Science.gov

Sample records for high-grade pleomorphic sarcoma

  1. High-grade undifferentiated pleomorphic sarcoma of the pelvis: A case report and review of literature

    PubMed Central

    Agafonoff, Slava; Vaidya, Shrikant K.; DeFade, Brian

    2016-01-01

    High-grade spindle cell sarcomas are rare undifferentiated pleomorphic cancers that present a treatment challenge to urological practices, especially when they present in the pelvis. We report a 46-year-old male patient who presented to our urology clinic with urinary retention after having a Foley catheter placed at an outlying facility. A voiding trial was attempted, but the patient failed this trial. This failure resulted in cystoscopy with bilateral retrograde pyelograms, which revealed a compressed bladder due to extrinsic compression. This finding had been evaluated with a computed tomography (CT) scan with and without intravenous contrast that showed a 14 cm pelvic mass with bladder displacement and compression. A fine needle aspiration was done at this outlying facility, prior to referral to our office, and it confirmed spindle cell pathology. The mass was surgically excised with the histology revealing a pelvic spindle cell sarcoma with positive surgical margins. Further, metastatic work-up with CT/positron emission tomography revealed bone and lung metastasis. The patient is currently undergoing chemotherapy and radiation. In this case study, we will review staging, management, differential diagnosis, chemotherapy, and radiation. PMID:27453666

  2. An extremely rare case of a high-grade pleomorphic cardiac sarcoma and likely cerebral metastasis in a young patient

    PubMed Central

    Wilson, TG; Jenkins, P; Hoschtitzky, A; McCabe, M

    2016-01-01

    To date, there have been less than a 100 confirmed case reports of primary cardiac malignant fibrous histiocytomas, a rare form of sarcoma. In this report, we discuss the case of a 15-year-old girl who initially presented with a histiocytic cerebral sarcoma that was treated with aggressive resection and chemotherapy. Three years later, the same patient developed increasing shortness of breath and was found to have a high-grade pleomorphic undifferentiated cardiac sarcoma that likely represents the primary tumour from which the cerebral lesion metastasised. This represents an extremely unique case; in 2010, a research group in Germany claimed the very first description of a true cardiac sarcoma with brain metastasis [1]. However, even as far back as 1960, there were three case reports [2] and more extensive sarcoma studies recently have revealed further cases [3]. Nevertheless, there have probably been less than 10 cases in the literature up until this point. PMID:27594909

  3. An extremely rare case of a high-grade pleomorphic cardiac sarcoma and likely cerebral metastasis in a young patient.

    PubMed

    Wilson, T G; Jenkins, P; Hoschtitzky, A; McCabe, M

    2016-01-01

    To date, there have been less than a 100 confirmed case reports of primary cardiac malignant fibrous histiocytomas, a rare form of sarcoma. In this report, we discuss the case of a 15-year-old girl who initially presented with a histiocytic cerebral sarcoma that was treated with aggressive resection and chemotherapy. Three years later, the same patient developed increasing shortness of breath and was found to have a high-grade pleomorphic undifferentiated cardiac sarcoma that likely represents the primary tumour from which the cerebral lesion metastasised. This represents an extremely unique case; in 2010, a research group in Germany claimed the very first description of a true cardiac sarcoma with brain metastasis [1]. However, even as far back as 1960, there were three case reports [2] and more extensive sarcoma studies recently have revealed further cases [3]. Nevertheless, there have probably been less than 10 cases in the literature up until this point. PMID:27594909

  4. Pleomorphic malignant fibrous histiocytoma/undifferentiated high-grade pleomorphic sarcoma of the scrotum in a patient presenting as fournier gangrene: a case report.

    PubMed

    Guo, Juan; Zhou, Shengmei; Rao, Nagesh P; Pez, Gholam H

    2010-10-01

    Pleomorphic malignant fibrous histiocytoma (MFH), also known as undifferentiated high-grade pleomorphic sarcoma according to the latest World Health Organization classification, is a diagnosis of exclusion and extremely rare in adult scrotal/paratesticular region. Clinical presentation of scrotal/paratesticular pleomorphic MFH is usually a painless and gradual scrotal swelling. We report a case of scrotal MFH in a 63-year-old man who presented as Fournier gangrene after 10-month painful scrotal swelling and multiple procedures. The specimen of emergent debridement was submitted for pathologic and bacteriologic examination. Microscopically, the lesion had marked architectural and cytologic pleomorphism. The neoplastic cells were positive for vimentin, but negative for all lineage-specific markers. Fluorescence in-situ hybridization showed an aneuploid karyotype and negative results for lipomatous tumor abnormalities. Bacterial cultures of the specimen showed extensive growth of virulent polymicrobes. The diagnosis of scrotal/paratesticular pleomorphic MFH with concurrent Fournier gangrene was made. Thoracic computed tomography scan showed bilateral multiple pulmonary nodules. The patient died 1 month later. PMID:20505510

  5. [Undifferentiated high grade pleomorphic sarcoma/ malignant fibrous histiocytoma associated a gouty tophus. a case report].

    PubMed

    Szlabi, Susana; Flores, Jorge O; Diller, Ana L de; Cabrera, Marta

    2012-01-01

    Antecedentes: La gota es una enfermedad metabólica por depósito de cristales de ácido úrico, que compromete articulaciones y tejidos blandos tanto en sus etapas agudas como crónicas. Constituye un suceso poco común la aparición de un tumor en el sitio propio de la lesión. Objetivo: presentar un caso de asociación infrecuente entre tofo gotoso y sarcoma. Material y métodos: hombre de 83 años que consultó por tumoración en codo izquierdo de aproximadamente 40 años de evolución, que comenzó a doler espontáneamente. Con la presunción diagnóstica de tofo gotoso se trató quirúrgicamente. La lesión recidivó a los 60 días, se realizó reintervención y radioterapia por diagnóstico de tumor mesenquimal maligno asociado a tofo gotoso. A los 10 meses desarrolló metástasis ganglionar homolateral, falleció antes de los 2 años de la consulta inicial. Resultados: El diagnóstico de la primer biopsia fue tofo gotoso. En el material de reintervención se diagnosticó tofo gotoso asociado a neoplasia mesenquimal fusocelular de alto grado; la inmunohistoquímica reveló: vimentina +/+, MYO D1 -/-, ASMA -/-, FVIII -/-, A1ATT -/-, CD68-/-, S100-/- con resultado diagnóstico final de sarcoma pleomórfico indiferenciado de alto grado. Conclusión: Es infrecuente que los tofos gotosos se asocien a otras enfermedades y menos que lo hagan a tumores. En la bibliografía se han reportado tres casos previos concurrentes con neoplasias, las cuales fueron angiosarcoma, tumor de células gigantes y fibrohistiocitoma maligno. Estos últimos tienen una alta tendencia a recidivar y poseen capacidad de dar metástasis, especialmente a pulmones y ganglios regionales. Palabras clave: tofo gotoso, fibrohistiocitoma maligno, sarcoma pleomórfico indiferenciado.

  6. Primary and metastatic high-grade pleomorphic sarcoma/malignant fibrous histiocytoma of the gastrointestinal tract: an approach to the differential diagnosis in a series of five cases with emphasis on myofibroblastic differentiation.

    PubMed

    Agaimy, Abbas; Gaumann, Andreas; Schroeder, Josef; Dietmaier, Wolfgang; Hartmann, Arndt; Hofstaedter, Ferdinand; Wünsch, Peter H; Mentzel, Thomas

    2007-11-01

    Primary and metastatic so-called malignant fibrous histiocytoma/undifferentiated high-grade pleomorphic sarcoma (MFH) is rare in the gastrointestinal (GI) tract with approximately 50 primary and five metastatic cases reported so far. We evaluated two primary gastric and three metastatic intestinal high-grade pleomorphic sarcomas with features of storiform-pleomorphic MFH. Gastric tumours occurred in a 79-year-old man and a 68-year-old woman. One patient died post-operatively, and the other was disease-free at 6 months. Three patients presented with GI metastasis 24, 60 and 0 months after diagnosis of MFH of the heart (n = 1) and the thigh (n = 2). Metastases were located in the small (n = 1) and large bowel (n = 2) and were characteristically pedunculated and polypoid with oedematous haemorrhagic stroma. Concurrent metastases (brain, lung, bone) were present in all three cases. Tumours expressed alpha-smooth muscle actin (four of five), platelet-derived growth factor receptor (PDGFR) alpha (three of three) and PDGFRbeta (two of three) but were negative for CD117, CD34 and other lineage-specific markers. Ultrastructural examination revealed myo/fibroblastic features. Both gastric MFH were wild type for KIT and PDGFRalpha. In conclusion, primary and metastatic MFH of the GI tract commonly express PDGFRalpha and show a myo/fibroblastic phenotype. They should be distinguished from a variety of primary and metastatic pleomorphic neoplasms, in particular high-grade sarcomatous GI stromal tumours (GIST), pleomorphic leiomyosarcoma, sarcomatoid carcinoma and other mimics. PMID:17874130

  7. Sapanisertib or Pazopanib Hydrochloride in Treating Patients With Locally Advanced or Metastatic Sarcoma

    ClinicalTrials.gov

    2016-10-31

    High Grade Sarcoma; Metastatic Leiomyosarcoma; Metastatic Malignant Peripheral Nerve Sheath Tumor; Metastatic Synovial Sarcoma; Metastatic Undifferentiated Pleomorphic Sarcoma; Myxofibrosarcoma; Recurrent Leiomyosarcoma; Recurrent Malignant Peripheral Nerve Sheath Tumor; Recurrent Synovial Sarcoma; Recurrent Undifferentiated Pleomorphic Sarcoma; Uterine Corpus Leiomyosarcoma

  8. An unusual pleomorphic sarcoma in a hybrid mallard

    USGS Publications Warehouse

    Roffe, Thomas J.

    1987-01-01

    An unusual pleomorphic sarcoma from a hybrid mallard (Anas platyrhynchos) is described. Rhabdomyosarcoma was considered in the original differential diagnoses but rejected due to lack of specific characteristics generally seen in these tumors. The histologic characteristics described are consistent with mammalian sarcomas recorded in the literature as malignant fibrous histiocytoma.

  9. Mediastinal pleomorphic sarcoma in an immunodeficient patient: case report and review of the literature.

    PubMed

    Hernandez, Arlene; Gill, Farrukh Iqbal; Aventura, Emily; Mason, Carol; Shellito, Judd

    2012-01-01

    Pleomorphic sarcoma, widely known as malignant fibrous histiocytoma (MFH), is a soft tissue sarcoma. The occurrence of this malignancy in the mediastinum is rare. To our knowledge, only 13 cases of MFH of the mediastinum have been previously reported. Furthermore, only three cases of MFH in patients infected with human immunodeficiency virus (HIV) have been previously described. Here we present a 44-year-old African-American male who complained of epigastric pain radiating to the right chest. On admission, a chest radiograph revealed a widened mediastinum, and chest computerized tomography (CT) identified a large mass in the posterior mediastinum. Histologic diagnosis revealed a high-grade MFH. He was also incidentally diagnosed with HIV infection. The rarity of this malignancy and uncommon site of presentation in association with an immunodeficient state makes this case unique. This is the first report in the literature of an HIV-infected patient presenting with this uncommon tumor in the mediastinum. PMID:22533109

  10. PROLONGED FEVER WITHOUT OBVIOUS ETIOLOGY FINALLY DIAGNOSED AS HIGH-GRADE SOFT TISSUE SARCOMA.

    PubMed

    Doroftei, Nicoleta Alina; de Visscher, Nathalie

    2016-01-01

    Sarcomas represent a challenge t clinicians as they are rare and diagnosis is often delayed. Soft tissue sarcomas are relatively uncommon cancers, the incidence is estimated about 1% from all malignant tumors. Due to aggressive biological behavior of pleomorphic undifferentiated sarcoma diagnosis must hi made as quickly as possible. MRI is the standard imaging modality for investigating possibl3 malignant masses. Excisional biopsy appears to be necessary for differentiating soft tissus sarcoma from expanding hematoma. FDG PET scans may be helpful in specific circum. stances (as was the situation in our case, because the lesion is composed from two components: an area without precise edges and a necrotic-cystic area).

  11. Radiation-induced undifferentiated pleomorphic sarcoma after radiation therapy for a desmoid tumour.

    PubMed

    Di Marco, J; Kaci, R; Orcel, P; Nizard, R; Laredo, J-D

    2016-02-01

    Radiation-induced sarcoma is a long-term complication of radiation therapy. The most common secondary neoplasia is the undifferentiated pleomorphic sarcoma, which is usually described in the deep soft tissue of the trunk or extremities. Radiation-induced sarcomas have a poor prognosis. An early diagnosis and management are needed to improve the survival rate of such patients. We presently report a case of a radiation-induced undifferentiated pleomorphic sarcoma of the left gluteus maximus muscle, which developed 25 years after an initial diagnosis of aggressive fibromatosis and 21 years after a tumour recurrence. This case study illustrates the risk of developing a sarcoma in a radiation field and the need for long-term follow-up after radiation therapy. Unnecessary radiation therapy, in particular in the case of benign conditions in young patients, should be avoided.

  12. 14-3-3 fusion oncogenes in high-grade endometrial stromal sarcoma

    PubMed Central

    Lee, Cheng-Han; Ou, Wen-Bin; Mariño-Enriquez, Adrian; Zhu, Meijun; Mayeda, Mark; Wang, Yuexiang; Guo, Xiangqian; Brunner, Alayne L.; Amant, Frédéric; French, Christopher A.; West, Robert B.; McAlpine, Jessica N.; Gilks, C. Blake; Yaffe, Michael B.; Prentice, Leah M.; McPherson, Andrew; Jones, Steven J. M.; Marra, Marco A.; Shah, Sohrab P.; van de Rijn, Matt; Huntsman, David G.; Dal Cin, Paola; Debiec-Rychter, Maria; Nucci, Marisa R.; Fletcher, Jonathan A.

    2012-01-01

    14-3-3 proteins are ubiquitously expressed regulators of various cellular functions, including proliferation, metabolism, and differentiation, and altered 14-3-3 expression is associated with development and progression of cancer. We report a transforming 14-3-3 oncoprotein, which we identified through conventional cytogenetics and whole-transcriptome sequencing analysis as a highly recurrent genetic mechanism in a clinically aggressive form of uterine sarcoma: high-grade endometrial stromal sarcoma (ESS). The 14-3-3 oncoprotein results from a t(10;17) genomic rearrangement, leading to fusion between 14-3-3ε (YWHAE) and either of two nearly identical FAM22 family members (FAM22A or FAM22B). Expression of YWHAE–FAM22 fusion oncoproteins was demonstrated by immunoblot in t(10;17)-bearing frozen tumor and cell line samples. YWHAE–FAM22 fusion gene knockdowns were performed with shRNAs and siRNAs targeting various FAM22A exons in an t(10;17)-bearing ESS cell line (ESS1): Fusion protein expression was inhibited, with corresponding reduction in cell growth and migration. YWHAE–FAM22 maintains a structurally and functionally intact 14-3-3ε (YWHAE) protein-binding domain, which is directed to the nucleus by a FAM22 nuclear localization sequence. In contrast to classic ESS, harboring JAZF1 genetic fusions, YWHAE–FAM22 ESS display high-grade histologic features, a distinct gene-expression profile, and a more aggressive clinical course. Fluorescence in situ hybridization analysis demonstrated absolute specificity of YWHAE–FAM22A/B genetic rearrangement for high-grade ESS, with no fusions detected in other uterine and nonuterine mesenchymal tumors (55 tumor types, n = 827). These discoveries reveal diagnostically and therapeutically relevant models for characterizing aberrant 14-3-3 oncogenic functions. PMID:22223660

  13. Congenital high-grade sarcoma presenting as skin nodules and respiratory distress in a neonate.

    PubMed

    Powers, J W; Teitell, M; Milisavljevic, V

    2008-02-01

    We report, to our knowledge, the first case of a congenital, widespread, aggressive high-grade sarcoma, presented as multiple skin nodules and respiratory distress in a neonate that had a t(9;22)(q22;q11-12) cytogenetic abnormality suggestive of a more indolent extraskeletal myxoid chondrosarcoma (EMC). EMC is generally thought of as a slow-growing tumor that presents between the fourth and sixth decades of life. Our patient was a 45,XY, t(13;14) newborn who presented at birth with subcutaneous nodules involving the face, scalp, back and extremities, as well as multiple intrathoracic, intraabdominal and intracranial masses. Diagnosis was made using electron microscopy and immunohistochemical and cytogenetic studies. Despite attempts to control rapid growth of lesions using high-dose steroids and cis-retinoic acid, patient's clinical status continued to deteriorate and life support was withdrawn at the 26 day of life.

  14. High-grade endometrial stromal sarcoma presenting in a 28-year-old woman during pregnancy: a case report

    PubMed Central

    2010-01-01

    Introduction To the best of our knowledge, soft tissue sarcomas have not prevously been reported as a complication during pregnancy. Case presentation A 28-year-old Caucasian woman was diagnosed with a transperitoneal sarcoma during pregnancy. Morphological, immunohistochemical, chromosomal and mutational analyses pointed towards a high-grade endometrial stromal sarcoma. Although surgery and chemotherapy are possible during pregnancy, we were unable to perform these in this case. Conclusion The potential to treat gynecological cancer during pregnancy should always be assessed individually. PMID:20684773

  15. Kras activation in p53-deficient myoblasts results in high-grade sarcoma formation with impaired myogenic differentiation

    PubMed Central

    McKinnon, Timothy; Venier, Rosemarie; Dickson, Brendan C.; Kabaroff, Leah; Alkema, Manon; Chen, Li; Shern, Jack F.; Yohe, Marielle E.; Khan, Javed; Gladdy, Rebecca A.

    2015-01-01

    While genomic studies have improved our ability to classify sarcomas, the molecular mechanisms involved in the formation and progression of many sarcoma subtypes are unknown. To better understand developmental origins and genetic drivers involved in rhabdomyosarcomagenesis, we describe a novel sarcoma model system employing primary murine p53-deficient myoblasts that were isolated and lentivirally transduced with KrasG12D. Myoblast cell lines were characterized and subjected to proliferation, anchorage-independent growth and differentiation assays to assess the effects of transgenic KrasG12D expression. KrasG12D overexpression transformed p53−/− myoblasts as demonstrated by an increased anchorage-independent growth. Induction of differentiation in parental myoblasts resulted in activation of key myogenic regulators. In contrast, Kras-transduced myoblasts had impaired terminal differentiation. p53−/− myoblasts transformed by KrasG12D overexpression resulted in rapid, reproducible tumor formation following orthotopic injection into syngeneic host hindlimbs. Pathological analysis revealed high-grade sarcomas with myogenic differentiation based on the expression of muscle-specific markers, such as Myod1 and Myog. Gene expression patterns of murine sarcomas shared biological pathways with RMS gene sets as determined by gene set enrichment analysis (GSEA) and were 61% similar to human RMS as determined by metagene analysis. Thus, our novel model system is an effective means to model high-grade sarcomas along the RMS spectrum. PMID:25992772

  16. Xenograft models for undifferentiated pleomorphic sarcoma not otherwise specified are essential for preclinical testing of therapeutic agents

    PubMed Central

    Becker, Marc; Graf, Claudine; Tonak, Marcus; Radsak, Markus P.; Bopp, Tobias; Bals, Robert; Bohle, Rainer M.; Theobald, Matthias; Rommens, Pol-Maria; Proschek, Dirk; Wehler, Thomas C.

    2016-01-01

    Undifferentiated pleomorphic sarcoma not otherwise specified belongs to the heterogeneous group of soft tissue tumors. It is preferentially located in the upper and lower extremities of the body, and surgical resection remains the only curative treatment. Preclinical animal models are crucial to improve the development of novel chemotherapeutic agents for the treatment of undifferentiated pleomorphic sarcoma. However, this approach has been hampered by the lack of reproducible animal models. The present study established two xenograft animal models generated from stable non-clonal cell cultures, and investigated the difference in chemotherapeutic effects on tumor growth between undifferentiated pleomorphic sarcoma in vivo and in vitro. The cell cultures were generated from freshly isolated tumor tissues of two patients with undifferentiated pleomorphic sarcoma. For the in vivo analysis, these cells were injected subcutaneously into immunodeficient mice. The mice were monitored for tumor appearance and treated with the most common or innovative chemotherapeutic agents available to date. Furthermore, the same drugs were administered to in vitro cell cultures. The most effective tumor growth inhibition in vitro was observed with doxorubicin and the histone deacetylase inhibitor suberoylanilide hydroxamic acid (SAHA), also known as vorinostat. In the in vivo xenograft mouse model, the combination of doxorubicin and the tyrosine kinase inhibitor pazopanib induced a significant tumor reduction. By contrast, treatment with vorinostat did not reduce the tumor growth. Taken together, the results obtained from drug testing in vitro differed significantly from the in vivo results. Therefore, the novel and reproducible xenograft animal model established in the present study demonstrated that in vivo models are required to test potential chemotherapeutic agents for the treatment of undifferentiated pleomorphic sarcoma prior to clinical use, since animal models are more similar

  17. Carcinoma ex-pleomorphic adenoma of the parotid gland consisting of high-grade salivary duct carcinoma and keratinizing squamous cell carcinoma.

    PubMed

    Magaki, Shino D; Bhuta, Sunita; Abemayor, Elliot; Nabili, Vishad; Sepahdari, Ali R; Lai, Chi K

    2015-09-01

    Carcinoma ex-pleomorphic adenoma (CXPA) is a rare salivary gland malignancy that presents diagnostic difficulties partly because of its wide range of histologic presentations. We report a case of a 77-year-old man, who presented with a 6-year history of a parotid mass that had undergone rapid growth within weeks. Magnetic resonance imaging revealed an infiltrative mass in the parotid gland, and the fine-needle aspiration (FNA) biopsy result was highly suspicious for carcinoma. Subsequent excision of the tumor demonstrated a poorly differentiated epithelial neoplasm consisting of keratinizing squamous cell carcinoma (SCC) and adenocarcinoma with regions of both ductal carcinoma in situ and invasive salivary duct carcinoma (SDC). Only focal areas exhibited a benign pleomorphic adenoma component. To our knowledge, this is the first case of a CXPA that consists of both a high-grade SDC and a keratinizing SCC in the parotid gland.

  18. Case of Pleomorphic Dermal Sarcoma of the Eyelid Treated with Micrographic Surgery and Secondary Intention Healing

    PubMed Central

    Kim, Jung-In; Choi, Young-Jun; Seo, Hyun-Min; Kim, Han-Saem; Lim, Jae Yun; Kim, Dong-Hoon; Chae, Seoung Wan; Lee, Ga-Young

    2016-01-01

    Pleomorphic dermal sarcoma (PDS) is a rare mesenchymal neoplasm sharing histopathological features with atypical fibroxanthoma (AFX), but has additional features of deep invasion of the superficial subcutis, tumor necrosis and vascular/perineural invasion. It is not well documented in the literature because of its rarity, and its clinical course has been debated due to the lack of homogenous criteria. We describe here the case of a 91-year-old female with a 6-month history of a solitary, asymptomatic, well-defined, 3.4-cm-sized, reddish, hard, protruding mass on the lateral aspect of the right upper eyelid. On the basis of initial punch biopsy results, storiform cellular infiltrate of pleomorphic spindle and polygonal cells with frequent atypical mitoses, the lesion was identified as AFX. Following the initial biopsy, micrographic surgery was performed and a tumor-free margin was confirmed. Considering the conservation of the periocular function and the advanced age of the patient, we planned secondary intention healing rather than primary suturing. After surgery, skeletal muscle infiltration was found and the diagnosis was revised to PDS by a pathologist based on the currently accepted criteria for PDS. There has been no evidence of recurrence or periocular functional defects during a 2-year follow-up without adjuvant therapy. Although the PDS is highly malignant, complete excision under micrographic surgery can prevent recurrence without adjuvant therapy. Also, the secondary intention healing is an effective method for closure of large defects on the face. PMID:27746645

  19. Predictors of local recurrence in high-grade soft tissue sarcomas: hydrogen peroxide as a local adjuvant.

    PubMed

    Wooldridge, Adam N; Kolovich, Gregory P; Crist, Martha K; Mayerson, Joel L; Scharschmidt, Thomas J

    2013-02-01

    Soft tissue sarcomas have a mortality rate of 40% to 60%, with local recurrence being a poor prognostic factor for overall survival. Three-percent nondiluted hydrogen peroxide is hypothesized to be an effective local adjuvant. The purpose of this study was to identify risk factors for local recurrence in high-grade soft tissue sarcomas and to determine whether using hydrogen peroxide as a local adjuvant reduced the risks of local recurrence and surgical-site infection. Retrospective data were collected for 106 patients surgically treated for high-grade soft tissue sarcomas between 2002 and 2010. The primary endpoint was local recurrence. Eighteen (16.98%) cases of local recurrence occurred. Predictors of local recurrence were margin status, estimated blood loss, and histology (ie, malignant peripheral nerve sheath tumor), with hazard ratios of 4.44 (95% confidence interval [CI], 1.32-14.95), 1.19 (95% CI, 1.06-1.35), and 9.21 (95% CI, 2.11-40.16), respectively. Hydrogen peroxide yielded a statistically insignificant improvement in local recurrence, with a hazard ratio of 0.81 (95% CI, 0.27-2.48) and a reduced risk of surgical site infection, with a hazard ratio of 0.52 (95% CI, 0.15-1.81). Margin status, increased blood loss, and histologic subtype are associated with increased local recurrence risk. Using hydrogen peroxide improved local control and infection rates, but the difference was not statistically significant.

