution, the ability to image an entire limb in a short time, and dynamic assessment are several of the positive attributes of sonography. This article will review the normal appearance of peripheral nerves as shown with sonography. In addition, the most common applications for sonography of the peripheral nerves will be reviewed, which include entrapment neuropathies, intraneural ganglion cyst, nerve trauma, and peripheral nerve sheath tumors. Clinical information related to nerve disorders is also included, as it provides valuable information that can be obtained during sonographic examinations, increasing diagnostic accuracy. PMID:26931790

  18. Cyclooxygenase-2 (COX-2) expression in canine intracranial meningiomas.

    PubMed

    Rossmeisl, J H; Robertson, J L; Zimmerman, K L; Higgins, M A; Geiger, D A

    2009-09-01

    Meningiomas are the most common canine intracranial tumour. Neurologic disability and death from treatment failure remain problematic despite current surgical and radiotherapeutic treatments for canine intracranial meningiomas. Cyclooxygenase-2 (COX-2) over-expression has been demonstrated in multiple canine malignancies, and COX-2 inhibitory treatment strategies have been shown to have both preventative and therapeutic effects in spontaneous and experimental models of cancer. The purpose of this study was to evaluate COX-2 expression in canine intracranial meningiomas. Immunohistochemical and Western blot (WB) analyses showed COX-2 expression in multiple tissues of the normal canine brain, and 87% (21/24) of intracranial meningiomas studied were immunoreactive to COX-2. No significant associations between COX-2 immunoreactivity and tumour grade were identified. Further studies are required to elucidate the physiologic roles of constitutive COX-2 expression in the central nervous system as well as its participation in meningioma tumourigenesis. PMID:19691646

  19. Nerve biopsy

    MedlinePlus

    Nerve biopsy may be done to help diagnose: Axon degeneration (destruction of the axon portion of the nerve cell) Damage to the ... Demyelination Inflammation of the nerve Leprosy Loss of axon tissue Metabolic neuropathies Necrotizing vasculitis Sarcoidosis

  20. Pinched Nerve

    MedlinePlus

    ... Enhancing Diversity Find People About NINDS NINDS Pinched Nerve Information Page Table of Contents (click to jump ... being done? Clinical Trials Organizations What is Pinched Nerve? The term "pinched nerve" is a colloquial term ...

  1. 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…

  2. 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.

  3. 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

  4. Chemically extracted acellular allogeneic nerve graft combined with ciliary neurotrophic factor promotes sciatic nerve repair.

    PubMed

    Zhang, Yanru; Zhang, Hui; Katiella, Kaka; Huang, Wenhua

    2014-07-15

    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

  5. 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.

  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. Fractionated stereotactic radiation therapy improves cranial neuropathies in patients with skull base meningiomas: a retrospective cohort study

    PubMed Central

    2012-01-01

    Background Skull base meningiomas commonly present with cranial neuropathies. Fractionated stereotactic radiation therapy (FSRT) has been used to treat these tumors with excellent local control, but rates of improvement in cranial neuropathies have not been well defined. We review the experience at Thomas Jefferson University using FSRT in the management of these patients with a focus on symptom outcomes. Methods We identified 225 cases of skull base meningiomas treated with FSRT at Thomas Jefferson University from 1994 through 2009. The target volume was the enhancing tumor, treated to a standard prescription dose of 54 Gy. Symptoms at the time of RT were classified based on the cranial nerve affected. Logistic regression was performed to determine predictors of symptom improvement after FSRT. Results The median follow-up time was 4.4 years. In 92% of cases, patients were symptomatic at the time of RT; the most common were impaired visual field/acuity (58%) or extraocular movements (34%). After FSRT, durable improvement of at least one symptom occurred in 57% of cases, including 40% of visual acuity/visual field deficits, and 40% of diplopia/ptosis deficits. Of all symptomatic patients, 27% experienced improvement of at least one symptom within 2 months of the end of RT. Conclusions FSRT is very effective in achieving improvement of cranial neuropathies from skull base meningiomas, particularly visual symptoms. Over half of treated patients experience a durable improvement of at least one symptom, frequently within 2 months from the end of RT. PMID:23270432

  8. Embolized meningiomas: risk of overgrading and neo-angiogenesis.

    PubMed

    Barresi, Valeria; Branca, Giovanni; Granata, Francesca; Alafaci, Concetta; Caffo, Maria; Tuccari, Giovanni