  20. Immunophenotype and K-RAS mutation in mucinous ovarian adenocarcinoma with mural nodule of high-grade sarcoma: case report.

    PubMed

    Desouki, Mohamed M; Fadare, Oluwole; Kanbour, Anisa; Kanbour-Shakir, Amal

    2014-03-01

    Ovarian mucinous tumors with mural nodules are rare. The mural nodules are microscopically divergent neoplasms of varying sizes that may be benign (eg, sarcoma-like and carcinosarcoma-like), or malignant (eg, anaplastic carcinoma and sarcoma). The K-RAS gene mutation in ovarian mucinous neoplasms with mural nodules has not been previously reported. This is a case report of a 25-year-old female diagnosed with ovarian invasive mucinous adenocarcinoma with mural nodule of high-grade sarcoma. The mucinous tumor component demonstrated a K-RAS codon 12/13 mutation (p.G12V, c.35 G>T), whereas the sarcomatous component demonstrated a K-RAS codon 12/13 mutation (p.G12D, c.35 G>A). Although both tumor components revealed a mutation in codon 12 of K-RAS, they were of different nucleotide substitutions, indicating that these 2 tumor components were of different clonal origins. However, the fact that the 2 mutations identified in the tumor components are the most common mutations reported in mucinous tumors of the ovary, raises the possibility that sarcomatous mural nodules simply represent a form of dedifferentiation in mucinous tumors.

  1. Oncogene and therapeutic target analyses in atypical fibroxanthomas and pleomorphic dermal sarcomas

    PubMed Central

    Pütz, Katharina; Tantcheva-Poor, Iliana; Mauch, Cornelia; Büttner, Reinhard; Quaas, Alexander

    2016-01-01

    Background Until now, almost nothing is known about the tumorigenesis of atypical fibroxanthoma (AFX) and pleomorphic dermal sarcoma (PDS). Our hypothesis is that AFX is the non-infiltrating precursor lesion of PDS. Materials and Methods We performed the world-wide most comprehensive immunohistochemical and mutational analysis in well-defined AFX (n=5) and PDS (n=5). Results In NGS-based mutation analyses of selected regions by a 17 hotspot gene panel of 102 amplicons we could detect TP53 mutations in all PDS as well as in the only analyzed AFX and PDS of the same patient. Besides, we detected mutations in the CDKN2A, HRAS, KNSTRN and PIK3CA genes. Performing immunohistochemistry for CTNNB1, KIT, CDK4, c-MYC, CTLA-4, CCND1, EGFR, EPCAM, ERBB2, IMP3, INI-1, MKI67, MDM2, MET, p40, TP53, PD-L1 and SOX2 overexpression of TP53, CCND1 and CDK4 was seen in AFX as well as in PDS. IMP3 was upregulated in 2 AFX (weak staining) and 4 PDS (strong staining). FISH analyses for the genes FGFR1, FGFR2 and FGFR3 revealed negative results in all tumors. Conclusions UV-induced TP53 mutations as well as CCND1/CDK4 changes seem to play essential roles in tumorigenesis of PDS. Furthermore, we found some more interesting mutated genes in other oncogene pathways (activating mutations of HRAS and PIK3CA). All AFX and PDS investigated immunohistochemically presented with similar oncogene expression profiles (TP53, CCND1, CDK4 overexpression) and the single case with an AFX and PDS showed complete identical TP53 and PIK3CA mutation profiles in both tumors. This reinforces our hypothesis that AFX is the non-infiltrating precursor lesion of PDS. PMID:26943575

  2. Analysis of Clinical and Molecular Factors Impacting Oncologic Outcomes in Undifferentiated Pleomorphic Sarcoma

    PubMed Central

    Roland, Christina L.; May, Caitlin D.; Watson, Kelsey L.; Al Sannaa, Ghadah A.; Dineen, Sean P.; Feig, Rachel; Landers, Sharon; Ingram, Davis R.; Wang, Wei-Lien; Guadagnolo, B. Ashleigh; Feig, Barry; Hunt, Kelly K.; Cormier, Janice N.; Lazar, Alexander; Torres, Keila E.

    2016-01-01

    Background Undifferentiated pleomorphic sarcomas (UPS) present a diagnostic and therapeutic challenge. Identification of prognostic molecular markers is required for the discovery of novel treatment approaches. The aim of this study was to correlate clinicopathologic variables, expression of tyrosine kinase receptors, and markers of cell cycle progression and survival with oncologic outcomes. Methods A tissue microarray containing 208 primary UPS samples was analyzed by immunohistochemistry for protein markers and in situ hybridization for microRNA. Staining results were correlated with clinicopathologic features and oncologic outcomes. Univariate and multivariate analyses were conducted to assess associations between expression of protein markers, mi-RNA and outcome. Results At a median follow-up of 3.9 years (9 years for survivors), 5-year disease-specific survival (DSS) was 63%. Clinical variables associated with improved DSS included age < 61 years, tumor size < 10 cm, margin-negative resection and sporadic-tumor status. At the protein level, loss of cyclin D1 (p=0.06), pEGFR (p=0.023), pIGF-1R (p=0.022), and PTEN (p<0.001) and overexpression of AXL (p=0.015) were associated with reduced DSS on univariate analysis. Ki67, PCNA and pEGFR were more highly expressed in sporadic UPS than radiation-associated (RA-UPS) while RA-UPS samples expressed higher levels of both phosphorylated and total IGF-1R. Discussion Cyclin D1, AXL and PTEN are associated with cancer-specific outcomes and warrant further investigation in UPS. The differences in protein expression in sporadic versus RA-UPS may indicate that the activated molecular signaling nodes may be different for each specific histology and could also explain the aggressive phenotype seen in RA-UPS when compared to the sporadic lesions. PMID:26847678

  3. Gene expression identifies heterogeneity of metastatic behavior among high-grade non-translocation associated soft tissue sarcomas

    PubMed Central

    2014-01-01

    Background The biologic heterogeneity of soft tissue sarcomas (STS), even within histological subtypes, complicates treatment. In earlier studies, gene expression patterns that distinguish two subsets of clear cell renal carcinoma (RCC), serous ovarian carcinoma (OVCA), and aggressive fibromatosis (AF) were used to separate 73 STS into two or four groups with different probabilities of developing metastatic disease (PrMet). This study was designed to confirm our earlier observations in a larger independent data set. Methods We utilized these gene sets, hierarchical clustering (HC), and Kaplan-Meier analysis, to examine 309 STS, using Affymetrix chip expression profiling. Results HC using the combined AF-, RCC-, and OVCA-gene sets identified subsets of the STS samples. Analysis revealed differences in PrMet between the clusters defined by the first branch point of the clustering dendrogram (p = 0.048), and also among the four different clusters defined by the second branch points (p < 0.0001). Analysis also revealed differences in PrMet between the leiomyosarcomas (LMS), dedifferentiated liposarcomas (LipoD), and undifferentiated pleomorphic sarcomas (UPS) (p = 0.0004). HC of both the LipoD and UPS sample sets divided the samples into two groups with different PrMet (p = 0.0128, and 0.0002, respectively). HC of the UPS samples also showed four groups with different PrMet (p = 0.0007). HC found no subgroups of the LMS samples. Conclusions These data confirm our earlier studies, and suggest that this approach may allow the identification of more than two subsets of STS, each with distinct clinical behavior, and may be useful to stratify STS in clinical trials and in patient management. PMID:24950699

  4. Primary Mesenteric Undifferentiated Pleomorphic Sarcoma Masquerading as a Colon Carcinoma: A Case Report and Review of the Literature

    PubMed Central

    Diaz-Beveridge, Robert; Melian, Marcos; Zac, Carlos; Navarro, Edwin; Akhoundova, Dilara; Chrivella, Melitina; Aparicio, Jorge

    2015-01-01

    Undifferentiated pleomorphic sarcoma (UPS) is the most common sarcoma that appears in older patients, usually in the extremities and the retroperitoneum. Other locations are rare. By definition, in UPS, although the malignant cells tend to appear fibroblastic or myofibroblastic, they should not show differentiation towards a more specific line of differentiation. In this sense, we report the case of an 80-year-old patient with an initial clinical diagnosis of a locally advanced colonic neoplasm that was later confirmed as a primary mesenteric UPS. Primary mesenteric UPS are extremely rare with less than 20 cases reported. We also review the pathologic and radiologic diagnostic criteria and the natural history of these tumours. PMID:26380135

  5. Complete response after rechallenge with trabectedin in a patient with previously responding high-grade undifferentiated sarcoma

    PubMed Central

    Di Donato, Samantha; Fargnoli, Rossana; Dona, Manjola; Bertulli, Rossella; Parisi, Elisabetta; Fantini, Lorenzo; Sbaraglia, Marta; Panella, Mauro

    2016-01-01

    Evidence supporting rechallenge in patients responding to first exposure to trabectedin is limited. We report on a 39-year-old woman with advanced high-grade undifferentiated sarcoma (US) retreated twice with trabectedin after first response. The patient presented in June 2006 with an abdominal mass originating from the rear fascia of the rectus abdominis. Staging examinations did not indicate metastases and she underwent surgery; pathology showed a high-grade (FNCLCC G3) US. Subsequently, the patient received five cycles of adjuvant chemotherapy with epirubicin and ifosfamide. In February 2009 a computed tomography (CT) scan showed an abdominal mass involving the transverse mesocolon. R0 surgery was performed. In September 2009, peritoneal lesions appeared. Trabectedin was initiated at a dose of 1.5 mg/m2 by a 24 h intravenous infusion every 3 weeks, without relevant toxicity. After six cycles (March 2010), CT and PET-CT scans showed complete disappearance of metastases. In February 2012, new secondary lesions in the subdiaphragmatic region and a peritoneal lesion appeared. We rechallenged the patient with the same schedule of trabectedin; a complete response was achieved after two cycles. In October 2013, new secondary lesions in the subdiaphragmatic region and a retroperitoneal lesion were found. We rechallenged with the same schedule of trabectedin; PET-CT scans after two cycles showed complete response on the subdiaphragmatic lesion. Radiotherapy on the retroperitoneal lesion was performed. The patient underwent a total of 18 cycles and remains free from radiologically detectable disease. We report complete radiological remission after two rechallenges with trabectedin in a patient with previously responding high-grade US. PMID:27348763

  6. Cixutumumab and Doxorubicin Hydrochloride in Treating Patients With Unresectable, Locally Advanced, or Metastatic Soft Tissue Sarcoma

    ClinicalTrials.gov

    2016-05-16

    Adult Angiosarcoma; Adult Desmoplastic Small Round Cell Tumor; Adult Epithelioid Sarcoma; Adult Extraskeletal Myxoid Chondrosarcoma; Adult Extraskeletal Osteosarcoma; Adult Fibrosarcoma; Adult Leiomyosarcoma; Adult Liposarcoma; Adult Malignant Mesenchymoma; Adult Malignant Peripheral Nerve Sheath Tumor; Adult Rhabdomyosarcoma; Adult Synovial Sarcoma; Adult Undifferentiated High Grade Pleomorphic Sarcoma of Bone; Childhood Angiosarcoma; Childhood Desmoplastic Small Round Cell Tumor; Childhood Epithelioid Sarcoma; Childhood Fibrosarcoma; Childhood Leiomyosarcoma; Childhood Liposarcoma; Childhood Malignant Mesenchymoma; Childhood Malignant Peripheral Nerve Sheath Tumor; Childhood Pleomorphic Rhabdomyosarcoma; Childhood Rhabdomyosarcoma With Mixed Embryonal and Alveolar Features; Childhood Synovial Sarcoma; Dermatofibrosarcoma Protuberans; Malignant Adult Hemangiopericytoma; Malignant Childhood Hemangiopericytoma; Metastatic Childhood Soft Tissue Sarcoma; Previously Treated Childhood Rhabdomyosarcoma; Recurrent Adult Soft Tissue Sarcoma; Recurrent Childhood Rhabdomyosarcoma; Recurrent Childhood Soft Tissue Sarcoma; Stage III Adult Soft Tissue Sarcoma; Stage IV Adult Soft Tissue Sarcoma; Untreated Childhood Rhabdomyosarcoma

  7. EF5 to Evaluate Tumor Hypoxia in Patients With High-Grade Soft Tissue Sarcoma or Mouth Cancer

    ClinicalTrials.gov

    2013-01-15

    Stage I Adult Soft Tissue Sarcoma; Stage I Squamous Cell Carcinoma of the Lip and Oral Cavity; Stage II Adult Soft Tissue Sarcoma; Stage II Squamous Cell Carcinoma of the Lip and Oral Cavity; Stage III Adult Soft Tissue Sarcoma; Stage III Squamous Cell Carcinoma of the Lip and Oral Cavity

  8. Prognostic microRNAs modulate the RHO adhesion pathway: A potential therapeutic target in undifferentiated pleomorphic sarcomas.

    PubMed

    Wong, Philip; Hui, Angela; Su, Jie; Yue, Shijun; Haibe-Kains, Benjamin; Gokgoz, Nalan; Xu, Wei; Bruce, Jeff; Williams, Justin; Catton, Charles; Wunder, Jay S; Andrulis, Irene L; Gladdy, Rebecca; Dickson, Brendan; O'Sullivan, Brian; Liu, Fei-Fei

    2015-11-17

    A common and aggressive subtype of soft-tissue sarcoma, undifferentiated pleomorphic sarcoma (UPS) was examined to determine the role of micro-RNAs (miRNAs) in modulating distant metastasis. Following histopathologic review, 110 fresh frozen clinically annotated UPS samples were divided into two independent cohorts for Training (42 patients), and Validation (68 patients) analyses. Global miRNA profiling on the Training Set and functional analysis in vitro suggested that miRNA-138 and its downstream RHO-ROCK cell adhesion pathway was a convergent target of miRNAs associated with the development of metastasis. A six-miRNA signature set prognostic of distant metastasis-free survival (DMFS) was developed from Training Set miRNA expression values. Using the six-miRNA signature, patients were successfully categorized into high- and low-risk groups for DMFS in an independent Validation Set, with a hazard ratio (HR) of 2.25 (p = 0.048). After adjusting for other known prognostic variables such as age, gender, tumor grade, size, depth, and treatment with radiotherapy, the six-miRNA signature retained prognostic value with a HR of 3.46 (p < 0.001). A prognostic miRNA biomarker for clinical validation was thus identified along with a functional pathway that modulates UPS metastatic phenotype. PMID:25970788

  9. Doxorubicin With Upfront Dexrazoxane for the Treatment of Advanced or Metastatic Soft Tissue Sarcoma

    ClinicalTrials.gov

    2016-07-11

    Sarcoma, Soft Tissue; Soft Tissue Sarcoma; Undifferentiated Pleomorphic Sarcoma; Leiomyosarcoma; Liposarcoma; Synovial Sarcoma; Myxofibrosarcoma; Angiosarcoma; Fibrosarcoma; Malignant Peripheral Nerve Sheath Tumor; Epithelioid Sarcoma

  10. Radiation Therapy With or Without Combination Chemotherapy or Pazopanib Hydrochloride Before Surgery in Treating Patients With Newly Diagnosed Non-Rhabdomyosarcoma Soft Tissue Sarcomas That Can Be Removed by Surgery

    ClinicalTrials.gov

    2016-11-04

    Adult Fibrosarcoma; Alveolar Soft Part Sarcoma; Angiomatoid Fibrous Histiocytoma; Atypical Fibroxanthoma; Clear Cell Sarcoma of Soft Tissue; Epithelioid Malignant Peripheral Nerve Sheath Tumor; Epithelioid Sarcoma; Extraskeletal Myxoid Chondrosarcoma; Extraskeletal Osteosarcoma; Fibrohistiocytic Neoplasm; Glomus Tumor of the Skin; Inflammatory Myofibroblastic Tumor; Intimal Sarcoma; Leiomyosarcoma; Liposarcoma; Low Grade Fibromyxoid Sarcoma; Low Grade Myofibroblastic Sarcoma; Malignant Cutaneous Granular Cell Tumor; Malignant Peripheral Nerve Sheath Tumor; Malignant Triton Tumor; Mesenchymal Chondrosarcoma; Myxofibrosarcoma; Myxoid Chondrosarcoma; Myxoinflammatory Fibroblastic Sarcoma; Nerve Sheath Neoplasm; PEComa; Pericytic Neoplasm; Plexiform Fibrohistiocytic Tumor; Sclerosing Epithelioid Fibrosarcoma; Stage IB Soft Tissue Sarcoma; Stage IIB Soft Tissue Sarcoma; Stage III Soft Tissue Sarcoma; Stage IV Soft Tissue Sarcoma; Synovial Sarcoma; Undifferentiated (Embryonal) Sarcoma; Undifferentiated High Grade Pleomorphic Sarcoma of Bone

  11. Undifferentiated Pleomorphic Sarcoma Presenting as Abdominal Pain with a Pulsatile Mass.

    PubMed

    Moradi, Arash; Afsharfard, Abolfazl; Atqiaee, Khashayar

    2016-01-01

    Malignant fibrous histiocytoma (MFH) is a rare tumor that mostly involves adults aged 50 to 70. The most common anatomic location is the lower extremities. MFH of the retroperitoneum usually manifests late in its course and may be initially mistaken with other more common diagnosis. Here, the authors describe a 60-year-old man that was brought to the emergency department with a chief complaint of periumbilical abdominal pain. Our patient presented with symptoms consistent with a symptomatic aortic aneurysm, but a mass was encountered during surgery. In such circumstances the diagnosis of malignant sarcoma must be kept in mind and attempts at full resection with tumor-free margins are necessary. PMID:27563479

  12. Undifferentiated Pleomorphic Sarcoma Presenting as Abdominal Pain with a Pulsatile Mass

    PubMed Central

    Afsharfard, Abolfazl

    2016-01-01

    Malignant fibrous histiocytoma (MFH) is a rare tumor that mostly involves adults aged 50 to 70. The most common anatomic location is the lower extremities. MFH of the retroperitoneum usually manifests late in its course and may be initially mistaken with other more common diagnosis. Here, the authors describe a 60-year-old man that was brought to the emergency department with a chief complaint of periumbilical abdominal pain. Our patient presented with symptoms consistent with a symptomatic aortic aneurysm, but a mass was encountered during surgery. In such circumstances the diagnosis of malignant sarcoma must be kept in mind and attempts at full resection with tumor-free margins are necessary. PMID:27563479

  13. Array comparative genomic hybridization reveals frequent alterations of G1/S checkpoint genes in undifferentiated pleomorphic sarcoma of bone.

    PubMed

    Niini, Tarja; Lahti, Leo; Michelacci, Francesca; Ninomiya, Shinsuke; Hattinger, Claudia Maria; Guled, Mohamed; Böhling, Tom; Picci, Piero; Serra, Massimo; Knuutila, Sakari

    2011-05-01

    Undifferentiated pleomorphic sarcoma of bone (UPSb) is a rare tumor often difficult to differentiate from fibrosarcoma of bone (FSb), diagnostically. We applied array comparative genomic hybridization (array CGH) to screen for genes with potential importance in the tumor and compared the results with alterations seen in FSb. Twenty-two fresh frozen tissue specimens from 20 patients (18 primary tumors and 4 local recurrences) with UPSb were studied. DNA was isolated and hybridized onto Agilent 244K CGH oligoarrays. The hybridization data were analyzed using Agilent DNA Analytics Software. The number of changes ranged from 2 to 168 (average = 66). Losses were most frequently seen at 8p, 9p, 10, 13q, and 18q, and gains at 4q, 5p, 6p, 7p, 8q, 12p, 14q, 17q, 19p, 20q, 22q, and X. Homozygous deletions of CDKN2A, RB1, TP53, and ING1 were seen in 8/20, 7/20, 3/20, and 2/20 cases, respectively. Hypermethylation of both p16(INK4a) and p14(ARF) was found in two cases with loss at CDKN2A. Inactivation either of CDKN2A, RB1, or TP53 was detected in 18/20 cases. One case showed high level gains of CDK4 and MDM2. Frequent gains were seen at MYC, PDGFRA, KIT, and KDR. Immunohistochemical positivity of KIT, PDGFRA, KDR, and PDGFRB was found in 8/14, 5/14, 4/14, and 4/14 cases, respectively. The regions most significantly discriminating between UPSb and FSb included RB1 and MYC. No homozygous deletions of RB1 were found in FSb. In conclusion, our analysis showed the disruption of G1/S checkpoint regulation to be crucial for the oncogenesis of UPSb.

  14. Disseminated pleomorphic myofibrosarcoma in a grizzly bear (Ursus arctos horribilis).

    PubMed

    Mete, A; Woods, L; Famini, D; Anderson, M

    2012-01-01

    The pathological and diagnostic features of a widely disseminated pleomorphic high-grade myofibroblastic sarcoma are described in a 23-year-old male brown bear (Ursus arctos horribilis). Firm, solid, white to tan neoplastic nodules, often with cavitated or soft grey-red necrotic centres, were observed throughout most internal organs, subcutaneous tissues and skeletal muscles on gross examination. Microscopically, the tumour consisted of pleomorphic spindle cells forming interlacing fascicles with a focal storiform pattern with large numbers of bizarre polygonal multinucleate cells, frequently within a collagenous stroma. Immunohistochemistry, Masson's trichrome stain and transmission electron microscopy designated the myofibroblast as the cell of origin. This is the first case of a high-grade myofibrosarcoma in a grizzly bear.

  15. Disseminated pleomorphic myofibrosarcoma in a grizzly bear (Ursus arctos horribilis).

    PubMed

    Mete, A; Woods, L; Famini, D; Anderson, M

    2012-01-01

    The pathological and diagnostic features of a widely disseminated pleomorphic high-grade myofibroblastic sarcoma are described in a 23-year-old male brown bear (Ursus arctos horribilis). Firm, solid, white to tan neoplastic nodules, often with cavitated or soft grey-red necrotic centres, were observed throughout most internal organs, subcutaneous tissues and skeletal muscles on gross examination. Microscopically, the tumour consisted of pleomorphic spindle cells forming interlacing fascicles with a focal storiform pattern with large numbers of bizarre polygonal multinucleate cells, frequently within a collagenous stroma. Immunohistochemistry, Masson's trichrome stain and transmission electron microscopy designated the myofibroblast as the cell of origin. This is the first case of a high-grade myofibrosarcoma in a grizzly bear. PMID:22297075

  16. Talimogene Laherparepvec and Radiation Therapy in Treating Patients With Newly Diagnosed Soft Tissue Sarcoma That Can Be Removed by Surgery

    ClinicalTrials.gov

    2016-10-04

    Leiomyosarcoma; Liposarcoma; Sarcoma Differentiation Score 2; Sarcoma Differentiation Score 3; Stage IA Soft Tissue Sarcoma; Stage IB Soft Tissue Sarcoma; Stage IIA Soft Tissue Sarcoma; Stage IIB Soft Tissue Sarcoma; Undifferentiated Pleomorphic Sarcoma

  17. High-grade sarcomatous overgrowth in solitary fibrous tumors: a clinicopathologic study of 10 cases.

    PubMed

    Collini, Paola; Negri, Tiziana; Barisella, Marta; Palassini, Elena; Tarantino, Eva; Pastorino, Ugo; Gronchi, Alessandro; Stacchiotti, Silvia; Pilotti, Silvana

    2012-08-01

    We describe 10 solitary fibrous tumors (SFT) with high-grade malignant overgrowth, all of which showed the presence of a synchronous or previous classic SFT/malignant SFT (MSFT) component. Seven were "dedifferentiated," with an abrupt transition from a classic SFT/MSFT to a high-grade component consisting of a nondistinctive high-grade sarcoma in 4 cases and divergent differentiation in 3. The nondistinctive high-grade component consisted of epithelioid and/or spindle cells often associated with overt pleomorphism or small round cell sarcomas. The divergent differentiation featured a rhabdomyosarcoma in 2 cases and an osteosarcoma in 1. Three cases (tentatively called "evolved") showed a gradual transition from classic SFT/MSFT to a nondistinctive high-grade sarcoma or presented features of high-grade sarcoma at the time of metastasis (assessed by fine-needle aspiration cytology) without any component suggesting a diagnosis of classic SFT/MSFT. The high-grade component showed loss of CD34 expression in half of the dedifferentiated SFTs and all of the dedifferentiated SFTs with divergent differentiation, whereas Ki-67 was markedly increased in all of the evaluable cases and paralleled the tumor grade. In 4 cases, the expression and phosphorylation status of key factors that control transcription and protein synthesis were also investigated. Both S6 and 4E-BP1 showed low activation in the low-grade MSFT and a high level of activation in the high-grade component. Seven of the 10 patients died of their disease during follow-up, with a median overall survival of 73 months (range, 5 to 288 mo). The median time to distant metastasis was 156 months after the initial diagnosis, and median overall survival from the first signs of metastasis was 8 months.

  18. Alisertib in Treating Patients With Advanced or Metastatic Sarcoma

    ClinicalTrials.gov

    2016-02-02

    Myxofibrosarcoma; Recurrent Adult Soft Tissue Sarcoma; Recurrent Leiomyosarcoma; Recurrent Liposarcoma; Recurrent Malignant Peripheral Nerve Sheath Tumor; Recurrent Undifferentiated Pleomorphic Sarcoma; Stage III Soft Tissue Sarcoma; Stage IV Soft Tissue Sarcoma

  19. Diagnostic and prognostic gene expression signatures in 177 soft tissue sarcomas: hypoxia-induced transcription profile signifies metastatic potential

    PubMed Central

    Francis, Princy; Namløs, Heidi Maria; Müller, Christoph; Edén, Patrik; Fernebro, Josefin; Berner, Jeanne-Marie; Bjerkehagen, Bodil; Åkerman, Måns; Bendahl, Pär-Ola; Isinger, Anna; Rydholm, Anders; Myklebost, Ola; Nilbert, Mef

    2007-01-01

    Background Soft tissue sarcoma (STS) diagnosis is challenging because of a multitude of histopathological subtypes, different genetic characteristics, and frequent intratumoral pleomorphism. One-third of STS metastasize and current risk-stratification is suboptimal, therefore, novel diagnostic and prognostic markers would be clinically valuable. We assessed the diagnostic and prognostic value of array-based gene expression profiles using 27 k cDNA microarrays in 177, mainly high-grade, STS of 13 histopathological subtypes. Results Unsupervised analysis resulted in two major clusters – one mainly containing STS characterized by type-specific genetic alterations and the other with a predominance of genetically complex and pleomorphic STS. Synovial sarcomas, myxoid/round-cell liposarcomas, and gastrointestinal stromal tumors clustered tightly within the former cluster and discriminatory signatures for these were characterized by developmental genes from the EGFR, FGFR, Wnt, Notch, Hedgehog, RAR and KIT signaling pathways. The more pleomorphic STS subtypes, e.g. leiomyosarcoma, malignant fibrous histiocytoma/undifferentiated pleomorphic sarcoma and dedifferentiated/pleomorphic liposarcoma, were part of the latter cluster and were characterized by relatively heterogeneous profiles, although subclusters herein were identified. A prognostic signature partly characterized by hypoxia-related genes was identified among 89 genetically complex pleomorphic primary STS and could, in a multivariate analysis including established prognostic markers, independently predict the risk of metastasis with a hazard ratio of 2.2 (P = 0.04). Conclusion Diagnostic gene expression profiles linking signaling pathways to the different STS subtypes were demonstrated and a hypoxia-induced metastatic profile was identified in the pleomorphic, high-grade STS. These findings verify diagnostic utility and application of expression data for improved selection of high-risk STS patients. PMID:17359542

  20. Vismodegib and Gamma-Secretase/Notch Signalling Pathway Inhibitor RO4929097 in Treating Patients With Advanced or Metastatic Sarcoma

    ClinicalTrials.gov

    2016-06-09

    Adult Alveolar Soft Part Sarcoma; Adult Angiosarcoma; Adult Desmoplastic Small Round Cell Tumor; Adult Epithelioid Hemangioendothelioma; Adult Epithelioid Sarcoma; Adult Extraskeletal Myxoid Chondrosarcoma; Adult Extraskeletal Osteosarcoma; Adult Fibrosarcoma; Adult Leiomyosarcoma; Adult Liposarcoma; Adult Malignant Mesenchymoma; Adult Malignant Peripheral Nerve Sheath Tumor; Adult Rhabdomyosarcoma; Adult Synovial Sarcoma; Adult Unclassified Pleomorphic Sarcoma; Chondrosarcoma; Clear Cell Sarcoma of the Kidney; Conjunctival Kaposi Sarcoma; Dermatofibrosarcoma Protuberans; Gastrointestinal Stromal Tumor; Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Metastatic Osteosarcoma; Ovarian Sarcoma; Recurrent Adult Soft Tissue Sarcoma; Recurrent Adult Unclassified Pleomorphic Sarcoma of Bone; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Kaposi Sarcoma; Recurrent Osteosarcoma; Recurrent Uterine Corpus Sarcoma; Small Intestine Leiomyosarcoma; Stage III Adult Soft Tissue Sarcoma; Stage III Uterine Sarcoma; Stage IV Adult Soft Tissue Sarcoma; Stage IV Uterine Sarcoma; Unclassified Pleomorphic Sarcoma of Bone

  1. A non-comparative phase II study of dose intensive chemotherapy with doxorubicin and ifosfamide followed by high dose ICE consolidation with PBSCT in non-resectable, high grade, adult type soft tissue sarcomas.

    PubMed

    Hartmann, Jörg Thomas; Horger, M; Kluba, T; Königsrainer, A; de Zwart, P; von Weyhern, C Hann; Eckert, F; Budach, W; Bokemeyer, C

    2013-12-01

    The objective was to determine the role of dose intensive induction chemotherapy in patients with soft tissue sarcomas (STS) that were considered unresectable. Treatment consisted of 2-3 cycles of doxorubicin (Dox) and ifosfamide (Ifo) followed by high dose chemotherapy with ifosfamide, carboplatin, etoposide (HD-ICE) plus peripheral blood stem cell transplantation (PBSCT). 30 out of 631 consecutive patients, median age 46 years (21-62), with high grade STS were included. 29 patients completed at least 2 cycles of Dox/Ifo. HD-ICE was withheld because of progressive disease (PD) in 5 patients, neurotoxicity in 6 cases, insufficient peripheral blood stem cell (PBSC) mobilization, complete remission (CR) and refusal in 1 patient each. HD-ICE was associated with non-haematological grade III toxicity including emesis, mucositis, fever, neurotoxicity, and transaminase level elevation. Two additional patients attained a partial response after HD-ICE. Overall, 24 of 30 (80%) patients underwent surgery, with complete tumor resections in 19 patients (63% of all patients, 79% of the operated subgroup); however, 2 of these required amputation. After a median follow up period of 50 months in surviving patients (range, 26-120), 5-year PFS and OS rates were 39% and 48%, respectively. Induction chemotherapy plus consolidation HD-ICE is generally feasible, but is associated with significant neurotoxicity. The advantage of HD-ICE over conventional dose chemotherapy plus external beam radiation therapy (EBRT) in non-resectable disease remains unproven.

  2. Variation in the Gross Tumor Volume and Clinical Target Volume for Preoperative Radiotherapy of Primary Large High-Grade Soft Tissue Sarcoma of the Extremity Among RTOG Sarcoma Radiation Oncologists

    SciTech Connect

    Wang Dian; Bosch, Walter; Kirsch, David G.; Al Lozi, Rawan; El Naqa, Issam; Roberge, David; Finkelstein, Steven E.; Petersen, Ivy; Haddock, Michael; Chen, Yen-Lin E.; Saito, Naoyuki G.; Hitchcock, Ying J.; Wolfson, Aaron H.; DeLaney, Thomas F.

    2011-12-01

    Purpose: To evaluate variability in the definition of preoperative radiotherapy gross tumor volume (GTV) and clinical target volume (CTV) delineated by sarcoma radiation oncologists. Methods and Materials: Extremity sarcoma planning CT images along with the corresponding diagnostic MRI from two patients were distributed to 10 Radiation Therapy Oncology Group sarcoma radiation oncologists with instructions to define GTV and CTV using standardized guidelines. The CT data with contours were then returned for central analysis. Contours representing statistically corrected 95% (V95) and 100% (V100) agreement were computed for each structure. Results: For the GTV, the minimum, maximum, mean (SD) volumes (mL) were 674, 798, 752 {+-} 35 for the lower extremity case and 383, 543, 447 {+-} 46 for the upper extremity case. The volume (cc) of the union, V95 and V100 were 882, 761, and 752 for the lower, and 587, 461, and 455 for the upper extremity, respectively. The overall GTV agreement was judged to be almost perfect in both lower and upper extremity cases (kappa = 0.9 [p < 0.0001] and kappa = 0.86 [p < 0.0001]). For the CTV, the minimum, maximum, mean (SD) volumes (mL) were 1145, 1911, 1605 {+-} 211 for the lower extremity case and 637, 1246, 1006 {+-} 180 for the upper extremity case. The volume (cc) of the union, V95, and V100 were 2094, 1609, and 1593 for the lower, and 1533, 1020, and 965 for the upper extremity cases, respectively. The overall CTV agreement was judged to be almost perfect in the lower extremity case (kappa = 0.85 [p < 0.0001]) but only substantial in the upper extremity case (kappa = 0.77 [p < 0.0001]). Conclusions: Almost perfect agreement existed in the GTV of these two representative cases. Tshere was no significant disagreement in the CTV of the lower extremity, but variation in the CTV of upper extremity was seen, perhaps related to the positional differences between the planning CT and the diagnostic MRI.

  3. Malignant solitary fibrous tumor with high-grade nuclear atypia: an alternate entity for the undetermined tumor group.

    PubMed

    Yamada, Yuichi; Kohashi, Kenichi; Bekki, Hirofumi; Ishii, Takeaki; Iura, Kunio; Maekawa, Akira; Yamamoto, Hidetaka; Iwamoto, Yukihide; Oda, Yoshinao

    2015-02-01

    Recently, a novel fusion transcript, NAB2-STAT6, and its variants have also been reported to be specific diagnostic markers for solitary fibrous tumors (SFTs). In this study, we validated the existence of the NAB2-STAT6 fusion gene in SFTs and examined its relation with the pathological features. Frozen samples from 9 tumors were assessed for fusion gene. The detected fusion genes exhibited large intron sequences and the insertion of unknown and previously unreported sequences. The fusion genes were not detected in the 2 malignant cases with high-grade nuclear atypia, nuclear pleomorphism and necrosis, that was confirmed by multiplex PCR method. In addition, 1 of the 2 NAB2-STAT6 fusion gene-negative tumors showed amplification of the MDM2 and CDK4 genes. It was suggested that a certain proportion of tumors previously diagnosed as malignant SFTs with high-grade nuclear atypia lacking NAB2-STAT6 should be categorized into a special subtype of SFT, which is genetically different from conventional SFTs, and which cannot be apparently distinguished from dedifferentiated liposarcoma or undifferentiated pleomorphic sarcoma.

  4. Metastatic salivary pleomorphic adenoma.

    PubMed

    Sim, D W; Maran, A G; Harris, D

    1990-01-01

    Pleomorphic adenomas of the salivary gland are usually regarded as benign tumours. We report a case in which a solitary pulmonary metastasis arose from a pleomorphic adenoma of the right parotid gland. The mechanism of metastasis is discussed.

  5. Critical reappraisal of primary osseous composite sarcoma (malignant mesenchymoma) - analysis of four cases and literature review.

    PubMed

    Chow, Louis T C

    2016-06-01

    In accordance with recent terminology, it is proposed that malignant mesenchymoma should be renamed 'composite sarcoma' and defined as 'a sarcoma composed of two or more cellular types each of which is sufficiently differentiated to permit clear recognition of its histogenetic type microscopically, immunohistochemically or ultrastructurally; excluding fibrosarcomatous and high-grade pleomorphic undifferentiated sarcomatous component, dedifferentiated sarcoma and the combination of osteosarcoma and chondrosarcoma which is regarded as a single histogenetic type'. Four cases of primary osseous composite sarcoma (POCS) were identified among 928 primary bone sarcomas. Their age ranged from 10 to 87 years, peak incidence in the second decade with equal sex distribution. Most presented with pain, commonest in the knee, affecting the metaphysis, appearing radiologically as expansile infiltrative osteolytic lesions with cortical erosion, periosteal reaction, variable extent of osteoblastic areas and soft tissue extension. All contained variable amounts of conventional high-grade osteosarcoma with or without chondrosarcoma component; the other constituents were liposarcoma, rhabdomyosarcoma and leiomyosarcoma. In all cases, Ki67 proliferative index was over 35%, there was no CDK4 and MDM2 amplification. The absence of low-grade component supported the de novo origin of POCS rather than derivation from divergent dedifferentiation. The two older patients with hitherto undescribed osteoleiomyosarcoma died 2 and 10 months after operation, whereas the two younger with osteorhabdomyosarcoma and osteoliposarcoma enjoyed disease-free survival at 16 and 6 years after chemotherapy despite the latter showing lung metastasis at presentation. Identification of the different lines of differentiation together with their approximate amounts and histological grades is therefore mandatory for POCS as multi-agent chemotherapy catered for each sarcoma component might offer hope for long

  6. Management of Breast Sarcoma.

    PubMed

    Hsu, Cary; McCloskey, Susan A; Peddi, Parvin F

    2016-10-01

    Breast sarcomas are exceptionally rare mesenchymal neoplasms composed of many histologic subtypes. Therapy is guided by principles established in the management of extremity sarcomas. The anatomic site does influence treatment decisions, particularly the surgical management. Surgery should be undertaken with the aim of achieving a widely negative margin. Selected patients can be managed with breast-conserving surgery. Breast reconstruction is increasingly being undertaken for selected patients. Radiation therapy and chemotherapy are used selectively for large, high-grade sarcomas for which there is significant concern for local and distant recurrence. PMID:27542642

  7. Epigenetic re-expression of HIF-2α suppresses soft tissue sarcoma growth

    PubMed Central

    Nakazawa, Michael S.; Eisinger-Mathason, T. S. Karin; Sadri, Navid; Ochocki, Joshua D.; Gade, Terence P. F.; Amin, Ruchi K.; Simon, M. Celeste

    2016-01-01

    In soft tissue sarcomas (STS), low intratumoural O2 (hypoxia) is a poor prognostic indicator. HIF-1α mediates key transcriptional responses to hypoxia, and promotes STS metastasis; however, the role of the related HIF-2α protein is unknown. Surprisingly, here we show that HIF-2α inhibits high-grade STS cell growth in vivo, as loss of HIF-2α promotes sarcoma proliferation and increases calcium and mTORC1 signalling in undifferentiated pleomorphic sarcoma and dedifferentiated liposarcoma. We find that most human STS have lower levels of EPAS1 (the gene encoding HIF-2α) expression relative to normal tissue. Many cancers, including STS, contain altered epigenetics, and our findings define an epigenetic mechanism whereby EPAS1 is silenced during sarcoma progression. The clinically approved HDAC inhibitor Vorinostat specifically increases HIF-2α, but not HIF-1α, accumulation in multiple STS subtypes. Vorinostat inhibits STS tumour growth, an effect ameliorated by HIF-2α deletion, implicating HIF-2α as a biomarker for Vorinostat efficacy in STS. PMID:26837714

  8. Local Mesenchymal Stem/Progenitor Cells Are a Preferential Target for Initiation of Adult Soft Tissue Sarcomas Associated with p53 and Rb Deficiency

    PubMed Central

    Choi, Jinhyang; Curtis, Stephen J.; Roy, David M.; Flesken-Nikitin, Andrea; Nikitin, Alexander Yu.

    2010-01-01

    The cell of origin and pathogenesis of the majority of adult soft tissue sarcomas (STS) remains poorly understood. Because mutations in both the P53 and RB tumor suppressor genes are frequent in STS in humans, we inactivated these genes by Cre-loxP–mediated recombination in mice with floxed p53 and Rb. Ninety-three percent of mice developed spindle cell/pleomorphic sarcomas after a single subcutaneous injection of adenovirus carrying Cre-recombinase. Similar to human STS, these sarcomas overexpress Cxcr4, which contributes to their invasive properties. Using irradiation chimeras generated by transplanting bone marrow cells from mice carrying either the Rosa26StoploxPLacZ or the Z/EG reporter, as well as the floxed p53 and Rb genes, into irradiated p53loxP/loxPRbloxP/loxP mice, it was determined that sarcomas do not originate from bone marrow–derived cells, such as macrophages, but arise from the local resident cells. At the same time, dermal mesenchymal stem cells isolated by strict plastic adherence and low levels of Sca-1 expression (Sca-1low, CD31negCD45neg) have shown enhanced potential for malignant transformation according to soft agar, invasion, and tumorigenicity assays, after the conditional inactivation of both p53 and Rb. Sarcomas formed after transplantation of these cells have features typical for undifferentiated high-grade pleomorphic sarcomas. Taken together, our studies indicate that local Sca-1low dermal mesenchymal stem/progenitor cells are preferential targets for malignant transformation associated with deficiencies in both p53 and Rb. PMID:20864684

  9. Efficacy of trabectedin in advanced soft tissue sarcoma: beyond lipo- and leiomyosarcoma

    PubMed Central

    De Sanctis, Rita; Marrari, Andrea; Marchetti, Silvia; Mussi, Chiara; Balzarini, Luca; Lutman, Fabio Romano; Daolio, Primo; Bastoni, Stefano; Bertuzzi, Alexia Francesca; Quagliuolo, Vittorio; Santoro, Armando

    2015-01-01

    Objective Trabectedin is effective in leiomyosarcoma and liposarcoma, especially the myxoid variant, related to the presence of the FUS-CHOP transcript. We evaluated the efficacy of trabectedin in specific subgroups of patients with soft tissue sarcomas (STS). Methods Seventy-two patients with advanced anthracycline-pretreated STS, who received trabectedin at a dose of 1.5 mg/m2 every 3 weeks by continuous 24-hour infusion, were retrospectively analyzed. Best response rate according to Response Evaluation Criteria In Solid Tumors (RECIST) criteria and severe adverse events (AEs) according to National Cancer Institute Common Terminology Criteria for Adverse Events (NCI-CTCAE v4.02) were evaluated. Secondary endpoints included progression-free survival and overall survival (OS). Results Median age was 48 (range, 20–75) years, with a median Eastern Cooperative Oncology Group performance status of 0. The median number of previous chemotherapy regimens was 1 (range, 0–5). Median number of trabectedin cycles was 3 (range, 1–17). About 69/72 patients (95.8%) were evaluable for response: 9 patients (13%) achieved partial response and 26 (37.7%) stable disease. According to histotype, clinical benefit (partial response + stable disease) was reported in synovial sarcoma (n=5), retroperitoneal liposarcoma (n=10), myxoid liposarcoma (n=5), leiomyosarcoma (n=8), high-grade undifferentiated pleomorphic sarcoma (n=5), Ewing/peripheral primitive neuroectodermal tumor (n=1), and malignant peripheral nerve sheath tumor (n=1). Any grade AEs were noncumulative, reversible, and manageable. G3/G4 AEs included anemia (n=1, 1.4%), neutropenia (n=7, 9.6%), liver toxicity (n=6, 8.3%), and fatigue (n=2, 2.8%). With a median follow-up time of 11 (range, 2–23) months, median progression-free survival and OS of the entire cohort were 2.97 months and 16.5 months, respectively. Conclusion Our experience confirms trabectedin as an effective therapeutic option for metastatic lipo- and

  10. CIC-rearranged Sarcomas: A Study of 20 Cases and Comparisons With Ewing Sarcomas.

    PubMed

    Yoshida, Akihiko; Goto, Keisuke; Kodaira, Makoto; Kobayashi, Eisuke; Kawamoto, Hiroshi; Mori, Taisuke; Yoshimoto, Seiichi; Endo, Otone; Kodama, Narihito; Kushima, Ryoji; Hiraoka, Nobuyoshi; Motoi, Toru; Kawai, Akira

    2016-03-01

    The CIC gene rearrangement exists in a subset of small round cell sarcomas. As the nosologic relationship of these sarcomas to Ewing sarcomas remains undetermined, we examined 20 CIC-rearranged sarcomas to compare their clinicopathologic features with those of Ewing sarcomas. The CIC-rearranged sarcomas were from a group of 14 men and 6 women with a median age of 24.5 years. The primary tumor sites included the limbs, trunk wall, internal trunk, lung, cerebrum, and pharynx. A comparison of the demographic and clinical characteristics of the 20 patients with CIC-rearranged sarcomas with those of the 53 near-consecutive patients with EWSR1-rarranged Ewing sarcomas showed that there were no differences with respect to their ages and sexes. Although none of the CIC-rearranged sarcomas arose in the bone, 40% of the Ewing sarcomas primarily affected the skeleton. The overall survival of patients with Ewing sarcomas was significantly better than that for patients with CIC-rearranged sarcomas. A histologic comparison of the CIC-rearranged sarcomas with 20 EWSR1-rearranged Ewing sarcomas showed significantly higher degrees of lobulation, nuclear pleomorphism, the prominence of the nucleoli, spindle cell elements, and myxoid changes in the CIC-rearranged sarcomas. Distinguishing immunohistochemical features included heterogenous CD99 reactivity, nuclear WT1 expression, and calretinin expression in the CIC-rearranged sarcomas and NKX2.2 expression in the Ewing sarcomas. CIC-rearranged sarcomas are distinct from Ewing sarcomas clinically, morphologically, and immunohistochemically, and they should be considered a separate entity rather than being grouped within the same family of tumors.

  11. Myoepithelial carcinoma arising in recurrent pleomorphic adenoma in maxillary sinus.

    PubMed

    Gupta, Ashumi; Manipadam, Marie Therese; Michael, Rajiv

    2013-09-01

    Myoepithelial carcinoma is characterized by nearly exclusive myoepithelial differentiation and evidence of malignancy. It may arise de novo or in preexisting benign tumors including pleomorphic adenoma and benign myoepithelioma. A 39-year-old lady presented with painless progressive swelling on the right cheek and right side of palate. On surgery, there was a mass in right maxillary sinus which was surgically excised and diagnosed on histopathology as pleomorphic adenoma. Subsequently, there were two recurrences. The first recurrence was in the right maxilla after 2 years that was removed surgically and diagnosed as pleomorphic adenoma. One year later, she came with rapidly progressive swelling in bilateral cheeks and face. Intraoperatively, there was a large tumor in both maxillary sinuses with extensive local infiltration. Histologically, it was diagnosed as myoepithelial carcinoma. Carcinoma ex pleomorphic adenoma is usually a high grade malignancy. It occurs most commonly in parotid gland followed by submandibular glands, minor salivary glands and occasionally in sublingual gland. To the best of our knowledge, this is the first case of myoepithelial carcinoma arising in a recurrent pleomorphic adenoma in the maxillary sinus.