    2013-06-01

    Pre-operative embolization (POE) of meningiomas may induce histological changes which simulate malignancy, possibly resulting in overgrading. Aims of the present study were to identify clues to distinguish malignancy-related features from POE-related changes and to test for overgrading the grading scheme currently in use, in embolized meningiomas. In addition, we aimed to analyze whether the POE procedure may stimulate neo-angiogenesis in meningiomas. The histological features of a series of embolized meningiomas were evaluated and considered for grading assessment. In the same cases neo-angiogenesis was quantified by the evaluation of microvessel density (MVD) and correlated with the interval between POE and surgery. Necrosis and macronucleoli represented common findings in embolized meningiomas. Nonetheless, in most of the cases, necrosis showed an abrupt line of demarcation from the viable tumour tissue, and macronucleoli were restricted to peri-necrotic areas. Suggesting that these were POE-associated changes, exclusion of necrotic areas with an abrupt line of transition and focal macronucleoli from grading assessment resulted in increased specificity and positive predictive value in the identification of recurring meningiomas. In our cohort, MVD significantly increased with the time between POE and surgery, suggesting that POE procedure may induce neo-angiogenesis in meningiomas. In conclusion, a risk of overgrading there exists in embolized meningiomas, as a consequence of the frequent evidence of necrosis and prominent nucleoli in these tumours. In order to avoid overgrading, we suggest that necrosis showing an abrupt line of demarcation and focal peri-necrotic macronucleoli are not included in grading assessment. Also, caution should be used in the interpretation of MVD as a prognostic factor in embolized meningioma, as it may also result from POE procedure. PMID:23504284

  9. High incidence of meningioma among Hiroshima atomic bomb survivors.

    PubMed

    Shintani, T; Hayakawa, N; Hoshi, M; Sumida, M; Kurisu, K; Oki, S; Kodama, Y; Kajikawa, H; Inai, K; Kamada, N

    1999-03-01

    Since the atomic bomb explosions in Hiroshima and Nagasaki, high incidences of leukemia, thyroid cancer and other tumors have been reported as atomic bomb-induced tumors. We investigated the incidence of meningioma among Hiroshima atomic bomb survivors. Sixty-eight patients surgically treated for meningioma who had been within 2.0 km of the hypocenter of the explosion were identified. Six hundred and seven non-exposed patients with meningioma were also studied. Treatment dates were from 1975 to 1992. The incidences of meningioma among 68 subjects within 2.0 km and 607 non-exposed patients were 8.7 and 3.0 cases per 10(5) persons per year, respectively. The incidences of meningioma among the survivors of Hiroshima in 5-year intervals since 1975 were 5.3, 7.4, 10.1, and 14.9, respectively. The incidences of meningioma classified by distances from the hypocenter of 1.5-2.0 km, 1.0-1.5 km and less than 1.0 km were 6.3, 7.6 and 20.0, respectively. The incidences of meningioma classified by doses to the brain of 0-0.099 Sv, 0.1-0.99 Sv and more than 1.0 Sv were 7.7, 9.2 and 18.2, respectively. The incidence of meningioma among Hiroshima atomic bomb survivors has increased since 1975. There was a significant correlation between the incidence and the dose of radiation to the brain. The present findings strongly suggest that meningioma is one of the tumors induced by atomic bombing in Hiroshima. PMID:10408177

  10. Foramen Magnum Meningioma: a Case Report and Review of Literature

    PubMed Central

    Jurinovic, Pavao; Bulicic, Ana Repic; Marcic, Marino; Mise, Nikolina Ivica; Titlic, Marina; Suljic, Enra

    2016-01-01

    Introduction: Meningiomas are slow-growing benign tumors that arise at any location where arachnoid cells reside. Although meningiomas account for a sizable proportion of all primary intracranial neoplasms (14.3–19%), only 1.8 to 3.2% arise at the foramen magnum. Their indolent development at the craniocervical junction makes clinical diagnosis complex and often leads to a long interval between onset of symptoms and diagnosis. Case report: We report a case of a 79-year-old male patient, presented with ataxia and sense of threatening fainting during verticalization. Magnetic resonance imaging revealed the presence of meningioma in the right side of craniospinal junction. PMID:27041817