  12. Pleomorphic adenoma of the palate.

    PubMed

    Erdem, Mehmet Ali; Cankaya, Abdulkadir Burak; Güven, Gülşah; Olgaç, Vakur; Kasapoğlu, Cetin

    2011-05-01

    Pleomorphic adenoma is the most common mixed benign tumor of major salivary glands. Approximately 80% of these tumors arise in the parotid gland, whereas 7% arise in the minor salivary glands. The most common sites for minor salivary gland where pleomorphic adenoma arises are the palates followed by lips and cheek. We report a palate mass in a 46-year-old male patient. The initial cytologic diagnosis by fine-needle aspiration biopsy was pleomorphic adenoma. This report describes a case of pleomorphic adenoma regarding all distinctive diagnoses with the review of the literature.

  13. High-Grading Lunar Samples

    NASA Technical Reports Server (NTRS)

    Allen, Carlton; Sellar, Glenn; Nunez, Jorge; Mosie, Andrea; Schwarz, Carol; Parker, Terry; Winterhalter, Daniel; Farmer, Jack

    2009-01-01

    Astronauts on long-duration lunar missions will need the capability to high-grade their samples to select the highest value samples for transport to Earth and to leave others on the Moon. We are supporting studies to define the necessary and sufficient measurements and techniques for high-grading samples at a lunar outpost. A glovebox, dedicated to testing instruments and techniques for high-grading samples, is in operation at the JSC Lunar Experiment Laboratory. A reference suite of lunar rocks and soils, spanning the full compositional range found in the Apollo collection, is available for testing in this laboratory. Thin sections of these samples are available for direct comparison. The Lunar Sample Compendium, on-line at http://www-curator.jsc.nasa.gov/lunar/compendium.cfm, summarizes previous analyses of these samples. The laboratory, sample suite, and Compendium are available to the lunar research and exploration community. In the first test of possible instruments for lunar sample high-grading, we imaged 18 lunar rocks and four soils from the reference suite using the Multispectral Microscopic Imager (MMI) developed by Arizona State University and JPL (see Farmer et. al. abstract). The MMI is a fixed-focus digital imaging system with a resolution of 62.5 microns/pixel, a field size of 40 x 32 mm, and a depth-of-field of approximately 5 mm. Samples are illuminated sequentially by 21 light emitting diodes in discrete wavelengths spanning the visible to shortwave infrared. Measurements of reflectance standards and background allow calibration to absolute reflectance. ENVI-based software is used to produce spectra for specific minerals as well as multi-spectral images of rock textures.

  14. Pleomorphic adenoma of the epiglottis.

    PubMed

    Baptista, P M; Garcia-Tapia, R; Vazquez, J J

    1992-10-01

    Pleomorphic adenoma is the most common benign tumor of the major salivary glands. A pleomorphic adenoma in the larynx constitutes a rarity. A small number of cases have been reported in the literature. We report on a case seen in our hospital, and have reviewed those cases published in the medical literature during the last 25 years.

  15. Pleomorphic adenoma of the palate.

    PubMed

    Clauser, Luigi; Mandrioli, Stefano; Dallera, Vittorio; Sarti, Elisabetta; Galiè, Manilio; Cavazzini, Luigi

    2004-11-01

    Pleomorphic adenoma, is the most common tumor (50%) of the major and minor salivary glands. Seventy percent of the tumors of the minor salivary glands are pleomorphic adenomas, and the most common intraoral site is the palate, followed by the upper lip and buccal mucosa. Pleomorphic adenoma appears as a painless firm mass and, in most cases, does not cause ulceration of the overlying mucosa. Generally it is mobile, except when it occurs in the hard palate. Intraoral mixed tumors, especially those noted within the palate, lack a well-defined capsule. Lesions of the palate frequently involve periosteum or bone. Approximately 25% of benign mixed tumors undergo malignant transformation. Treatment for the pleomorphic adenoma is radical surgery. Inadequate resection leads to local recurrence. The authors report a palate pleomorphic adenoma in a 67-year-old female patient.

  16. Pediatric Sarcomas.

    PubMed

    Williams, Regan F; Fernandez-Pineda, Israel; Gosain, Ankush

    2016-10-01

    Pediatric sarcomas are a heterogeneous group of tumors accounting for approximately 10% of childhood solid tumors. Treatment is focused on multimodality therapy, which has improved the prognosis over the past two decades. Current regimens focus on decreasing treatment for low-risk patients to decrease the long-term side effects while maximizing therapy for patients with metastatic disease to improve survival. Pediatric sarcomas can be divided into soft tissue sarcomas and osseous tumors. Soft tissue sarcomas are further delineated into rhabdomyosarcomas, which affect young children and nonrhabdomyosarcomas, which are most common in adolescents. The most common bone sarcomas are osteosarcomas and Ewing's sarcoma. PMID:27542645

  17. Ewing sarcoma

    MedlinePlus

    Bone cancer - Ewing sarcoma; Ewing family of tumors; Primitive neuroectodermal tumors (PNET); Bone neoplasm - Ewing sarcoma ... NCCN clinical practice guidelines in oncology (NCCN guidelines): bone cancer. Updated 2016. www.nccn.org/professionals/physician_gls/ ...

  18. Multiple pulmonary metastases from benign pleomorphic adenoma.

    PubMed

    Sit, Ko Yung; Chui, Wing Hung; Wang, Elaine; Chiu, Shui Wah

    2008-01-01

    Metastasizing pleomorphic adenoma is a rare condition of metastasis from a histologically benign salivary gland tumor. We report a case of metastasizing pleomorphic adenoma presenting with multiple bilateral lung metastases, and discuss the clinical aspects of this disease.

  19. A case of metastasizing pleomorphic adenoma.

    PubMed

    Goodisson, D W; Burr, R G; Creedon, A J; Stirling, R W; Morgan, P R; Odell, E W; Buff, R G

    1999-03-01

    The pleomorphic adenoma is the most common benign salivary neoplasm. A case is presented in which a palatal pleomorphic adenoma seeded a metastasis in the medullary cavity of the anterior maxilla, apparently by hematogenous spread after surgical manipulation.

  20. Retroperitoneal Sarcomas.

    PubMed

    Porpiglia, Andrea S; Reddy, Sanjay S; Farma, Jeffrey M

    2016-10-01

    Retroperitoneal sarcomas are rare tumors, representing only 15% of all sarcomas. The mainstay of therapy is surgical resection with negative margins. However, this is challenging because of the late presentation of many of these tumors and involvement with adjacent structures. Decisions on radiation therapy and chemotherapy should be made in a multidisciplinary setting at a tertiary referral center.

  1. Leiomyosarcoma Ex Pleomorphic Adenoma of the Parotid Gland: A Case Report and Literature Review

    PubMed Central

    Liu, Jingxuan; Marzouk, Mark

    2016-01-01

    There is only one previously reported incident in the English literature of sarcoma ex pleomorphic adenoma of the parotid and there are only 8 cases of primary parotid leiomyosarcoma. In our case, a 79-year-old female patient presented to our care with left preauricular pain, swelling, and facial weakness. After CT imaging, she underwent left total parotidectomy. A spindle cell lesion was identified intraoperatively and the facial nerve was sacrificed. Subsequent analysis of the lesion yielded a diagnosis of leiomyosarcoma ex pleomorphic adenoma. After 30 fractions of radiation therapy, scans were negative for tumor. However, 18 months after first experiencing symptoms, she was found to have metastases to the brainstem and lung. When diagnosing sarcoma ex pleomorphic adenoma of the parotid gland, it is important to perform thorough immunohistochemical staining and exclude a previous history of sarcoma or other sources of metastases. Complete resection is critical due to the tumor's local aggressiveness and metastatic potential. Although these tumors are not very responsive to chemotherapy or radiation, adjuvant treatment is commonly used when margins are unclear.

  2. Leiomyosarcoma Ex Pleomorphic Adenoma of the Parotid Gland: A Case Report and Literature Review

    PubMed Central

    Liu, Jingxuan; Marzouk, Mark

    2016-01-01

    There is only one previously reported incident in the English literature of sarcoma ex pleomorphic adenoma of the parotid and there are only 8 cases of primary parotid leiomyosarcoma. In our case, a 79-year-old female patient presented to our care with left preauricular pain, swelling, and facial weakness. After CT imaging, she underwent left total parotidectomy. A spindle cell lesion was identified intraoperatively and the facial nerve was sacrificed. Subsequent analysis of the lesion yielded a diagnosis of leiomyosarcoma ex pleomorphic adenoma. After 30 fractions of radiation therapy, scans were negative for tumor. However, 18 months after first experiencing symptoms, she was found to have metastases to the brainstem and lung. When diagnosing sarcoma ex pleomorphic adenoma of the parotid gland, it is important to perform thorough immunohistochemical staining and exclude a previous history of sarcoma or other sources of metastases. Complete resection is critical due to the tumor's local aggressiveness and metastatic potential. Although these tumors are not very responsive to chemotherapy or radiation, adjuvant treatment is commonly used when margins are unclear. PMID:27672466

  3. Leiomyosarcoma Ex Pleomorphic Adenoma of the Parotid Gland: A Case Report and Literature Review.

    PubMed

    Coulter, Michael; Liu, Jingxuan; Marzouk, Mark

    2016-01-01

    There is only one previously reported incident in the English literature of sarcoma ex pleomorphic adenoma of the parotid and there are only 8 cases of primary parotid leiomyosarcoma. In our case, a 79-year-old female patient presented to our care with left preauricular pain, swelling, and facial weakness. After CT imaging, she underwent left total parotidectomy. A spindle cell lesion was identified intraoperatively and the facial nerve was sacrificed. Subsequent analysis of the lesion yielded a diagnosis of leiomyosarcoma ex pleomorphic adenoma. After 30 fractions of radiation therapy, scans were negative for tumor. However, 18 months after first experiencing symptoms, she was found to have metastases to the brainstem and lung. When diagnosing sarcoma ex pleomorphic adenoma of the parotid gland, it is important to perform thorough immunohistochemical staining and exclude a previous history of sarcoma or other sources of metastases. Complete resection is critical due to the tumor's local aggressiveness and metastatic potential. Although these tumors are not very responsive to chemotherapy or radiation, adjuvant treatment is commonly used when margins are unclear. PMID:27672466

  4. Potential Therapeutic Targets in Uterine Sarcomas

    PubMed Central

    Cuppens, Tine; Tuyaerts, Sandra; Amant, Frédéric

    2015-01-01

    Uterine sarcomas are rare tumors accounting for 3,4% of all uterine cancers. Even after radical hysterectomy, most patients relapse or present with distant metastases. The very limited clinical benefit of adjuvant cytotoxic treatments is reflected by high mortality rates, emphasizing the need for new treatment strategies. This review summarizes rising potential targets in four distinct subtypes of uterine sarcomas: leiomyosarcoma, low-grade and high-grade endometrial stromal sarcoma, and undifferentiated uterine sarcoma. Based on clinical reports, promising approaches for uterine leiomyosarcoma patients include inhibition of VEGF and mTOR signaling, preferably in combination with other targeted or cytotoxic compounds. Currently, the only targeted therapy approved in leiomyosarcoma patients is pazopanib, a multitargeted inhibitor blocking VEGFR, PDGFR, FGFR, and c-KIT. Additionally, preclinical evidence suggests effect of the inhibition of histone deacetylases, tyrosine kinase receptors, and the mitotic checkpoint protein aurora kinase A. In low-grade endometrial stromal sarcomas, antihormonal therapies including aromatase inhibitors and progestins have proven activity. Other potential targets are PDGFR, VEGFR, and histone deacetylases. In high-grade ESS that carry the YWHAE/FAM22A/B fusion gene, the generated 14-3-3 oncoprotein is a putative target, next to c-KIT and the Wnt pathway. The observation of heterogeneity within uterine sarcoma subtypes warrants a personalized treatment approach. PMID:26576131

  5. Uterine sarcoma Part II-Uterine endometrial stromal sarcoma: The TAG systematic review.

    PubMed

    Horng, Huann-Cheng; Wen, Kuo-Chang; Wang, Peng-Hui; Chen, Yi-Jen; Yen, Ming-Shyen; Ng, Heung-Tat

    2016-08-01

    Endometrial stromal tumors are rare uterine tumors (<1%). Four main categories include endometrial stromal nodule, low-grade endometrial stromal sarcoma (LG-ESS), high-grade endometrial stromal sarcoma (HG-ESS), and uterine undifferentiated sarcoma (UUS). This review is a series of articles discussing the uterine sarcomas. LG-ESS, a hormone-dependent tumor harboring chromosomal rearrangement, is an indolent tumor with a favorable prognosis, but characterized by late recurrences even in patients with Stage I disease, suggesting the requirement of a long-term follow-up. Patients with HG-ESS, based on the identification of YWHAE-NUTM2A/B (YWHAE-FAM22A/B) gene fusion, typically present with advanced stage diseases and frequently have recurrences, usually within a few years after initial surgery. UUS is, a high-grade sarcoma, extremely rare, lacking a specific line of differentiation, which is a diagnosis of exclusion (the wastebasket category, which fails to fulfill the morphological and immunohistochemical criteria of translocation-positive ESS). Surgery is the main strategy in the management of uterine sarcoma. Due to rarity, complex biological characteristics, and unknown etiology and risk factors of uterine sarcomas, the role of adjuvant therapy is not clear. Only LG-ESS might respond to progestins or aromatase inhibitors. PMID:27590366

  6. [Image diagnosis and pleomorphic adenoma].

    PubMed

    Ruiz Jaureguizuría, J C; Crovetto de la Torre, M A; Bárcena Robredo, M V; Grande Icarán, D

    1989-01-01

    We examined the imaging technical findings of 11 benign pleomorphic adenomas of major salivary glands. The imaging technical included sialography, echography, computed tomography and gammagraphy. We compared the diagnostic usefulness of each of these imaging technical. The purpose of this paper is to identify the actual advantages, disadvantages and uses of these diagnostic methods.

  7. Kaposi's Sarcoma

    MedlinePlus

    Kaposi's sarcoma (KS) is a cancer that causes patches of abnormal tissue to grow under the skin, in the lining of ... of cancer cells, blood vessels, and blood cells. KS is caused by infection with human herpesvirus-8 ( ...

  8. Tumor-targeting Salmonella typhimurium A1-R in combination with doxorubicin eradicate soft tissue sarcoma in a patient-derived orthotopic xenograft (PDOX) model

    PubMed Central

    Murakami, Takashi; DeLong, Jonathan; Eilber, Fritz C.; Zhao, Ming; Zhang, Yong; Zhang, Nan; Singh, Arun; Russell, Tara; Deng, Samantha; Reynoso, Jose; Quan, Cuong; Hiroshima, Yukihiko; Matsuyama, Ryusei; Chishima, Takashi; Tanaka, Kuniya; Bouvet, Michael; Chawla, Sant; Endo, Itaru; Hoffman, Robert M.

    2016-01-01

    A patient with high grade undifferentiated pleomorphic soft-tissue sarcoma from a striated muscle was grown orthotopically in the right biceps femoris muscle of mice to establish a patient-derived orthotopic xenograft (PDOX) model. Twenty PDOX mice were divided into 4 groups: G1, control without treatment; G2, Salmonella typhimurium (S. typhimurium)A1-R administered by intratumoral (i.t.) injection once a week for 4 weeks; G3, doxorubicin (DOX) administered by intraperitoneal (i.p.) injection once a week for 4 weeks; G4, S. typhimurium A1-R (i.t.) administered once a week for 2 weeks followed by i.p. doxorubicin once a week for 2 weeks. On day 25 from the initiation of treatment, tumor volume in G2, G3, and G4 was significantly lower than G1. Mice found without gross tumor included one mouse (20%) in G2; one mouse (20%) in G3; and 3 mice (60%) in G4. Body weight loss did not significantly differ between the 3 treated groups or from the untreated control. Histological examination revealed eradication of tumor only in G4 where mice were treated with S. typhimurium A1-R followed by DOX. Our present study indicates future clinical potential of combining S. typhimurium A1-R with chemotherapy such as DOX for soft tissue sarcoma patients. PMID:26859573

  9. Tumor-targeting Salmonella typhimurium A1-R in combination with doxorubicin eradicate soft tissue sarcoma in a patient-derived orthotopic xenograft (PDOX) model.

    PubMed

    Murakami, Takashi; DeLong, Jonathan; Eilber, Fritz C; Zhao, Ming; Zhang, Yong; Zhang, Nan; Singh, Arun; Russell, Tara; Deng, Samantha; Reynoso, Jose; Quan, Cuong; Hiroshima, Yukihiko; Matsuyama, Ryusei; Chishima, Takashi; Tanaka, Kuniya; Bouvet, Michael; Chawla, Sant; Endo, Itaru; Hoffman, Robert M

    2016-03-15

    A patient with high grade undifferentiated pleomorphic soft-tissue sarcoma from a striated muscle was grown orthotopically in the right biceps femoris muscle of mice to establish a patient-derived orthotopic xenograft (PDOX) model. Twenty PDOX mice were divided into 4 groups: G1, control without treatment; G2, Salmonella typhimurium (S. typhimurium)A1-R administered by intratumoral (i.t.) injection once a week for 4 weeks; G3, doxorubicin (DOX) administered by intraperitoneal (i.p.) injection once a week for 4 weeks; G4, S. typhimurium A1-R (i.t.) administered once a week for 2 weeks followed by i.p. doxorubicin once a week for 2 weeks. On day 25 from the initiation of treatment, tumor volume in G2, G3, and G4 was significantly lower than G1. Mice found without gross tumor included one mouse (20%) in G2; one mouse (20%) in G3; and 3 mice (60%) in G4. Body weight loss did not significantly differ between the 3 treated groups or from the untreated control. Histological examination revealed eradication of tumor only in G4 where mice were treated with S. typhimurium A1-R followed by DOX. Our present study indicates future clinical potential of combining S. typhimurium A1-R with chemotherapy such as DOX for soft tissue sarcoma patients.

  10. Sarcomas of Soft Tissue and Bone.

    PubMed

    Ferrari, Andrea; Dirksen, Uta; Bielack, Stefan

    2016-01-01

    The definition of soft tissue and bone sarcomas include a large group of several heterogeneous subtypes of mesenchymal origin that may occur at any age. Among the different sarcomas, rhabdomyosarcoma, synovial sarcoma, Ewing sarcoma and osteosarcoma are aggressive high-grade malignancies that often arise in adolescents and young adults. Managing these malignancies in patients in this age bracket poses various clinical problems, also because different therapeutic approaches are sometimes adopted by pediatric and adult oncologists, even though they are dealing with the same condition. Cooperation between pediatric oncologists and adult medical oncologists is a key step in order to assure the best treatment to these patients, preferably through their inclusion into international clinical trials. PMID:27595362

  11. Endosialin expression in soft tissue sarcoma as a potential marker of undifferentiated mesenchymal cells

    PubMed Central

    Thway, Khin; Robertson, David; Jones, Robin L; Selfe, Joanna; Shipley, Janet; Fisher, Cyril; Isacke, Clare M

    2016-01-01

    Background: Soft tissue sarcomas are a group of neoplasms with differentiation towards mesenchymal tissue, many of which are aggressive and chemotherapy resistant. Histology and immunoprofiles often overlap with neoplasms of other lineages, and establishing an accurate histopathological diagnosis is crucial for correct management, and therapeutic stratification. The endosialin cell surface glycoprotein is predominantly expressed by stromal fibroblasts and pericytes in epithelial neoplasms; however, tumour cell expression has been reported in small series of sarcomas. Methods: We assessed endosialin expression by immunohistochemistry in a large set of 514 human soft tissue sarcomas. Results: Tumour cell endosialin expression was seen in 89% of undifferentiated pleomorphic sarcomas (104/117), 77% adult fibrosarcomas/spindle cell sarcomas (20/26), 62% synovial sarcomas (37/60), 51% leiomyosarcomas (94/185) and 31% rhabdomyosarcomas (39/126). Conclusions: Endosialin immunohistochemistry has potential to distinguish undifferentiated and poorly differentiated sarcomas from other poorly differentiated, non-mesenchymal neoplasms. A Phase II trial randomising patients with advanced sarcomas to receive chemotherapy with/without an endosialin therapeutic antibody has recently completed enrolment. Endosialin expression could be used to select patients for such clinical trials. Based on our results, patients with undifferentiated pleomorphic sarcoma may be particularly suitable for such a therapeutic approach. PMID:27434038

  12. Benign pleomorphic adenomas in children.

    PubMed

    Malone, B; Baker, S R

    1984-01-01

    Benign pleomorphic adenomas of the salivary glands in children are rare. Reported are 30 patients under the age of 21 years presenting with this neoplasm. Twelve patients were first seen with recurrent or persistent tumor following previous attempts at removal. Retreatment resulted in control of the neoplasm in eight patients with follow-up from 5 to 24 years. Two additional patients have developed malignant degeneration of their neoplasms. All 18 previously untreated patients have remained free of recurrence. As with adults, the treatment of choice for benign pleomorphic adenomas of the parotid gland developing in children is parotidectomy with preservation of the facial nerve. Tumors arising in the submandibular gland are best treated by complete excision of the gland as well as the tumor.

  13. Pleomorphic adenoma of the larynx.

    PubMed

    Argat, M; Born, I A; Maier, H; Mohadjer, C

    1994-01-01

    Tumors arising from minor salivary glands are extremely rare neoplasms in the larynx. Of the few reports of pleomorphic adenomas in this site, most have subglottic locations while only one case has involved the true vocal cord and seven cases have had supraglottic locations. We present a case of benign mixed tumor located in the posterior commissure which, to our knowledge, is the first reported in the world literature.

  14. TAZ and YAP are frequently activated oncoproteins in sarcomas

    PubMed Central

    Fullenkamp, Colleen A.; Hall, Sarah L.; Jaber, Omar I.; Pakalniskis, Brittany L.; Savage, Erica C.; Savage, Johanna M.; Ofori-Amanfo, Georgina K.; Lambertz, Allyn M.; Ivins, Stephanie D.; Stipp, Christopher S.; Miller, Benjamin J.; Milhem, Mohammed M.; Tanas, Munir R.

    2016-01-01

    TAZ (WWTR1) and YAP are transcriptional coactivators and oncoproteins inhibited by the Hippo pathway. Herein we evaluate 159 sarcomas representing the most prevalent sarcoma types by immunohistochemistry for expression and activation (nuclear localization) of TAZ and YAP. We show that 50% of sarcomas demonstrate activation of YAP while 66% of sarcomas demonstrate activated TAZ. Differential activation of TAZ and YAP are identified in various sarcoma types. At an RNA level, expression of WWTR1 or YAP1 predicts overall survival in undifferentiated pleomorphic sarcoma and dedifferentiated liposarcoma. Immunohistochemistry demonstrates that TAZ and YAP expression and activation are positively correlated with grade in the well-differentiated liposarcoma to dedifferentiated liposarcoma tumor progression sequence as well as conventional chondrosarcomas. TAZ and YAP are constitutively activated oncoproteins in sarcoma cell lines. Knock-down of TAZ and YAP demonstrate differential activity for the two proteins. Verteporfin decreases colony formation in soft agar as well as CTGF expression in sarcoma cell lines harboring activated TAZ and YAP. PMID:27129148

  15. Hepatocyte Growth Factor-mediated satellite cells niche perturbation promotes development of distinct sarcoma subtypes.

    PubMed

    Morena, Deborah; Maestro, Nicola; Bersani, Francesca; Forni, Paolo Emanuele; Lingua, Marcello Francesco; Foglizzo, Valentina; Šćepanović, Petar; Miretti, Silvia; Morotti, Alessandro; Shern, Jack F; Khan, Javed; Ala, Ugo; Provero, Paolo; Sala, Valentina; Crepaldi, Tiziana; Gasparini, Patrizia; Casanova, Michela; Ferrari, Andrea; Sozzi, Gabriella; Chiarle, Roberto; Ponzetto, Carola; Taulli, Riccardo

    2016-03-17

    Embryonal Rhabdomyosarcoma (ERMS) and Undifferentiated Pleomorphic Sarcoma (UPS) are distinct sarcoma subtypes. Here we investigate the relevance of the satellite cell (SC) niche in sarcoma development by using Hepatocyte Growth Factor (HGF) to perturb the niche microenvironment. In a Pax7 wild type background, HGF stimulation mainly causes ERMS that originate from satellite cells following a process of multistep progression. Conversely, in a Pax7 null genotype ERMS incidence drops, while UPS becomes the most frequent subtype. Murine EfRMS display genetic heterogeneity similar to their human counterpart. Altogether, our data demonstrate that selective perturbation of the SC niche results in distinct sarcoma subtypes in a Pax7 lineage-dependent manner, and define a critical role for the Met axis in sarcoma initiation. Finally, our results provide a rationale for the use of combination therapy, tailored on specific amplifications and activated signaling pathways, to minimize resistance emerging from sarcomas heterogeneity.

  16. Histiocytic sarcoma

    PubMed Central

    Machado, Eduardo Silva; de Miranda, Ana Carolina; Escopelli, Ticiane; Caron, Ruggero; Escopelli, Alessandra Cristhina

    2011-01-01

    A 59-year-old white woman, SC, after being treated for pneumonia, presented with an increase in the size of lymph nodes. The immunohistochemical examination diagnosed histiocytic sarcoma. Relapse occurred 12 months after starting chemotherapy. The patient evolved with febrile neutropenia, septic shock and death. PMID:23284265

  17. Pleomorphic adenoma of the breast.

    PubMed

    Chen, K T

    1990-06-01

    The clinicopathologic features of 2 new and 24 previously reported cases of pleomorphic adenoma of the breast are reviewed. This benign breast tumor resembles its salivary gland counterpart histologically. The circumscription and preferential juxta-areolar location suggest large breast duct origin of the tumors. Inappropriate surgery, i.e., mastectomy, was performed in 42% of the cases. Misdiagnosis and the resulting inappropriate treatment can easily be avoided if the pathologist includes this entity in the differential diagnosis when confronted with unusual differentiated epithelial breast lesions.

  18. Metastasizing pleomorphic adenoma with myoepithelial cell predominance.

    PubMed

    Cresson, D H; Goldsmith, M; Askin, F B; Reddick, R L; Postma, D S; Siegal, G P

    1990-12-01

    The biological behavior of pleomorphic adenomas (mixed tumors) of salivary gland origin is complex. Tumors with benign histologic features may exhibit recurrence and locally aggressive behavior especially after incomplete excision. A small percentage of pleomorphic adenomas have obvious malignant components in epithelial or in both epithelial and mesenchymal components and can metastasize. There are also rare case reports which appear to document typical pleomorphic adenomas of salivary gland with histologically identical visceral and lymph node metastases. Recently myoepithelial cell proliferation has been identified as a possible predictor of aggressive clinical behavior in otherwise histologically benign pleomorphic adenomas. We report such a parotid gland lesion with local recurrence and retroperitoneal spread. DNA-flow cytometry of cells from the paraffin-embedded primary and metastasis showed similar aneuploid populations. Aneuploidy appeared to reflect the malignant potential of this particular pleomorphic adenoma and suggests that DNA-flow cytometry of salivary gland tumors may yield important prognostic information.

  19. Aortic intimal sarcoma with embolic metastases.

    PubMed

    Wright, E P; Glick, A D; Virmani, R; Page, D L

    1985-12-01

    A 46-year-old woman died from massive bowel infarction. At autopsy, a primary sarcoma was found growing along the intimal surface of the aorta at the level of the celiac axis. Tumor emboli were found in distal aortic branches and most abdominal organs. Immunoperoxidase for Factor VIII and electron microscopy (EM) did not support an endothelial origin. EM showed myofibroblastic differentiation. Review of the literature yields an array of diagnostic histologic terms for these tumors, hampering case comparison. The literature does suggest, however, that the clinical presentation of these rare neoplasms correlates nicely with the location and gross morphology of the lesion. We therefore propose a clinicopathologic classification, categorizing the lesions as intimal (obstructive and nonobstructive) and mural. The former are typically pleomorphic sarcomas and are probably of myofibroblastic origin, whereas the latter are usually leiomyosarcomas or fibrosarcomas that probably originate in the media or adventitia. PMID:3000205

  20. BCOR-CCNB3 (Ewing-like) sarcoma: a clinicopathologic analysis of 10 cases, in comparison with conventional Ewing sarcoma.

    PubMed

    Puls, Florian; Niblett, Angela; Marland, Gillian; Gaston, Czar Louie L; Douis, Hassan; Mangham, D Chas; Sumathi, Vaiyapuri P; Kindblom, Lars-Gunnar

    2014-10-01

    BCOR-CCNB3 fusion transcripts resulting from an X-chromosomal paracentric inversion were recently identified in a series of unclassifiable soft tissue and bone sarcomas with Ewing sarcoma-like morphology. The morphologic and clinical features of these sarcomas are, as yet, not well characterized. Here we describe the clinicopathologic features of 10 cases of BCOR-CCNB3 sarcoma and compare their clinical course with typical Ewing sarcoma. Nine of 10 patients were male, and all were 11 to 18 years of age. Seven tumors were located in the bone and 3 in the deep soft tissues. The histomorphologic spectrum was quite wide, with 7 tumors predominately showing small primitive cell morphology with angulated nuclei simulating so-called atypical Ewing sarcoma and 3 predominately showing spindle cell morphology. Recurrent and metastatic lesions showed increased cellularity and marked pleomorphism. Immunohistochemistry showed expression of CCNB3 (100%), bcl2 (90%), CD99 (60%), and CD117 (60%). Reverse transcription polymerase chain reaction for BCOR-CCNB3 fusion transcripts was positive in all 9 cases, which yielded sufficient extracted RNA. Five- and 10-year survival rates were 75% and 56%, respectively. BCOR-CCNB3 sarcomas located in axial skeleton and soft tissues showed a significantly shorter survival. The Ewing sarcoma overall survival was not statistically different, although there was a trend for longer survival of patients with BCOR-CCNB3 sarcomas in the extremities. In conclusion, this study provides a detailed description of the histologic spectrum, immunohistochemical features, and clinical characteristic of BCOR-CCNB3 sarcoma justifying distinction from Ewing sarcoma with its typical EWS/FUS-ETS translocations. Ideally immunohistochemistry is used in combination with reverse transcription polymerase chain reaction for definitive diagnosis. PMID:24805859

  1. Systemic therapy for endometrial stromal sarcomas: current treatment options.

    PubMed

    Serkies, Krystyna; Pawłowska, Ewa; Jassem, Jacek

    2016-01-01

    Uterine endometrial stromal sarcomas including true low-grade endometrial stromal sarcoma (LG-ESS) and high-grade (HG-ESS) or undifferentiated endometrial sarcoma (UES) constitute a group of rare, aggressive malignancies. Most LG-ESSs express steroid receptors. Surgery is the principal primary therapy for endometrial stromal sarcomas and should be considered in all cases. These malignancies are relatively radio- and chemoresistant. Chemotherapy is used in recurrent and advanced HG-ESS and UES. Currently, the combination of gemcitabine and docetaxel is considered the most effective regimen, but at the expense of substantial toxicity. In steroid receptor positive advanced LG-ESS hormonal therapy, mainly with progestins, allows in some patients for a long-term survival. Aromatase inhibitors seem to be equally effective as first- and subsequent-line of treatment, and are well tolerated. The role of molecular-targeted therapies in endometrial stromal sarcomas remains to be established. PMID:27629136

  2. Giant Pleomorphic Adenoma of the Parotid Gland.

    PubMed

    Sajid, Muhammad; Rehman, Sajid; Misbah, Junaid

    2015-10-01

    Salivary gland tumours are a relatively rare entity. Pleomorphic adenoma is the most common amongst these, comprising 60 - 70% of all parotid tumours. Pleomorphic adenomas are benign and tend to increase in size slowly. Here we are presenting a case of giant pleomorphic adenoma of the parotid, being the largest in size to be excised in Pakistan in recorded literature measuring 24 x 22 x 12 cm and weighing 1.8 kgs. Superficial parotidectomy was done with an excellent cosmetic outcome. PMID:26522191

  3. [Pleomorphic adenoma of the maxillary sinus].

    PubMed

    Leunig, A; Grevers, G

    1994-11-01

    Pleomorphic adenoma is the most common benign tumour of the salivary glands, especially the parotid gland. In the present paper we introduce the rare case of a pleomorphic adenoma of the maxillary sinus in a 82-year-old man who was referred to our outpatient clinic with nasal obstruction and occasional events of nose bleeding. Thorough investigation, using endoscopy and computed tomography, revealed a mass extending from the left maxillary sinus to the nasal cavity; the tumour was removed surgically; pathological examination showed a pleomorphic adenoma with no signs of malignancy.

  4. Metastasizing pleomorphic adenoma of the nasal septum.

    PubMed

    Freeman, S B; Kennedy, K S; Parker, G S; Tatum, S A

    1990-11-01

    Pleomorphic adenoma is the most common benign tumor of glandular tissue occurring in the head and neck region. There have been several reports of metastasis of this benign-appearing tumor from the salivary glands to distant sites, suggesting hematogenous spread and implantation. Although occurrence of pleomorphic adenoma on the nasal septum has been described, to our knowledge this is the first reported case of recurrent septal pleomorphic adenoma with histologically benign tissue in an enlarged metastatic ipsilateral submandibular lymph node, suggesting lymphatic spread. The literature concerning the subject is reviewed. Wide septal excision and modified neck dissection is the recommended treatment.

  5. [The recurrent multifocal pleomorphic adenoma].

    PubMed

    Vigili, M G; Sciarretta, F; Marzetti, A; Marzetti, F

    1993-01-01

    Pleomorphic adenoma (P.A.), the most common tumor of the salivary gland, demonstrates a peculiar clinicopathological behaviour for numerous reasons: the high recurrence rate following primary surgery (up to 50%), the appearance of malignancy (2-9%), the reported number of distant metastases histologically identical to the primary P.A. From among 71 cases of benign parotid tumors treated from Nov. 89 to Nov. 92 in the ENT Department of "Regina Elena", the National Cancer Institute in Rome, six particular cases showed multiple force of P.A. recurring after primary surgery performed from 3 to 32 years previously and are object of discussion in this study. All of these six cases had multiple recurrences, usually manifest as nodular clusters in the parotid area, while in three cases appeared as well a recurrence in the soft tissue of the neck, far removed from the parotid space, with no involvement of neck nodes as was revealed through histological examination following neck dissection. A hypothetical mechanism of diffusion is discussed. The Authors agree with the opinion which holds the surgeon's inability to successfully eradicate primary tumors responsible for the high frequency of recurrences. The surgical technique of "enucleation" is, in fact, inadequate in P.A. excision owing the high risk of mishandling or rupturing the tumor capsule with a consequent seeding of the tumor onto the surgical bed. Lateral lobectomy, with identification of the facial nerve, or total conservative parotidectomy (for deep lobe adenoma) are correct techniques in treating primary P.A.. The Authors also discuss management of recurrent P.A. in relation to facial nerve involvement. Preservation of the seventh nerve with eventual post-operative radiation should be considered an alternative to nerve sacrifice in selected cases of recurrent pleomorphic adenoma.

  6. Palatal pleomorphic adenoma in a child.

    PubMed

    Noghreyan, A; Gatot, A; Maor, E; Fliss, D M

    1995-04-01

    Benign salivary gland tumours in childhood are rare. We present a case of a pleomorphic adenoma arising in a minor salivary gland within the hard palate, review the literature and discuss the diagnostic and therapeutic features of the condition.

  7. Pleomorphic adenoma of the nasal septum.

    PubMed

    Jassar, P; Stafford, N D; MacDonald, A W

    1999-05-01

    Pleomorphic adenoma is the commonest benign tumour of the major salivary glands. It can also occur in minor salivary glands, mainly in the oral cavity, but also in other sites in the head and neck both within and outwith the upper aerodigestive tract. We present a rare case of pleomorphic adenoma of the nasal septum with consideration of the clinical management and a review of the literature.

  8. [Pleomorphic adenoma with bilateral pulmonary metastasis].

    PubMed

    De Kerangal, X; Poirrier, P; Soulard, R; Dot, J M; Ségneuric, J B; L'Her, P; Jancovici, R; Saint-Blancart, P

    2001-11-01

    We report a new case of pleomorphous adenoma of the submaxillary glands with multiple lung metastases. Histological proof was obtained on the thoracoscopic surgical specimen. Clinically, this benign tumor presents as a malignant tumor. Diagnosis has been a subject of debate; surgical resection is indicated. Diagnosis is achieved by elimination in a patient with one or several nodules occurring in a context of recurrent pleomorphous adenoma.

  9. Studies on high grade cerebral gliomas

    SciTech Connect

    Bleehen, N.M. )

    1990-04-01

    A brief review of attempts in the United Kingdom to improve the results of treatment of high grade (grade 3, 4) supra-tentorial astrocytomas is presented. The radiosensitizer misonidazole failed to improve the results of post-surgical radiotherapy, however, multivariate analysis of data from these patients has provided a prognostic index of use in defining good and poor prognosis patients. An overview study of adjuvant nitrosourea therapy trials has shown a small significant advantage for the chemotherapy. A study of chemosensitization by benznidazole of CCNU treatment of patients in relapse failed to demonstrate any effect. 13 references.

  10. Gene therapy for high-grade glioma

    PubMed Central

    Natsume, Atsushi

    2008-01-01

    The treatment of high-grade gliomas remains difficult despite recent advances in surgery, radiotherapy and chemotherapy. True advances may emerge from the increasing understanding in molecular biology and discovery of novel mechanisms for the delivery of tumoricidal agents. In an attempt to overcome this formidable neoplasm, molecular approaches using gene therapy have been investigated clinically since 1992. The clinical trials have mainly been classified into three approaches: suicide gene therapy, immune gene therapy and oncolytic viral therapy. In this article, we review these approaches, which have been studied in previous and ongoing clinical trials. PMID:19262115

  11. Polyploidy in pleomorphic adenomas with cytological atypia.

    PubMed

    Thunnissen, F B; Peterse, J L; Buchholtz, R; Van der Beek, J M; Bosman, F T

    1992-01-01

    Occasionally, in fine-needle aspirates of pleomorphic salivary gland adenomas, considerable cytonuclear atypia is present, which may give rise to a false-positive diagnosis. In this study DNA cytophotometry was performed on Feulgen restained smears prepared from material obtained by needle aspirates of normal salivary glands (n = 4), pleomorphic adenomas with (n = 5) and without (n = 4) atypia and a carcinoma in a pleomorphic adenoma. The results showed a clear diploid DNA histogram in the specimens of normal salivary gland and pleomorphic adenomas without atypia. In contrast, in the pleomorphic adenomas with atypia a distinct polyploid pattern was present in three out of the five DNA histograms with DNA values in 2c, 4c and 8c ranges. In two of these cases a 16c peak was also present and in the two remaining cases tetraploidy was demonstrated. In the carcinoma a main stemline at 4c was found. This report once more emphasizes the possible atypia which may be present in FNA of pleomorphic adenomas of the salivary gland. The atypia is due to polyploidy in a histologically benign tumour.

  12. Etiology and management of recurrent parotid pleomorphic adenoma.

    PubMed

    Witt, Robert L; Eisele, David W; Morton, Randall P; Nicolai, Piero; Poorten, Vincent Vander; Zbären, Peter

    2015-04-01

    The objective of this review study was to encompass the relevant literature and current best practice options for this challenging, sometimes incurable problem. The source of the data was Ovid MEDLINE from 1946 to 2014. Review methods consisted of articles with clinical correlates. The most important cause of recurrence is enucleation with rupture and incomplete tumor excision at operation. Incomplete pseudocapsule, extracapsular extension, pseudopods of pleomorphic adenoma tissue, and satellite pleomorphic beyond the pseudocapsule are also likely linked to recurrent pleomorphic adenoma. Most recurrent pleomorphic adenoma are multinodular. Magnetic resonance imaging is the imaging study of choice for recurrent pleomorphic adenoma. Nerve integrity monitoring may reduce morbidity for recurrent pleomorphic adenoma. Treatment of recurrent pleomorphic adenoma must be individualized. Total parotidectomy, given the multicentricity of recurrent pleomorphic adenoma, is appropriate in many patients, but may be inadequate to control recurrent pleomorphic. There is accumulating evidence from retrospective series that postoperative radiation therapy results in significantly better local control.

  13. Clinical characteristics of high grade foveal hypoplasia.

    PubMed

    Park, Kyung-Ah; Oh, Sei Yeul

    2013-02-01

    To report clinical characteristics of high grade foveal hypoplasia. Patients with foveal hypoplasia of grade 3 or 4 on spectral domain optical coherence tomography according to a previously published scheme were enrolled. All patients underwent a full ophthalmologic assessment including visual acuity testing, slit lamp biomicroscopy, fundus examination, and evaluation of ocular alignment. The underlying causes of foveal hypoplasia were identified as albinism in five patients and aniridia in six patients. The mean logMAR visual acuity was 0.57 ± 0.24 (range 0.22-1.00) in the right eyes and 0.58 ± 0.21 (range 0.30-1.00) in the left eyes. On fundus examination in patients with albinism, two patients showed marked transparency, one patient showed moderate transparency, and two patients showed minimal transparency. Among six patients with aniridia, five patients showed normal macular pigmentation without macular reflex and one patient showed decreased macular pigmentation with no macular reflex. Patients with high grade macular hypoplasia tended to have poor visual acuities; however, the range of visual acuity was quite variable. Other factors associated with underlying disease could be the reason of this variability. Therefore, careful consideration should be given when assessing visual prognosis in foveal hypoplasia using optical coherence tomography. PMID:23124196

  14. Pleomorphic rhabdomyosarcoma showing smooth-muscle and fibrohistiocytic differentiation: a single case report.

    PubMed

    Eyden, Brian

    2010-02-01

    Rhabdomyosarcoma has traditionally been subclassified into alveolar, embryonal, and pleomorphic variants. Less commonly, spindle-cell, neuroendocrine, sclerosing, and lipid-rich or clear-cell subtypes are seen. The author recently encountered a myogenic sarcoma, with all the common markers of rhabdomyosarcoma, but expressing the unusual features of alpha-smooth-muscle actin and abundant rough endoplasmic reticulum (rER). This myogenic sarcoma, therefore, exhibited four lines of differentiation, and is documented here. The patient was a 65-year-old man with an inguinal soft tissue mass. Following surgical excision, the patient was given radiotherapy and was well without disease after 6 years. The tumor was positive for vimentin, desmin, alpha-smooth-muscle actin, alpha-sarcomeric actin, myogenin, MyoD1, and CD68. Cytoplasm was dominated by abundant rER intermingled with lipid droplets and lysosomes. Cell surfaces exhibited microvillous processes and focal adhesions, but no lamina. Subplasmalemmal smooth-muscle-type myofilaments with focal densities and rare sarcomeric filaments were seen. The low level of expression of some markers was interpreted as consistent with a poorly differentiated tumor. Given the four lines of differentiation--striated muscle, smooth muscle, fibroblastic, and histiocytic--a name reflecting its phenotype would be pleomorphic rhabdomyosarcoma showing smooth-muscle and fibrohistiocytic differentiation. PMID:20070153

  15. High-Grade Prostatic Intraepithelial Neoplasia

    PubMed Central

    Bostwick, David G; Liu, Lina; Brawer, Michael K; Qian, Junqi

    2004-01-01

    High-grade prostatic intraepithelial neoplasia is considered the most likely precursor of prostatic carcinoma. The only method of detection is biopsy; prostatic intraepithelial neoplasia (PIN) does not significantly elevate serum prostate-specific antigen concentration and cannot be detected by ultra-sonography. The incidence of PIN in prostate biopsies averages 9% (range, 4%–16%), representing 115,000 new cases of PIN diagnosed each year in United States. PIN has a high predictive value as a marker for adenocarcinoma, and its identification warrants repeated biopsy for concurrent or subsequent invasive carcinoma. Carcinoma will develop in most patients with PIN within 10 years. PIN is associated with progressive abnormalities of phenotype and genotype that are intermediate between normal prostatic epithelium and cancer, indicating impairment of cell differentiation and regulatory control with advancing stages of prostatic carcinogenesis. Androgen deprivation therapy decreases the prevalence and extent of PIN, suggesting that this form of treatment may play a role in chemoprevention. PMID:16985598

  16. Pleomorphic multicentric adenoma in the submandibular gland.

    PubMed

    Alves, Carlos Augusto Ferreira; Ribeiro Júnior, Ophir; Borba, Alexandre Meireles; Gouveia, Marcia Maria; Guimarães Júnior, Jayro; Aburad, Arlindo; de Souza, Suzana Cantanhede Orsini Machado

    2007-12-01

    Neoplasms of salivary glands represent a small group among the diseases involving the head and neck complex. In this group, the pleomorphic adenoma is the most frequent neoplasm, yet involves the submandibular gland in only 12.3% of cases. A patient presenting a swelling in the region of the submandibular gland was submitted to an incisional biopsy, where a fragment of the gland and one juxtaposed node were removed. Histologically they were defined as pleomorphic adenoma. Later, the patient was submitted to submandibulectomy and two other nodes were found close to the gland and removed. All specimens were histologically defined as pleomorphic adenoma. This multicentric finding is of great interest, perhaps explaining the recurrence rate of this neoplasm. The patient is in continuous follow-up and has not presented signs of recurrence.

  17. Sarcoma Foundation of America

    MedlinePlus

    ... Make a Donation Matching Gifts Sarcoma Dedication Page Stocks and Securities Workplace Giving Year-End Giving FAQ's ... Make a Donation Matching Gifts Sarcoma Dedication Page Stocks and Securities Workplace Giving Year-End Giving

  18. Dysplastic pleomorphic adenoma of the sublingual salivary gland.

    PubMed

    Clark, J; Bailey, B M; Eveson, J W

    1993-12-01

    All tumours of the sublingual gland are rare and paradoxically the large majority are malignant. A case of pleomorphic adenoma in the sublingual gland is described. The tumour showed areas of dysplasia and the difficulties in distinguishing this from benign pleomorphic adenoma or carcinoma in pleomorphic adenoma are considered, and the literature is reviewed.

  19. [A case of very late malignant degeneration of pleomorphic adenoma].

    PubMed

    Darche, V; Hustin, J; Lejuste, P; Robillard, T; Piette, E

    1998-12-01

    Incomplete excision of a pleomorphic adenoma exposes to a high risk of recurrence and tumor spread, making secondary surgery more difficult or a malignant transformation with a poor vital prognosis likely. Three histological types of pleomorphic adenomas can be observed when the tumor undergoes a transformation, namely the carcinoma ex-pleomorphic adenoma, the true malignant mixed tumor and the benign metastasizing mixed tumour.

  20. [Pleomorphic adenoma of minor salivary glands].

    PubMed

    Cwalina, Piotr; Skorek, Andrzej; Narozny, Waldemar; Stankiewicz, Czesław

    2002-01-01

    Pleomorphic adenoma, a benign tumor often seen in ENT practice, arises either from minor as well from major salivary glands. 5-14% of tumors occur in minor glands. Sixteen cases of minor salivary gland pleomorphic adenomas are studied. Eight of them originate from the oral cavity: 6 from the palate, one from the lower lip and one from the cheek. Two oral adenomas were malignant. In 4 patients tumors occur in the nasal cavity and in two other patients--in the neck. The clinical and pathological features of these patients are presented. Special attention is given to malignant transformation and the rate of recurrence of the tumors.

  1. Metastasizing pleomorphic adenoma of the salivary gland.

    PubMed

    Chen, K T

    1978-11-01

    A case of metastasizing pleomorphic adenoma in which both the primary tumor and metastasis were composed of benign pleomorphic structures is reported and previously reported cases reviewed. The metastasis commonly developed many years after the excision of the primary tumor and was usually preceded by local recurrences. The most common sites of metastasis were the bone and lung. Mitotic activity and infiltrative growth pattern are the histologic features in the primary tumor important in predicting the metastasizing potential. The treatment of choice for the metastatic tumor appears to be surgical excision. Radiotherapy seems to have a limited role other than providing temporary palliation.

  2. Giant pleomorphic adenoma of the parotid gland.

    PubMed

    Çetin, Mehmet Ali; Ikincioğulları, Aykut; Saygı, Gökçe; Hatipoğlu, Hatice Gül; Köseoğlu, Sabri; Dere, Hüseyin

    2012-01-01

    Pleomorphic adenomas are the most common benign tumors of the salivary glands. These adenomas generally present without pain and are slowly enlarged. However, they can reach enormous sizes, because they are often neglected by the patient and due to late diagnosis and intervention because of fear of surgery or sociocultural factors. This may lead to functional, aesthetic and social problems. In this article, we present a 55-year-old female patient with a giant pleomorphic adenoma in size of 15x15x20 cm, who presented with the complaint of a mass enlarged and swollen for 20 years in her left neck and face and underwent a successful surgery.

  3. Pleomorphic adenoma of the nasal columella.

    PubMed

    Ceylan, Alper; Celenk, Fatih; Poyraz, Aylar; Uslu, Sabri

    2008-01-01

    Pleomorphic adenoma (mixed tumor) is the most common benign neoplasm of the salivary glands. It is considered to occur primarily in the major salivary glands, such as the parotid and submandibular glands. It is much less common in the minor salivary glands, and rarely occurs at other sites, such as larynx, pharynx, trachea, lacrimal gland, and sinonasal tract. An external nose localization of this lesion is very rare. Irrespective of the site where the lesion originates, its preferred treatment should be surgery. We describe a very rare case of recurrent pleomorphic adenoma originating from the columella.

  4. Suprasellar salivary gland-like pleomorphic adenoma.

    PubMed

    Yao, Kun; Duan, Zejun; Bian, Yu; Wang, Mengyang; Qi, Xueling

    2014-01-01

    Suprasellar salivary gland-Like pleomorphic adenoma is not a common disease and seldom reported so far. We are reporting a case of a 23-year-old man with recurrent suprasellar salivary gland-like pleomorphic adenoma, who underwent an operation of subtotal, subfrontal resection under the wrong pathology diagnosis of benign teratoma in another hospital 4-year-ago. Four years later, he was admitted to our hospital for additional visual loss of the right eye (left, 1.0; right, 0.4) resulting from tumor regrowth. Magnetic resonance imaging revealed that suprasellar extension and compressed optic chiasm resulted in visual disturbance of the patient. The tumor was totally excised and histological examination evidenced the pathological features of intrasellar salivary gland-like pleomorphic adenoma. The patient did not receive any further treatment and he is free from tumor recurrence for 30 months after the operation. From this point of view, clinical prognosis of intrasellar salivary gland-like pleomorphic adenoma was good after total surgical resection.

  5. Targeted therapy for sarcomas

    PubMed Central

    Forscher, Charles; Mita, Monica; Figlin, Robert

    2014-01-01

    Sarcomas are tumors of mesenchymal origin that make up approximately 1% of human cancers. They may arise as primary tumors in either bone or soft tissue, with approximately 11,280 soft tissue tumors and 2,650 bone tumors diagnosed each year in the United States. There are at least 50 different subtypes of soft tissue sarcoma, with new ones described with ever-increasing frequency. One way to look at sarcomas is to divide them into categories on the basis of their genetic make-up. One group of sarcomas has an identifiable, relatively simple genetic signature, such as the X:18 translocation seen in synovial sarcoma or the 11:22 translocation seen in Ewing’s sarcoma. These specific abnormalities often lead to the presence of fusion proteins, such as EWS-FLI1 in Ewing’s sarcoma, which are helpful as diagnostic tools and may become therapeutic targets in the future. Another group of sarcomas is characterized by complex genetic abnormalities as seen in leiomyosarcoma, osteosarcoma, and undifferentiated sarcoma. It is important to keep these distinctions in mind when contemplating the development of targeted agents for sarcomas. Different abnormalities in sarcoma could be divided by tumor subtype or by the molecular or pathway abnormality. However, some existing drugs or drugs in development may interfere with or alter more than one of the presented pathways. PMID:24669185

  6. Comprehensive screening of alternative lengthening of telomeres phenotype and loss of ATRX expression in sarcomas.

    PubMed

    Liau, Jau-Yu; Lee, Jen-Chieh; Tsai, Jia-Huei; Yang, Ching-Yao; Liu, Tsung-Lin; Ke, Zhi-Long; Hsu, Hung-Han; Jeng, Yung-Ming

    2015-12-01

    According to cytogenetic aberrations, sarcomas can be categorized as complex or simple karyotype tumors. Alternative lengthening of telomeres is a telomere-maintenance mechanism common in sarcomas. Recently, this mechanism was found to be associated with loss of either α-thalassemia/mental retardation syndrome X-linked (ATRX) or death domain-associated (DAXX) protein. We previously reported that alternative lengthening of telomeres and loss of ATRX expression were common in leiomyosarcoma, angiosarcoma, pleomorphic liposarcoma, and dedifferentiated liposarcoma. In the present study, we screened an additional 245 sarcomas of other types to determine the prevalence of alternative lengthening of telomeres, loss of ATRX/DAXX expression, and their relationship. Undifferentiated pleomorphic sarcomas were frequently alternative lengthening of telomeres positive (65%) and loss of ATRX was seen in approximately half of the alternative lengthening of telomeres-positive tumors. Nineteen of 25 myxofibrosarcomas were alternative lengthening of telomeres-positive, but only one was ATRX deficient. Three of 15 radiation-associated sarcomas were alternative lengthening of telomeres positive, but none of them was ATRX deficient. Alternative lengthening of telomeres and/or loss of ATRX were uncommon in malignant peripheral nerve sheath tumors, gastrointestinal stromal tumors, and embryonal rhabdomyosarcomas. By contrast, none of the 71 gene fusion-associated sarcomas was ATRX deficient or alternative lengthening of telomeres positive. All tumors exhibited preserved DAXX expression. Combining our previous studies and this study, a total of 384 sarcomas with complex karyotypes were examined, 83 of which were ATRX deficient (22%). By telomere-specific fluorescence in situ hybridization, 45% (138/308) were alternative lengthening of telomeres positive, 55% (76/138) of which were ATRX deficient. Loss of ATRX was highly associated with alternative lengthening of telomeres (P<0.001). We

  7. Histologic and Genetic Advances in Refining the Diagnosis of “Undifferentiated Pleomorphic Sarcoma”

    PubMed Central

    Kelleher, Fergal C.; Viterbo, Antonella

    2013-01-01

    Undifferentiated pleomorphic sarcoma (UPS) is an inclusive term used for sarcomas that defy formal sub-classification. The frequency with which this diagnosis is assigned has decreased in the last twenty years. This is because when implemented, careful histologic assessment, immunohistochemistry, and ultra-structural evaluation can often determine lineage of differentiation. Further attrition in the diagnostic frequency of UPS may arise by using array-comparative genomic hybridization. Gene expression arrays are also of potential use as they permit hierarchical gene clustering. Appraisal of the literature is difficult due to a historical perspective in which specific molecular diagnostic methods were previously unavailable. The American Joint Committee on Cancer (AJCC) classification has changed with different inclusion criteria. Taxonomy challenges also exist with the older term “malignant fibrous histiocytoma” being replaced by “UPS”. In 2010 an analysis of multiple sarcoma expression databases using a 170-gene predictor, re-classified most MFH and “not-otherwise-specified” (NOS) tumors as liposarcomas, leiomyosarcomas or fibrosarcomas. Interestingly, some of the classifier genes are potential molecular therapeutic targets including Insulin-like growth factor 1 (IGF-1), Peroxisome proliferator-activated receptor γ (PPARγ), Nerve growth factor β (NGF β) and Fibroblast growth factor receptor (FGFR). PMID:24216705

  8. SYSTEMIC HISTIOCYTIC SARCOMA IN A COMMON HIPPOPOTAMUS (HIPPOPOTAMUS AMPHIBIUS).

    PubMed

    da Costa, Luciana Fachini; Malta, Marcelo Campos Cordeiro; Tinoco, Herlandes Penha; de Medeiros, Lilian Botelho; Bittar, Ana Luiza Sarkis Vieira; Hoffmann, Aline Rodrigues; Pierezan, Felipe; da Paixão, Tatiane Alves; Santos, Renato Lima

    2016-06-01

    A 37-yr-old captive common hippopotamus (Hippopotamus amphibius) developed lethargy and decline in mobility that progressed to death, despite supportive therapy. Histopathologic examination revealed severe, diffuse, intravascular and interstitial infiltration of neoplastic histiocytes in the spleen, liver, lymph nodes, lungs, large intestine, kidneys, and thyroid gland. Neoplastic cells were pleomorphic with marked anisocytosis and anisokaryosis, scattered multinucleated giant cells, numerous bizarre mitotic figures, and marked erythrophagocytosis. Immunohistochemistry demonstrated that neoplastic cells were positive for ionized calcium-binding adapter molecule 1 (a histiocytic marker) and negative for CD3 (a T-cell marker) and myeloperoxidase, confirming the diagnosis of systemic histiocytic sarcoma.

  9. SYSTEMIC HISTIOCYTIC SARCOMA IN A COMMON HIPPOPOTAMUS (HIPPOPOTAMUS AMPHIBIUS).

    PubMed

    da Costa, Luciana Fachini; Malta, Marcelo Campos Cordeiro; Tinoco, Herlandes Penha; de Medeiros, Lilian Botelho; Bittar, Ana Luiza Sarkis Vieira; Hoffmann, Aline Rodrigues; Pierezan, Felipe; da Paixão, Tatiane Alves; Santos, Renato Lima

    2016-06-01

    A 37-yr-old captive common hippopotamus (Hippopotamus amphibius) developed lethargy and decline in mobility that progressed to death, despite supportive therapy. Histopathologic examination revealed severe, diffuse, intravascular and interstitial infiltration of neoplastic histiocytes in the spleen, liver, lymph nodes, lungs, large intestine, kidneys, and thyroid gland. Neoplastic cells were pleomorphic with marked anisocytosis and anisokaryosis, scattered multinucleated giant cells, numerous bizarre mitotic figures, and marked erythrophagocytosis. Immunohistochemistry demonstrated that neoplastic cells were positive for ionized calcium-binding adapter molecule 1 (a histiocytic marker) and negative for CD3 (a T-cell marker) and myeloperoxidase, confirming the diagnosis of systemic histiocytic sarcoma. PMID:27468044

  10. TERT promoter hotspot mutations are recurrent in myxoid liposarcomas but rare in other soft tissue sarcoma entities

    PubMed Central

    2014-01-01

    Background Recently, recurrent point mutations in the telomerase reverse transcriptase (TERT) promoter region have been found in many human cancers, leading to a new transcription factor binding site, increased induction of TERT and subsequently to telomere maintenance. We determined the prevalence of TERT promoter mutations in soft tissue sarcomas of 341 patients comprising 16 entities and in 16 sarcoma cell lines covering 7 different soft tissue sarcoma types. Methods The sarcoma tissue samples were collected from the archives of the Institute of Pathology, University of Heidelberg and were composed of 39 myxoid liposarcomas (MLS), 61 dedifferentiated liposarcomas, 15 pleomorphic liposarcomas, 27 leiomyosarcomas, 25 synovial sarcomas (SS), 35 malignant peripheral nerve sheath tumors (MPNST), 40 undifferentiated pleomorphic sarcomas, 17 myxofibrosarcomas, 9 low grade fibromyxoid sarcomas, 10 cases of dermatofibrosarcoma protuberans, 31 solitary fibrous tumors (SFT), 8 extraskeletal myxoid chondrosarcomas, 9 angiosarcomas, 6 alveolar soft part sarcomas, 5 clear cell sarcomas and 4 epithelioid sarcomas. Sarcoma cell lines were obtained from the raising laboratories. A 193 bp fragment of the TERT promoter region covering the hot-spot mutations C228T and C250T was amplified, and direct sequencing of the PCR products was performed. Results TERT promoter mutations were detected in 36/341 sarcomas. They were highly recurrent in MLS (29/39; 74%) and were in the present MLS series not associated with the phenotype (myxoid vs. round cell variant), tumor grade, tumor site and patients’ median age or gender. In the remaining cases, TERT promoter mutations were found only in 7/302 sarcoma samples and confined to SFTs (4/31; 13%), MPNSTs (2/35; 6%), and SSs (1/25; 4%). Within the collection of sarcoma cell lines examined, TERT promoter mutations were detected in two MLS and in one of three MPNST cell lines. Conclusions TERT promoter mutations are frequent in MLSs including

  11. Primary prostate sarcoma: how to manage following diagnosis at transurethral resection

    PubMed Central

    Hicks, Natalie; Gurung, Pratik M.S.; Deshmukh, Nayneeta; Apakama, Ikechukwu; Patel, Prashant

    2016-01-01

    Primary prostate sarcomas are rare, reportedly comprising just 0.7% of all prostate malignancies. Here, we present the case of a 66-year-old man who was diagnosed with prostate stromal sarcoma after undergoing a routine transurethral resection of prostate for bladder outflow obstruction. Primary prostate sarcoma can be aggressive even when low-grade, with a high risk of local recurrence and, high malignant potential when high-grade. They require aggressive multimodality treatment with surgery, chemotherapy and radiotherapy for durable survival outcomes. They also require close surveillance with long-term follow-up. PMID:27147716

  12. Primary prostate sarcoma: how to manage following diagnosis at transurethral resection.

    PubMed

    Hicks, Natalie; Gurung, Pratik M S; Deshmukh, Nayneeta; Apakama, Ikechukwu; Patel, Prashant

    2016-05-03

    Primary prostate sarcomas are rare, reportedly comprising just 0.7% of all prostate malignancies. Here, we present the case of a 66-year-old man who was diagnosed with prostate stromal sarcoma after undergoing a routine transurethral resection of prostate for bladder outflow obstruction. Primary prostate sarcoma can be aggressive even when low-grade, with a high risk of local recurrence and, high malignant potential when high-grade. They require aggressive multimodality treatment with surgery, chemotherapy and radiotherapy for durable survival outcomes. They also require close surveillance with long-term follow-up.

  13. Can Kaposi Sarcoma Be Prevented?

    MedlinePlus

    ... early? Can Kaposi sarcoma be prevented? Kaposi sarcoma (KS) is caused by the Kaposi sarcoma associated herpesvirus ( ... to protect people against KSHV. For now, preventing KS depends on reducing the chance of becoming infected ...

  14. Stewart-Treves syndrome following idiopathic leg lymphoedema: remember sarcoma.

    PubMed

    Harrison, W D; Chandrasekar, C R

    2015-06-01

    Patients with skin complaints secondary to oedema are commonly encountered and can be a diagnostic challenge. Here, we present the case of a 78-year-old lady with Stewart-Treves syndrome, a rare cutaneous angiosarcoma. The histology showed angiocutaneous sarcoma with poorly defined margins. The prognosis remains extremely poor. A successful transfemoral amputation reduced the massive tumour burden and cleared a source of sepsis from this fungating tumour. Our aim is to highlight this rare but high-grade sarcoma which results from chronic lymphoedema. The red flag signs of sarcoma are masses greater than 5 cm, tumours which are rapidly growing, deep to fascia or painful, and tumours that recur after previous surgery. Non-healing or progressive ulceration in the background of chronic lymphoedema should raise the possibility of underlying malignancy. We need a low threshold for biopsy and early referral to an appropriate multidisciplinary team for the optimum management of the patient. PMID:26075513

  15. Spinal pleomorphic xanthoastrocytoma companied with periventricular tumor

    PubMed Central

    Zhao, Xintong; Jiang, Xiaochun; Wang, Xiangming

    2015-01-01

    Pleomorphic xanthoastrocytoma (PXA) is a low grade tumor that occurs in supratentorial area of children and young adult. In the previous reports, PXA of spinal cord or multicentre was extremely rare. A 60-year-old patient of spinal PXA and periventricular tumor presented with waist pain and weakness of double legs for one month. Neuroimaging showed that a lesion at the level of L2-L3 and periventricular tumor. Postoperative microscopy indicated that WHO grade II PXA. Photomicrograph of the lesion showed spindle cells, marked nuclear and cytoplasmic pleomorphism, with foamy cytoplasm. Immunohistochemical staining showed that GFAP and S-100 were positive. This is a rare case of synchronous multicentric PXA. Physicians should be realized multicentric dissemination by meninges or cerebrospinal fluid in PXA patients. It is important to describe the particular case in order to better understanding of clinical features. PMID:25755815

  16. [One case of laryngeal pleomorphic adenoma].

    PubMed

    An, Huiqin; Bu, Guiqing; Guo, Mingli

    2013-05-01

    A male patient, 55 years old, suffered from intermittent sound,voice depression and shortness of breath for one year,and from dysphagia for 3-4 months. Through fiber laryngoscopy,we could see tumor in the left posterior aryepiglottic fold. The tumor's surface was smooth. A portion of the tumor protruded to the laryngeal cavity and the aryepiglottic fold external,it also covered most of the glottis. Bilateral vocal cord were smooth and had good mobility. Throat CT demonstrated an irregular soft tissue mass on the left side of the aryepiglottic fold in supraglottic area with obscure normal boundary from adjacent structure. The left side of pyriform sinus became shallow without obvious bone destruction. The pathological report showed pleomorphic adenoma. The diagnosis was laryngeal pleomorphic adenoma. PMID:23898619

  17. Metastasizing pleomorphic adenoma of the salivary gland.

    PubMed

    Manucha, Varsha; Ioffe, Olga B

    2008-09-01

    Metastasizing pleomorphic adenoma of salivary glands is a group of rare tumors that are histologically identical to benign mixed tumors and that inexplicably metastasize. A review of the literature revealed that it usually occurs after multiple local recurrences, and the interval between diagnosis of primary pleomorphic adenoma and metastases ranges between 3 and 52 years. The most common site for metastasis is bone, followed by the head and neck and lung. No histologic or molecular parameters exist at the present time that could predict the development of metastasis in these neoplasms. Metastasectomy confers significant survival advantage over nonoperative treatment for localized and accessible metastases, but there is no definite treatment protocol available in cases of widespread metastases.

  18. Giant pleomorphic adenoma of the parotid gland.

    PubMed

    Takahama, Ademar; da Cruz Perez, Danyel Elias; Magrin, José; de Almeida, Oslei Paes; Kowalski, Luiz Paulo

    2008-01-01

    Pleomorphic adenoma is the most common type of all benign and malignant salivary gland tumors, involving more frequently the parotid gland. It is a benign tumor with a slow and continuous growth that without treatment can reach an enormous size. We present a case of a giant pleomorphic adenoma in a 78-year-old man with a history of more than 30 years of a growing lesion in the parotid gland. Clinical examination revealed a giant mass on the right side of the face, however without any sign of facial nerve damage. The tumor was completely resected by total parotidectomy and preservation of the facial nerve. Macroscopically, the tumor measured 28 cm and weighed 4.0 Kg. On the histological examination there was a predominance of epithelial and myoepithelial cells in a hyaline and myxoid stroma. It was not found any area of malignant transformation. In the post-operatory the aesthetic and functional results were excellent.

  19. Histiocytic sarcoma that mimics benign histiocytosis.

    PubMed

    Boisseau-Garsaud, A M; Vergier, B; Beylot-Barry, M; Nastasel-Menini, F; Dubus, P; de Mascarel, A; Eghbali, H; Beylot, C

    1996-06-01

    A 28-year-old man presented with a histiocytic sarcoma of a very uncommon origin, as it had developed for several years like a benign cutaneous histiocytosis resembling generalized eruptive histiocytoma before becoming acute, with nodal and massive pulmonary involvement. Despite various chemotherapies, the patient died within 8 months. Skin biopsies showed histiocytic proliferation in the dermis and node biopsies showed histiocytic proliferation with a sinusoidal pattern. Immunohistochemical analysis, performed on paraffin-embedded sections, demonstrated strong labeling of tumoral cells for CD68 and moderate labeling for CD3 and CD4. CD30 labeling was negative. S-100 protein was positive on a Langerhans' cell reactive subpopulation. Electron microscopy confirmed the histiocytic nature of malignant cells and showed cytoplasmic inclusions such as regularly laminated bodies, dense bodies and pleomorphic inclusions. No Birbeck granules were seen. A gene rearrangement study of T-cell receptor gamma and immunoglobulin heavy chain genes showed a germline configuration. Histiocytic sarcoma is an extremely rare true histiocytic malignancy, the existence of which has been recently debated since it has often been mistaken in the past for large cell lymphomas. Such a deceptive onset as benign cutaneous histiocytosis has not been described in the literature to our knowledge.

  20. [Malignant pleomorphic adenoma of the palate].

    PubMed

    Martín Vázquez, C; Muñoz Colado, M; Lorente Tortosa, J M; Abad Róyo, J M; Alvarez Montero, O L

    1998-03-01

    Pleomorphic adenoma or mixed tumor is the most common benign neoplasm of the minor salivary glands. These tumors rarely have malignant features. Three varieties are differentiated by histological features and tumor behavior. Whether malignant tumors develop on benign adenomas or are malignant from onset is still not known. A malignant mixed tumor of the minor salivary glands of the palate is reported. The difficulty of histological diagnosis, in spite of immunohistochemical techniques, is emphasized and the surgical treatment is described.

  1. Cutaneous leiomyomatosis and parotid pleomorphic adenoma.

    PubMed

    Ocampo-Candiani, Jorge; Vázquez-Martínez, Osvaldo; Regalado-Briz, Arturo; Barboza-Quintana, Oralia; Méndez-Olvera, Nora

    2005-01-01

    We present a case of cutaneous leiomyomas (CL) arising in a pleomorphic adenoma (PA) of the parotid gland. PA and CL are benign tumors arising from the parotid gland and the erector pilli muscle, respectively. They both have a benign clinical course and in most cases leiomyomas are multiple in nature. PAs of the parotid are the most frequent benign tumors of the major salivary glands. To our knowledge this is the first case of PA with CL.

  2. The soft tissue sarcomas

    SciTech Connect

    Eilber, F.R.; Morton, D.L.; Sondak, V.K.; Economou, J.S.

    1987-01-01

    New advances in multimodality therapy of sarcomas in all anatomic sites are thoroughly described. Multimodality therapy with limb-salvage surgery for extremity tumors, sarcomas of the head and neck, trunk, intraabdominal, visceral, and genitourinary tract and cardiopulmonary system are presented. Separate sections are devoted to the management of pediatric sarcomas, pulmonary metastasis and to the pathology and radiobiology, chemotherapy, and immunotherapy of sarcomas. The text also stresses the philosophy of achieving adequate local control without radical amputation by combined surgery and chemo/radiotherapy.

  3. Recurrent pleomorphic adenoma of the palate in a child.

    PubMed

    Shaaban, H; Bruce, J; Davenport, P J

    2001-04-01

    A rare case of recurrent pleomorphic adenoma of the palate in a 9-year-old boy is presented. Pleomorphic adenoma is relatively rare in children compared with its incidence in adults. However, it is the most common benign epithelial tumour of the salivary glands. The majority of pleomorphic adenomata in children occur in the major salivary glands, mainly the parotid gland. Pleomorphic adenomata of the minor salivary glands are rare in children and mainly occur in the palatal glands. Of the few cases of pleomorphic adenoma of the palate reported in children, only one case showed recurrence of the tumour after primary excision. We present the second case of recurrent pleomorphic adenoma of the palate in a child.

  4. Myxoid malignant fibrous histiocytoma and pleomorphic liposarcoma share very similar genomic imbalances.

    PubMed

    Idbaih, Ahmed; Coindre, Jean-Michel; Derré, Josette; Mariani, Odette; Terrier, Philippe; Ranchère, Dominique; Mairal, Aline; Aurias, Alain

    2005-02-01

    Malignant fibrous histiocytoma (MFH) is the most common soft tissue sarcoma. Nevertheless, the validity of this heterogeneous pathological entity has been recurrently questioned by pathologists. Recently, analyses by comparative genomic hybridization (CGH) of a large series of MFHs suggested that MFHs consist of morphologic modulation of other poorly differentiated sarcomas like leiomyosarcomas (LMS) or dedifferentiated liposarcomas (DLPS). We report here an analysis by CGH of 22 myxoid MFHs (mMFH), one of the five histological subtypes of MFH, and of nine pleomorphic liposarcomas (pLPS), a rare poorly differentiated LPS. The chromosome imbalances encountered in the series of mMFH were very similar to those observed in the series of pLPS studied in the laboratory and in the series of 14 pLPS published in the literature. The most frequent gains involved chromosome subregions: pericentromeric regions of 1, 5p, 19p, 19q and 20q. Losses found in the chromosomal arms 1q, 2q, 3p, 4q, 10q, 11q and 13q were also recurrent. The use of a clustering software did not separate the two pathological groups (mMFH and pLPS) on the basis of genomic data. Moreover, pLPS-mMFH represented, according to the clustering software results, an entity clearly distinguished from other soft tissue sarcomas, LMS in particular, with which they share common genetic aberrations. Additional studies are needed to identify genes targeted by these genomic aberrations, and implicated in the oncogenesis of these tumor subtypes. The characterization of common gene alterations in both tumor groups would suggest a closer relationship between these two types of soft tissue sarcomas. PMID:15702084

  5. Carcinoma ex pleomorphic adenoma: pathologic analysis of 73 cases.

    PubMed

    Lewis, J E; Olsen, K D; Sebo, T J

    2001-06-01

    Pathologic factors of predictive value for carcinoma ex pleomorphic adenoma (CXPA), an aggressive salivary gland malignancy, are poorly defined. Because residual mixed tumor may be relatively inconspicuous and various carcinoma subtypes are encountered, misdiagnosis is common. To describe the pathologic features and identify potential prognostic factors, we retrospectively examined 73 cases of CXPA of the major salivary glands treated at Mayo Clinic. Paraffin section immunostaining for keratins (AE1/AE3, CK7, CK20), epithelial membrane antigen, carcinoembryonic antigen, vimentin, actin, S-100 protein, glial fibrillary acidic protein, and p53 and c-erbB-2 oncoproteins was performed in 69 cases. DNA content and proliferation indices were determined by digital image analysis of Feulgen- and MIB-I-stained sections, retrospectively. Survival was calculated by the Kaplan-Meier method, and prognostic variables were analyzed with the log-rank test. The carcinoma component was predominant in 82% of tumors. Adenocarcinoma not otherwise specified (31 cases) and salivary duct carcinoma (24 cases) were the most frequent histologic subtypes. Sixty-two tumors were high grade (Broders 3 or 4). Residual mixed tumor was extensively hyalinized in 54 cases. Pathologic features significantly associated with overall survival included pathologic stage (P =.009), tumor size (P =.012), grade (P =.005), proportion of carcinoma (P =.004), extent of invasion (P =.002), and proliferation index of carcinoma (P =.03). Of 4 patients with intracapsular (noninvasive) carcinoma, none had an adverse outcome. The immunohistochemical profile of CXPA included positive staining reactions in the malignant component for AE1/AE3 in 97% of cases, CK7 in 94%, epithelial membrane antigen in 86%, carcinoembryonic antigen in 75%, vimentin in 52%, and S-100 protein in 29%. Expression of p53 and c-erbB-2 oncoproteins was detected in 41% and 30% of the carcinomas, respectively, but neither was associated with

  6. Detection of high-grade atypia nuclei in breast cancer imaging

    NASA Astrophysics Data System (ADS)

    Noël, Henri; Roux, Ludovic; Lu, Shijian; Boudier, Thomas

    2015-03-01

    Along with mitotic count, nuclear pleomorphism or nuclear atypia is an important criterion for the grading of breast cancer in histopathology. Though some works have been done in mitosis detection (ICPR 2012,1 MICCAI 2013,2 and ICPR 2014), not much work has been dedicated to automated nuclear atypia grading, especially the most difficult task of detection of grade 3 nuclei. We propose the use of Convolutional Neural Networks for the automated detection of cell nuclei, using images from the three grades of breast cancer for training. The images were obtained from ICPR contests. Additional manual annotation was performed to classify pixels into five classes: stroma, nuclei, lymphocytes, mitosis and fat. At total of 3,000 thumbnail images of 101 × 101 pixels were used for training. By dividing this training set in an 80/20 ratio we could obtain good training results (around 90%). We tested our CNN on images of the three grades which were not in the training set. High grades nuclei were correctly classified. We then thresholded the classification map and performed basic analysis to keep only rounded objects. Our results show that mostly all atypical nuclei were correctly detected.

  7. Subcutaneous pleomorphic adenomas in two different areas of the face.

    PubMed

    Tsukuno, Mari; Nakamura, Akiko; Takai, Shigeharu; Kurihara, Kunihiro

    2002-01-01

    A 41-year-old woman developed two subcutaneous pleomorphic adenomas on her face. Pleomorphic adenoma usually arises as a benign tumour of a major salivary gland and often develops multifocally within the gland. These two pleomorphic adenomas originated in the subcutaneous layer of the face, also multifocally. They were excised, she made a good recovery, and she had no signs of recurrence a year later.

  8. Pleomorphic adenoma of the minor salivary gland of the cheek.

    PubMed

    Sharma, Arpit; Deshmukh, Shraddha; Shaikh, Ahmed; Dabholkar, Jyoti

    2013-09-01

    Pleomorphic adenoma is the most common tumour of the salivary gland. While the majority arises from the parotid gland, only a small percentage arises from the minor salivary glands. The cheek, however, is a rarely affected site with respect to pleomorphic adenomas of the minor salivary glands. Herein, we report a case of pleomorphic adenoma of the cheek, which presented with intraoral swelling, and conclude that complete surgical excision can be a curative treatment for this benign tumour.

  9. Schwannoma-like pleomorphic adenoma of the parotid.

    PubMed

    Tille, Jean-Christophe; Reychler, Hervé; Hamoir, Marc; Schmitz, Sandra; Weynand, Birgit

    2011-10-21

    Pleomorphic adenoma is the most common benign salivary gland tumour. It can occur in any salivary gland, but is most frequently found in the parotid gland. Chondroid metaplasia is a frequent finding in pleomorphic adenoma. Other forms of metaplasia have been described, but are encountered less frequently. We report a rare case of unusual pleomorphic adenoma of the parotid gland with schwannoma-like feature.

  10. Oncocytic changes in pleomorphic adenoma: Report of a rare case

    PubMed Central

    Kaur, Milanjeet; Bhogal, Jasmine

    2015-01-01

    Pleomorphic adenoma is the most common benign salivary gland tumor, accounting for almost three-fourths of all such tumors. Cells with oncocytic change are a common finding in salivary glands and in salivary gland tumors. When found within pleomorphic adenomas, cells with oncocytic changes may be perceived as evidence of malignancy, and lead to a misdiagnosis of carcinoma ex-pleomorphic adenoma. A case of pleomorphic adenoma arising de novo in the minor salivary glands with oncocytic changes is discussed here. PMID:26392734

  11. [Metastasizing pleomorphic adenoma of the submandibular gland: a case report].

    PubMed

    Zheng, Ya-Ge; Zhao, Ye; Wu, Lan-Yan

    2007-06-01

    Metastasizing pleomorphic adenomas without histological evidence of malignancy have rarely been reported. A case of 30-year-old woman with a mass which showed a benign pleomorphic adenoma appearanced histologically in the left submandibular gland and right supercollarbone respectively was described. Eight years ago, the patient suffered from pleomorphic adenoma of the left submandibular gland. It revealed histopathologic features consistent with the recurrent and metastasizing tumor. The clinic pathological features, possible mechanism and prevention approach of metastasizing pleomorphic adenoma were discussed based on previously reports in the literature.

  12. 1. Mill exterior, high grade chute partially restored on the ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    1. Mill exterior, high grade chute partially restored on the outside of mill building center of picture. Looking northeast from below bridge. - Kennecott Copper Corporation, Concentration Mill, On Copper River & Northwestern Railroad, Kennicott, Valdez-Cordova Census Area, AK

  13. 40 CFR 246.200 - High-grade paper recovery.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... 40 Protection of Environment 25 2011-07-01 2011-07-01 false High-grade paper recovery. 246.200 Section 246.200 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY (CONTINUED) SOLID WASTES SOURCE... paper recovery....

  14. 40 CFR 246.200 - High-grade paper recovery.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... 40 Protection of Environment 24 2010-07-01 2010-07-01 false High-grade paper recovery. 246.200 Section 246.200 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY (CONTINUED) SOLID WASTES SOURCE... paper recovery....

  15. 40 CFR 246.200 - High-grade paper recovery.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ... 40 Protection of Environment 25 2014-07-01 2014-07-01 false High-grade paper recovery. 246.200 Section 246.200 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY (CONTINUED) SOLID WASTES SOURCE... paper recovery....

  16. 40 CFR 246.200 - High-grade paper recovery.

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ... 40 Protection of Environment 26 2013-07-01 2013-07-01 false High-grade paper recovery. 246.200 Section 246.200 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY (CONTINUED) SOLID WASTES SOURCE... paper recovery....

  17. 40 CFR 246.200 - High-grade paper recovery.

    Code of Federal Regulations, 2012 CFR

    2012-07-01

    ... 40 Protection of Environment 26 2012-07-01 2011-07-01 true High-grade paper recovery. 246.200 Section 246.200 Protection of Environment ENVIRONMENTAL PROTECTION AGENCY (CONTINUED) SOLID WASTES SOURCE... paper recovery....

  18. Small cell carcinoma ex-pleomorphic adenoma of the parotid gland.

    PubMed

    Cimino-Mathews, Ashley; Lin, Brian M; Chang, Steven S; Boahene, Kofi D; Bishop, Justin A

    2012-12-01

    Small cell carcinoma (SCC) is a high-grade malignancy usually encountered in the lungs but also seen in almost any site including the salivary glands. SCC is important to recognize because it often metastasizes widely and is treated with systemic chemotherapy. Carcinoma ex pleomorphic adenoma is a malignant epithelial neoplasm arising in a pre-existing benign mixed tumor (i.e., pleomorphic adenoma, PA). While virtually any salivary carcinoma can arise from a PA, to our knowledge SCC ex-PA has not been described. We report a case of a woman presenting with fullness of the right neck and a parotid gland mass. The tumor was resected and evaluated grossly, by light microscopy, and by immunohistochemistry. Grossly, a 1.6 cm well-circumscribed nodule was identified within the parotid. Microscopic examination revealed foci of SCC associated with high-grade adenocarcinoma, in the background of a PA. The SCC was immunoreactive for cytokeratin in a dot-like pattern and neuroendocrine markers synaptophysin and CD56. Despite the focal nature of the SCC in the parotid, a pure SCC metastasis was present in one neck level II lymph node. The patient was free of disease with 8 months of follow-up. Our case illustrates that: (1) although rare, in the salivary glands SCC may arise from lower grade neoplasms including PAs; (2) SCC ex PA may metastasize as pure SCC even if the primary SCC component was focal; (3) adequate sampling of PAs is crucial to prevent missing a rare SCC that must be treated with chemotherapy.

  19. Trial of Dasatinib in Advanced Sarcomas

    ClinicalTrials.gov

    2016-10-12

    Rhabdomyosarcoma; Malignant Peripheral Nerve Sheath Tumors; Chondrosarcoma; Sarcoma, Ewing's; Sarcoma, Alveolar Soft Part; Chordoma; Epithelioid Sarcoma; Giant Cell Tumor of Bone; Hemangiopericytoma; Gastrointestinal Stromal Tumor (GIST)

  20. Paratesticular Pleomorphic Rhabdomyosarcoma: A Report of Two Cases

    PubMed Central

    Boulma, Rami; Gargouri, Mohamed Mourad; Sallemi, Ahmed; Chlif, Mohamed; Fitouri, Zouhaier; Kallel, Yosri

    2013-01-01

    Pleomorphic rhabdomyosarcoma (RMS) is a rare tumor with an aggressive behavior, described mainly in adulthood. Herein we present two cases of paratesticular pleomorphic RMS in 71- and 16-year-old patients with metastases at initial diagnosis. Histological, immunohistochemical, and ultrastructural findings were essential to confirm diagnosis. Few months after radical orchiectomy, both patients died before or just after starting adjuvant chemotherapy. PMID:23476881

  1. [Pleomorphic adenoma in ectopic salivary tissue in a child

    PubMed

    Clarós, P; Turcanu, D; Clarós, A; Clarós, A; Vila Torres, J

    2000-01-01

    Benign tumors appearing in cervical ectopic salivary tissue are rare. Most of these tumors are pleomorphic adenomas and many occur in adults. We report two cases of pleomorphic adenoma developing in cervical ectopic salivary tissue in children and review the pathogenesis of salivary heterotopia and these benign tumors.

  2. [Primary pleomorphic adenoma of the principal paranasal sinus].

    PubMed

    Flöttmann, T; Helling, K

    1995-10-01

    The pleomorphic adenoma is the most frequent benign tumor occurring in the salivary glands. These tumors are very uncommon in the nasal fossae and paranasal sinuses. We report a case of pleomorphic adenoma in the nasal fossa and review the currently available literature. Different tumor localizations and therapeutic concepts are discussed.

  3. Ossifying Parotid Carcinoma ex Pleomorphic Adenoma

    PubMed Central

    Mohan, Suresh; Puram, Sidharth V.; Yarlagadda, Bharat; Nosé, Vania; Deschler, Daniel G.

    2015-01-01

    We present a unique case of an extensively ossified carcinoma ex pleomorphic adenoma (CXPA) in a 76-year-old man with a five-year history of a slowly growing parotid mass. Fine-needle aspiration of the mass was nondiagnostic. A computed tomography (CT) scan of the lesion revealed a well-circumscribed mass with peripheral calcification. Initial pathological analysis suggested a benign parotid mass, but rigorous decalcification revealed noninvasive CXPA. The patient underwent complete resection of the mass and remained disease-free nine months later. Extensive ossification of a seemingly benign parotid mass may mask areas of carcinoma that may progress if left untreated. PMID:26075129

  4. Pleomorphic adenoma of the human female breast.

    PubMed

    Agnantis, N J; Maounis, N; Priovolou-Papaevangelou, M; Baltatzis, I

    1992-02-01

    We are presenting an interesting rare benign breast tumor which meets the characteristics of a salivary gland pleomorphic adenoma. The tumor was misdiagnosed during frozen section procedure, because several clusters, mainly composed of myoepithelial cells and surrounded by a chondroid matrix, were mistaken for cancerous blasts. Additionally the clinical and mammographic findings were very suspicious. Although this particular tumor is very infrequent, the pathologist should be aware of the difficulties in the differential diagnosis during frozen section and thus defer his final answer to the paraffin sections.

  5. Pleomorphic adenoma of the human breast.

    PubMed

    Makek, M; von Hochstetter, A R

    1980-01-01

    Pleomorphic adenomas of the human breast are histologically identical to those of salivary glands. To date, the literature reports but 15 such benign tumors, all of which occurred in women. The present paper describes clinical and pathologic findings in three further cases, including one man. Considered together, unnecessary mastectomy occurred in 44.4%, indicating the importance of separating this benign entity from malignancies with stromal metaplasia. In case of examination by frozen section, a peripheral portion, if not the entire tumor, should be submitted to the pathologist.

  6. Surgical Outcomes of High-Grade Spinal Cord Gliomas

    PubMed Central

    Hida, Kazutoshi; Yano, Syunsuke; Aoyama, Takeshi; Koyanagi, Izumi; Houkin, Kiyohiro

    2015-01-01

    Study Design A retrospective study. Purpose The purpose of this study was to obtain useful information for establishing the guidelines for treating high-grade spinal cord gliomas. Overview of Literature The optimal management of high-grade spinal cord gliomas remains controversial. We report the outcomes of the surgical management of 14 high-grade spinal glioma. Methods We analyzed the outcomes of 14 patients with high-grade spinal cord gliomas who were surgically treated between 1989 and 2012. Survival was charted with the Kaplan-Meier plots and comparisons were made with the log-rank test. Results None of the patients with high-grade spinal cord gliomas underwent total resection. Subtotal resection was performed in two patients, partial resection was performed in nine patients, and open biopsy was performed in three patients. All patients underwent postoperative radiotherapy and six patients further underwent radiation cordotomy. The median survival time for patients with high-grade spinal cord gliomas was 15 months, with a 5-year survival rate of 22.2%. The median survival time for patients with World Health Organization grade III tumors was 25.5 months, whereas the median survival time for patients with glioblastoma multiforme was 12.5 months. Both univariate and multivariate Cox proportional hazards models demonstrated a significant effect only in the group that did not include cervical cord lesion as a factor associated with survival (p=0.04 and 0.03). Conclusions The surgical outcome of patients diagnosed with high-grade spinal cord gliomas remains poor. Notably, only the model which excluded cervical cord lesions as a factor significantly predicted survival. PMID:26713128

  7. Hepatocyte Growth Factor-mediated satellite cells niche perturbation promotes development of distinct sarcoma subtypes

    PubMed Central

    Morena, Deborah; Maestro, Nicola; Bersani, Francesca; Forni, Paolo Emanuele; Lingua, Marcello Francesco; Foglizzo, Valentina; Šćepanović, Petar; Miretti, Silvia; Morotti, Alessandro; Shern, Jack F; Khan, Javed; Ala, Ugo; Provero, Paolo; Sala, Valentina; Crepaldi, Tiziana; Gasparini, Patrizia; Casanova, Michela; Ferrari, Andrea; Sozzi, Gabriella; Chiarle, Roberto; Ponzetto, Carola; Taulli, Riccardo

    2016-01-01

    Embryonal Rhabdomyosarcoma (ERMS) and Undifferentiated Pleomorphic Sarcoma (UPS) are distinct sarcoma subtypes. Here we investigate the relevance of the satellite cell (SC) niche in sarcoma development by using Hepatocyte Growth Factor (HGF) to perturb the niche microenvironment. In a Pax7 wild type background, HGF stimulation mainly causes ERMS that originate from satellite cells following a process of multistep progression. Conversely, in a Pax7 null genotype ERMS incidence drops, while UPS becomes the most frequent subtype. Murine EfRMS display genetic heterogeneity similar to their human counterpart. Altogether, our data demonstrate that selective perturbation of the SC niche results in distinct sarcoma subtypes in a Pax7 lineage-dependent manner, and define a critical role for the Met axis in sarcoma initiation. Finally, our results provide a rationale for the use of combination therapy, tailored on specific amplifications and activated signaling pathways, to minimize resistance emerging from sarcomas heterogeneity. DOI: http://dx.doi.org/10.7554/eLife.12116.001 PMID:26987019

  8. [Pulmonary pleomorphic carcinoma; report of a case].

    PubMed

    Usami, N; Fukui, T; Ito, S; Sato, N; Uchiyama, M; Taniguchi, T; Yoshioka, H; Yokoi, K

    2005-10-01

    We report a case of a 64-year-old man with pleomorphic carcinoma of the lung and thymic cyst. He was admitted to our hospital because of an abnormal shadow observed on chest X-ray. Computed tomography (CT) showed a mass lesion located in the right upper lobe and a non-invasive anterior mediastinal tumor adjacent to the left brachiocepharic vein. On enhanced CT, the lung mass showed central low-attenuation areas with a substantial enhancement in the periphery. Preoperative transbronchial blushing cytology of the mass revealed adenocarcinoma. With a diagnosis of primary lung cancer (cT3N0M0) and mediastinal tumor, an operation was performed through a median sternotomy. The mediastinal tumor was excised and a right upper lobectomy and were also accomplished, because the lung tumor did not show adhesion or pleural invasion. Histopathologic examination of the resected specimen revealed that the lung tumor composed of a mixture of spindle and giant cell features and contained a component of adenocarcinoma and squamous cell carcinoma. This finding yielded a pathological diagnosis of pleomorphic carcinoma (pT2N0M0). The mediastinal tumor was diagnosed as thymic cyst. The postoperative course was uneventful, and he is currently well 6 months after surgery. PMID:16235854

  9. Mutations in chromatin machinery and pediatric high-grade glioma.

    PubMed

    Lulla, Rishi R; Saratsis, Amanda Muhs; Hashizume, Rintaro

    2016-03-01

    Pediatric central nervous system tumors are the most common solid tumor of childhood. Of these, approximately one-third are gliomas that exhibit diverse biological behaviors in the unique context of the developing nervous system. Although low-grade gliomas predominate and have favorable outcomes, up to 20% of pediatric gliomas are high-grade. These tumors are a major contributor to cancer-related morbidity and mortality in infants, children, and adolescents, with long-term survival rates of only 10 to 15%. The recent discovery of somatic oncogenic mutations affecting chromatin regulation in pediatric high-grade glioma has markedly improved our understanding of disease pathogenesis, and these findings have stimulated the development of novel therapeutic approaches targeting epigenetic regulators for disease treatment. We review the current perspective on pediatric high-grade glioma genetics and epigenetics, and discuss the emerging and experimental therapeutics targeting the unique molecular abnormalities present in these deadly childhood brain tumors. PMID:27034984

  10. Mutations in chromatin machinery and pediatric high-grade glioma

    PubMed Central

    Lulla, Rishi R.; Saratsis, Amanda Muhs; Hashizume, Rintaro

    2016-01-01

    Pediatric central nervous system tumors are the most common solid tumor of childhood. Of these, approximately one-third are gliomas that exhibit diverse biological behaviors in the unique context of the developing nervous system. Although low-grade gliomas predominate and have favorable outcomes, up to 20% of pediatric gliomas are high-grade. These tumors are a major contributor to cancer-related morbidity and mortality in infants, children, and adolescents, with long-term survival rates of only 10 to 15%. The recent discovery of somatic oncogenic mutations affecting chromatin regulation in pediatric high-grade glioma has markedly improved our understanding of disease pathogenesis, and these findings have stimulated the development of novel therapeutic approaches targeting epigenetic regulators for disease treatment. We review the current perspective on pediatric high-grade glioma genetics and epigenetics, and discuss the emerging and experimental therapeutics targeting the unique molecular abnormalities present in these deadly childhood brain tumors. PMID:27034984

  11. Photodynamic therapy of Cervical Intraepithelial Neoplasia (CIN) high grade

    NASA Astrophysics Data System (ADS)

    Carbinatto, Fernanda M.; Inada, Natalia M.; Lombardi, Welington; da Silva, Eduardo V.; Belotto, Renata; Kurachi, Cristina; Bagnato, Vanderlei S.

    2016-02-01

    Cervical intraepithelial neoplasia (CIN) is the precursor of invasive cervical cancer and associated with human papillomavirus (HPV) infection. Photodynamic therapy (PDT) is a technique that has been used for the treatment of tumors. PDT is based on the accumulation of a photosensitizer in target cells that will generate cytotoxic reactive oxygen species upon illumination, inducing the death of abnormal tissue and PDT with less damaging to normal tissues than surgery, radiation, or chemotherapy and seems to be a promising alternative procedure for CIN treatment. The CIN high grades (II and III) presents potential indications for PDT due the success of PDT for CIN low grade treatment. The patients with CIN high grade that were treated with new clinic protocol shows lesion regression to CIN low grade 60 days after the treatment. The new clinical protocol using for treatment of CIN high grade shows great potential to become a public health technique.

  12. What Is Uterine Sarcoma?

    MedlinePlus

    ... supporting tissues of the uterus (womb). About the uterus The uterus is a hollow organ, about the ... a baby out during childbirth. Cancers of the uterus and endometrium Sarcomas are cancers that start from ...

  13. Epidemic Kaposi Sarcoma

    MedlinePlus

    ... therapy are used to treat Kaposi sarcoma lesions . Photon radiation therapy treats lesions with high-energy light. ... complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information ...

  14. Classic Kaposi Sarcoma

    MedlinePlus

    ... therapy are used to treat Kaposi sarcoma lesions . Photon radiation therapy treats lesions with high-energy light. ... complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information ...

  15. Synovial sarcoma of the parotid gland: a case report and review of the literature.

    PubMed

    Rigante, M; Visocchi, M; Petrone, G; Mulè, A; Bussu, F

    2011-02-01

    Parotid gland tumours are very heterogeneous, being benign in 80% of cases, and generally arising from epithelial cells. Nevertheless, a small group of non-epithelial tumours representing just 5% of all salivary gland neoplasms has also been reported, the most common of these being haemangioma, especially in children. However, lymphomas, neuromas, neurofibromas, lipomas and sarcomas can also be found. Synovial cell sarcoma is a high grade histological variety of sarcoma and is generally located near large joints and bursae of the lower extremities, such as knee, tendon sheaths and bursal structures. It is rarely found in the head and neck region due to its lack of synovioblastic tissue. Herewith, the case of a young female, affected by a synovial sarcoma of the left parotid gland, is presented and a review is made of the literature on this rare specific localization focusing on management and outcome.

  16. Cystic Change in Pleomorphic Adenoma: A Rare Finding and a Diagnostic Dilemma

    PubMed Central

    Khetrapal, Shaan; Jetley, Sujata; Hassan, Mohd. Jaseem

    2015-01-01

    Pleomorphic adenoma forms the majority of salivary gland neoplasms. Cystic change in pleomorphic adenomas is a diagnostic dilemma and can mimic mucoepidermoid carcinoma, mucocele or carcinoma ex pleomorphic adenoma and squamous cell carcinoma. Hereby we report this interesting and rare case of cystic pleomorphic adenoma in a 32-year-old male. PMID:26675071

  17. Osteogenic Sarcoma: A 21st Century Review.

    PubMed

    Osasan, Stephen; Zhang, Mingyong; Shen, Fan; Paul, Paulose J; Persad, Sujata; Sergi, Consolato

    2016-09-01

    Compared to other bone tumors, bone osteogenic sarcoma (BOS) continues to confer a much grimmer prognosis as the survival benefit of traditional chemotherapy treatment regimens is still unsatisfactory. Chemotherapy was demonstrated to be effective in eradicating both primary tumor and pulmonary metastases in the last century, with effective agents used in various combination regimens having changed the survival rate from less than 10% to 75%. The most common primary bone cancer, BOS is conventionally a primary intramedullary high-grade malignant tumor characterized by malignant cells forming immature bone or osteoid. BOS is a disease with diverse morphological presentations. The treatment of all morphological variants seem to have been the same for over 30 years. The introduction of antiproliferative agents such as insulin growth factor-binding protein 3 hold promise of a potentially veritable therapeutic target. In this review, we highlight recent data on osteosarcoma to consolidate a platform able to connect bench and bedside. PMID:27630274

  18. Ewing Sarcoma of the External Ear Canal.

    PubMed

    Binnetoglu, Adem; Baglam, Tekin; Tokuc, Gulnur; Kecelioglu Binnetoglu, Kiymet; Gerin, Fatma; Sari, Murat

    2016-01-01

    Background. Ewing sarcoma (ES) is a high-grade malignant tumor that has skeletal and extraskeletal forms and consists of small round cells. In the head and neck region, reported localization of extraskeletal ES includes the larynx, thyroid gland, submandibular gland, nasal fossa, pharynx, skin, and parotid gland, but not the external ear canal. Methods. We present the unique case of a 2-year-old boy with extraskeletal ES arising from the external ear canal, mimicking auricular hematoma. Results. Surgery was performed and a VAC/IE (vincristine, adriamycin, cyclophosphamide alternating with ifosfamide, and etoposide) regimen was used for adjuvant chemotherapy for 12 months. Conclusion. The clinician should consider extraskeletal ES when diagnosing tumors localized in the head and neck region because it may be manifested by a nonspecific clinical picture mimicking common otorhinolaryngologic disorders. PMID:27313930

  19. A functioning pleomorphic carcinoma of the thyroid.

    PubMed Central

    Karnauchow, P. N.

    1976-01-01

    A metastasizing functioning pleomorphic carcinoma of the thyroid displayed a morphologic piture ranging from follicular to sarcomatous and carcinoid-like patterns. Spindle-cell elements were believed to be the result of metaplasia of the follicular epithelium. Development of carcinoid-like lesions in the secondary deposits of tumour supported the contention that so-called parafollicular or c-cells may be a phenomenon of metaplasia and not a histologic entity. The tumour was associated with a chromophobe adenoma of the pituitary and a benign schwannoma of the stomach. Clinically the patient did not show any other endocrinologic abnormalities and lived for 4 years from the time of diagnosis of the thyroid tumour. Images FIG. 1 FIG. 2 FIG. 3 FIG. 4 PMID:1277059

  20. Pleomorphic adenoma causing acute airway obstruction.

    PubMed

    Moraitis, D; Papakostas, K; Karkanevatos, A; Coast, G J; Jackson, S R

    2000-08-01

    A case is reported of a pleomorphic adenoma of the minor salivary glands of the oral cavity presenting with acute airway obstruction. This is the first reported case to our knowledge of a mixed salivary tumour of the upper respiratory tract causing upper airway obstruction and acute respiratory failure. The patient had to be intubated and transferred to the intensive care unit. After an elective tracheostomy was performed, the adenoma was excised from its fibrous capsule. It was found to originate from the soft palate and occupied the parapharyngeal space. A high index of suspicion should be kept in order to diagnose tumours of the parapharyngeal space with unusual presentation. These tumours which are usually benign should be considered in the differential diagnosis from more common infectious or traumatic conditions and surgical morbidity should be minimal.

  1. Pleomorphic adenoma of the buccal salivary gland.

    PubMed

    Khandekar, Shubhangi; Dive, Alka; Munde, Prashant; Wankhede, Neena Dongre

    2015-01-01

    Salivary gland swellings can result from tumors, an inflammatory process or cysts. It can sometimes be difficult to establish; whether pathology arises from the salivary gland itself or adjacent structures. Neoplasms of the salivary glands account for less than 1% of all tumors, 3-5% of all head and neck tumors and benign pleomorphic adenoma (PA) of minor salivary glands arising de novo is very rare. PA is the most common tumor of the salivary gland. While the majority arises from the parotid gland, only a small percentage arises from the buccal minor salivary gland. A case of PA of minor salivary glands in the buccal mucosa in a 70-year-old female is discussed. It includes review of literature, clinical features, histopathology, radiological findings and treatment of the tumor; with emphasis on diagnosis.

  2. Multiplex RT-PCR assay for the differential diagnosis of alveolar rhabdomyosarcoma and Ewing's sarcoma.

    PubMed Central

    Downing, J. R.; Khandekar, A.; Shurtleff, S. A.; Head, D. R.; Parham, D. M.; Webber, B. L.; Pappo, A. S.; Hulshof, M. G.; Conn, W. P.; Shapiro, D. N.

    1995-01-01

    Cytogenetic analysis has defined specific translocations associated with two of the most common small round cell tumors of childhood, t(11;22) in Ewing's sarcoma and t(2;13) in alveolar rhabdomyosarcoma. We and others have previously demonstrated the diagnostic utility of a reverse transcriptase polymerase chain reaction (RT-PCR) assay for the detection of the t(11;22) encoded EWS/FLI-1 chimeric message in Ewing's sarcoma. More recently, we have cloned the t(2;13)(q35;q14) translocation and have shown that it results in the fusion of the PAX3 gene on chromosome 2 to FKHR, a novel member of the fork-head family of transcription factors on chromosome 13. To define the morphological spectrum of childhood sarcomas that express the t(2;13) encoded PAX3/FKHR chimeric message, we have performed RT-PCR analysis on samples from 44 primary pediatric sarcomas and 8 sarcoma cell lines. PAX3/FKHR chimeric messages were detected in 24 of 27 alveolar, 2 of 12 embryonal, and 0 of 1 pleomorphic rhabdomyosarcoma and in 1 of 2 ectomesenchymomas. In contrast, none of 8 Ewing's sarcomas or 2 undifferentiated sarcomas expressed this message. Chimeric transcripts were detected in all cases with cytogenetic evidence of the (2;13) translocation, and in each case the chimeric PAX3/FKHR message had the identical junction sequence, suggesting that genomic chromosome breaks were clustered in a single intron in both genes. By combining the PAX3/FKHR RT-PCR assay with primers for detection of the Ewing's sarcoma t(11;22) encoded EWS/FLI-1 chimeric transcript, we have developed a multiplex RT-PCR reaction that allows the rapid and accurate identification of either translocation in a biopsy sample. Images Figure 1 Figure 2 Figure 3 Figure 4 PMID:7887445

  3. Langerhans cell sarcoma: an unusual microscopic presentation.

    PubMed

    Gagnon, A L; Daniel, S; Greer, K; Patterson, J W; Tchernev, G; Chokoeva, A A; Wollina, U; Lotti, T; Fioranelli, M; Roccia, M G; Guarneri, C; Aguilera, N

    2016-01-01

    A 70-year-old Caucasian male presented to our clinic for a pruritic eruption progressing over several months. He complained of fatigue with a 20-pound weight loss over the past year. On presentation, the patient had browny-yellow to violaceous, purpuric, macular and papular lesions on the legs, arms, lower abdomen and back. Initial biopsy showed an angiocentric infiltrate with a suggestion of intraluminal proliferation; CD31 and Fli-1 positivity suggested either reactive angioendotheliomatosis or an unusual intravascular histiocytosis. Further excisional biopsies demonstrated perivascular collections of cells with ample cytoplasm, prominent nuclear pleomorphism and mitotic activity. The nuclei demonstrated nuclear folding, grooves and indentations. The atypical cells were S100, CD1a and CD56 positive with immunohistochemistry. A diagnosis of Langerhans cell sarcoma (LCS) was made. LCS is a rare, aggressive malignancy that can involve multiple organs including the skin, lymph nodes, lung, bone marrow, spleen, heart, and brain. The skin and lymph nodes are commonly involved, and the cutaneous presentation varies greatly. Immunohistochemistry characteristically shows CD1a and S100 positivity. CD56 expression is uncommon and often portends a poor prognosis. There is no established treatment of LCS due to its rarity. Surgery, radiation, and chemotherapy have been used with varied outcomes. Our patient was treated with prednisone with improvement of cutaneous disease. He did not develop systemic involvement, but died 1.5 years later from complications associated with heart failure. Langerhans cell sarcoma should be considered when faced with an unusual angiocentric infiltrate in which initial immunohistochemical staining results may be misleading. PMID:27373133

  4. General Information about Ewing Sarcoma

    MedlinePlus

    ... Research Ewing Sarcoma Treatment (PDQ®)–Patient Version General Information About Ewing Sarcoma Go to Health Professional Version ... the PDQ Pediatric Treatment Editorial Board . Clinical Trial Information A clinical trial is a study to answer ...

  5. General Information about Kaposi Sarcoma

    MedlinePlus

    ... Research Kaposi Sarcoma Treatment (PDQ®)–Patient Version General Information About Kaposi Sarcoma Go to Health Professional Version ... the PDQ Adult Treatment Editorial Board . Clinical Trial Information A clinical trial is a study to answer ...

  6. General Information About Uterine Sarcoma

    MedlinePlus

    ... Research Uterine Sarcoma Treatment (PDQ®)–Patient Version General Information About Uterine Sarcoma Go to Health Professional Version ... the PDQ Adult Treatment Editorial Board . Clinical Trial Information A clinical trial is a study to answer ...

  7. Aberrant lacrimal gland and pleomorphic adenoma within the muscle cone.

    PubMed

    Mueller, E C; Borit, A

    1979-04-01

    Aberrant lacrimal gland tissue within the muscle cone formed a pleomorphic adenoma (benign mixed tumor). Histopathologically, the lesion was identical to similar neoplasms originating from lacrimal and other salivary glands as well as from other serous glands of the body.

  8. Parotid tail pleomorphic adenoma extending to the parapharyngeal space.

    PubMed

    Polat, Kerem; Doğan, Mansur; Yüce, Salim; Uysal, Ismail Önder; Müderris, Suphi

    2013-03-01

    Parapharyngeal space tumors are rare, accounting for 0.5% of head and neck neoplasms. Most of them are benign and originate in the salivary glands, especially the pleomorphic adenoma. We presented a 47-year-old man with parotid tail pleomorphic adenoma extending to the parapharyngeal space. The patient applied to our clinic with the complaints of a painless mass on his neck and in his mouth for 3 months. After fine needle aspiration biopsy, the mass was diagnosed as pleomorphic adenoma. The patient was hospitalized and operated in our clinic. As we see in literature review, parapharyngeal space tumors are rare, and most of them are pleomorphic adenomas arising from the deep lobe of the parotid gland and extend into the PPS.

  9. Carcinoma ex pleomorphic adenoma of the soft palate.

    PubMed

    Yoshihara, T; Tanaka, M; Itoh, M; Ishii, T

    1995-03-01

    A case of carcinoma ex pleomorphic adenoma arising in the soft palate is reported. The tumour presented had enlarged gradually over 10 years and finally occupied the oral cavity. The patient was admitted to our hospital due to disturbance of her speech and swallowing, and a sudden haemorrhage from the tumour. The initial pathological diagnosis by open biopsy was benign pleomorphic adenoma. After total resection, histological examination revealed that the tumour was composed partly of benign pleomorphic adenoma and partly of an adenocarcinomatous component. The carcinoma cells with prominent nucleoli were spheroid or polygonal in shape, and frequently formed ductal structures with areas of necrosis. Mitoses were also found. These findings showed that this tumour was a secondary carcinoma which had developed in a preexisting pleomorphic adenoma.

  10. [Cylindroma on a pleomorphic adenoma. Apropos of 2 case reports].

    PubMed

    Brocheriou, C; Baudin, J P; Verola, O

    1985-01-01

    Among a group of 863 patients with epithelial tumors of the salivary glands, including 470 with pleomorphic adenomas, the latter were associated with carcinomas in 20 cases and of these two were adenoid cystic carcinoma. In both cases these were isolated parotid tumors, lacking functional symptomatology and failing to recur 2 and 4 years respectively after parotidectomy. Diagnosis was mainly by histology and was based on the finding of a co-existing pleomorphic adenoma and an authentic adenoid cystic carcinoma, not to be confused with the benign pseudo-cylindromatous appearance sometimes detected in simple pleomorphic adenomas. This association of adenoid cystic carcinoma and pleomorphic adenoma increases the risk of recurrence and of metastases--long-term follow up is necessary. Prognosis is dependent mainly on the quality of the initial surgical excision.

  11. Sarcomas and pharmacogenetics.

    PubMed

    Biason, Paola; Toffoli, Giuseppe

    2005-09-01

    Sarcomas are a heterogeneous group of tumors, requiring different chemotherapeutic approaches. Recently, several regimens for metastatic tumors were evaluated with respect to the different responses to conventional chemotherapy of the various histologic subtypes of sarcomas. The impact of pharmacogenetics in the progress of chemotherapy appears to be crucial in defining the clinical response to many drugs, such as anthracycline or alkylating agents, that are widely used in treatment regimens for soft tissue sarcomas (STS) or sarcomas of the bone. Polymorphisms of metabolizing enzymes (e.g., cytochrome P450 and glutathione-S-transferase), transporter proteins (reduced folate carrier and P-glycoprotein) or target proteins (thymidylate synthase, methylenetetrahydrofolate reductase, dihydrofolate reductase, and c-KIT) may be responsible for an altered clinical outcome, in terms of both response and toxicity. The administration of new chemotherapeutic agents, such as imatinib for gastrointestinal tumors (GIST), requires the study of genetic polymorphisms possibly affecting the integrity of the target (c-KIT), which may provide valid information regarding possible developments of therapy. For STS and sarcoma of the bone, the genetic markers, which could be unambiguously predictive of the phenotypic profile of patients, are as yet undetermined.

  12. Primary pleuropulmonary synovial sarcoma.

    PubMed

    Mirzoyan, Michael; Muslimani, Ala'a; Setrakian, Sebouh; Swedeh, Mohamed; Daw, Hamed A

    2008-09-01

    Pleuropulmonary synovial sarcoma (PPSS) is increasingly recognized as a subtype of sarcoma because of the recent identification of a distinctive chromosomal translocation specific to synovial sarcoma. Soft-tissue synovial sarcoma is far more common than PPSS and typically develops in para-articular locations of the extremities, affects young and middle-aged adults, with no difference in distribution between the sexes, and has well-documented radiologic manifestations. Pleuropulmonary synovial sarcoma can arise in the chest wall, heart, mediastinum, pleura, or lung, and it shares patient demographics and several imaging features with its soft-tissue counterpart. Patients present with a cough, chest pain, or dyspnea. On chest radiographs, PPSS typically appears as a sharply marginated mass with uniform opacity, based in the pleura or in the lung, and often accompanied by an ipsilateral pleural effusion. Computed tomographic images show a well-circumscribed, heterogeneously enhanced lesion without associated involvement of bone and without calcifications (except in the case of a chest wall primary tumor). Magnetic resonance imaging provides superior demonstration of nodular soft tissue and multilocular fluid-filled internal components of PPSS, in addition to peripheral rim enhancement after the intravenous administration of a gadoliniumbased contrast material such as gadopentetate dimeglumine. Current treatment consists of surgical resection followed by chemotherapy, radiation therapy, or both. PMID:18824448

  13. Primary Intracranial Synovial Sarcoma

    PubMed Central

    Li, Luyuan; Sinson, Grant; Mueller, Wade

    2016-01-01

    Background. Synovial sarcoma is an aggressive soft tissue sarcoma with uncertain histological origin. The pathology frequently presents as a localized disease, especially near large joints around the knee and thigh. Intracranial disease, which is rare, has been reported as metastasis from synovial sarcoma. We report a case with no obvious primary extracranial pathology, suggesting primary intracranial disease; this has not been reported in the literature. Case Description. A 21-year-old male, with a prior right skull lesion resection for atypical spindle cell neoplasm, presented with headaches, gait instability, left arm weakness, and left homonymous hemianopsia. CT of head demonstrated a right parietal hemorrhagic lesion with mass effect, requiring surgical decompression. Histopathology revealed synovial sarcoma. FISH analysis noted the existence of the t(X;18)(p11.2;q11.2) chromosomal translocation. PET scan did not show other metastatic disease. He underwent stereotactic radiotherapy and adjuvant chemotherapy. At 2-year follow-up, he remained nonfocal without recurrence. Conclusion. We report the first known case of primary intracranial synovial sarcoma. Moreover, we stress that intracranial lesions may have a tendency for hemorrhage, requiring urgent lifesaving decompression. PMID:27247811

  14. Primary Intracranial Synovial Sarcoma.

    PubMed

    Patel, Mohit; Li, Luyuan; Nguyen, Ha Son; Doan, Ninh; Sinson, Grant; Mueller, Wade

    2016-01-01

    Background. Synovial sarcoma is an aggressive soft tissue sarcoma with uncertain histological origin. The pathology frequently presents as a localized disease, especially near large joints around the knee and thigh. Intracranial disease, which is rare, has been reported as metastasis from synovial sarcoma. We report a case with no obvious primary extracranial pathology, suggesting primary intracranial disease; this has not been reported in the literature. Case Description. A 21-year-old male, with a prior right skull lesion resection for atypical spindle cell neoplasm, presented with headaches, gait instability, left arm weakness, and left homonymous hemianopsia. CT of head demonstrated a right parietal hemorrhagic lesion with mass effect, requiring surgical decompression. Histopathology revealed synovial sarcoma. FISH analysis noted the existence of the t(X;18)(p11.2;q11.2) chromosomal translocation. PET scan did not show other metastatic disease. He underwent stereotactic radiotherapy and adjuvant chemotherapy. At 2-year follow-up, he remained nonfocal without recurrence. Conclusion. We report the first known case of primary intracranial synovial sarcoma. Moreover, we stress that intracranial lesions may have a tendency for hemorrhage, requiring urgent lifesaving decompression. PMID:27247811

  15. JAZF1/SUZ12 gene fusion in endometrial stromal sarcomas.

    PubMed

    Hrzenjak, Andelko

    2016-01-01

    Endometrial stromal sarcomas (ESSs) belong to the rarest uterine malignancies (prevalence category <1-9/1,000,000). According to the new 2014 World Health Organisation (WHO) classification, they are separated into four categories; benign endometrial stromal nodules (ESNs), low grade endometrial stromal sarcomas (LG-ESSs), high-grade endometrial stromal sarcomas (HG-ESSs) and undifferentiated uterine sarcomas (UUSs). Due to heterogeneous histopathologic appearance these tumors still represent diagnostic challenge, even for experienced pathologists. ESSs are genetically very heterogeneous and several chromosomal translocations and gene fusions have so far been identified in these malignancies. To date the JAZF1/SUZ12 gene fusion is by far the most frequent and seems to be the cytogenetic hallmark of ESN and LG-ESS. Based on present literature data this gene fusion is present in approximately 75% of ESN, 50% of LG-ESS and 15% of HG-ESS cases. The frequency of JAZF1/SUZ12 appearance varies between classic ESS and different morphologic variants. This gene fusion is suggested to become a specific diagnostic tool, especially in difficult borderline cases. In combination with the recently described YWHAE/FAM22 gene fusion the JAZF1/SUZ12 fusion could be used to differentiate between LG-ESS and HG-ESS. The purpose of this review is to summarize literature data published in last two and a half decades about this gene fusion, as a contribution to our understanding of ESS genetics and pathogenesis. PMID:26879382

  16. [One case of pleomorphic adenoma originates from inferior nasal turbinate].

    PubMed

    Hao, Fang; Xu, Xuehai

    2014-10-01

    Pleomorphic adenoma (PA) is the most common benign tumor of the salivary glands. Originating from the nasal cavity is very rare. This paper reports one case of pleomorphic adenoma of the inferior nasal turbinate to analyze the clinic characteristic of this disease. Although these tumors are rarely seen in everyday practice, one should consider this possibility as an uncommon aetiology when confronted with an intranasal mass.

  17. Hyperthermia in soft tissue sarcoma.

    PubMed

    Lindner, Lars H; Issels, Rolf D

    2011-03-01

    Patients with high-risk soft tissue sarcomas (STS)-FNCLCC grade 2-3, size >5 cm, deep to the fascia-are at risk for developing local recurrence and distant metastasis despite surgical tumor resection. Therefore, the management of high-risk STS requires a multidisciplinary approach. Besides surgery, radiotherapy, and chemotherapy, regional hyperthermia (RHT) has the potential to become the fourth standard treatment modality for the treatment of these patients. RHT means non-invasive selective heating of the tumor area to temperatures within the range of 40-43°C for 60 min by the use of an electromagnetic heating device. Thereby RHT is always applied in addition to radiotherapy or chemotherapy or both but is not effective as a single treatment. Beside direct cytotoxicity, RHT in combination with chemotherapy enhances the drug cytotoxicity mainly by increased chemical reaction and intratumoral drug accumulation. For the neoadjuvant setting, RHT in combination with a doxorubicin- and ifosfamide-based chemotherapy has been shown to dramatically improve the tumor response rate but also prevents from early disease progression as compared to chemotherapy alone. The addition of RHT to a multimodal treatment of high-risk STS consisting of surgery, radiotherapy, and chemotherapy either in the neoadjuvant setting but also after incomplete or marginal tumor resection has been shown to significantly improve local recurrence- and disease-free survival. Based on these results and in conjunction with the low RHT-related toxicity, RHT combined with preoperative or postoperative chemotherapy should be considered as an additional standard treatment option for the multidisciplinary treatment of locally advanced high-grade STS.

  18. Multicentric myofibroblastic sarcoma

    PubMed Central

    Wechalekar, Mihir Dilip; Ayres, Oliver; Farshid, Gelareh; Clayer, Mark; Cleland, Leslie G

    2014-01-01

    We report a case of synchronous, multicentric low-grade myofibroblastic sarcoma presenting in a 62-year-old man. He initially presented with inflammatory symmetric polyarthritis and adhesive capsulitis of his shoulder and hips bilaterally and did not respond to a trial of disease modifying antirheumatic drugs. Over a period of several years he developed progressive restriction of both knees and nodules on his hands, both knees and back. A biopsy of the nodule on his back was inconclusive and subsequent biopsies on his left and then right knee revealed a spindle cell neoplasm with an infiltrative growth pattern, mitotic figures, positive immunostaining for smooth muscle actin and focal myxoid change consistent with myofibroblastic sarcoma. While myofibroblastic sarcoma has been known to metastasise, to our knowledge, a multifocal presentation of this tumour has not been described previously. PMID:25368122

  19. Comparison of proliferating cell nuclear antigen index in benign and malignant salivary pleomorphic adenoma.

    PubMed

    Yang, L; Liu, B; Qin, C; Hashimura, K; Yamada, T; Sumitomo, S; Mori, M

    1994-01-01

    The expression of proliferating cell nuclear antigen (PCNA) was studied in benign and malignant pleomorphic adenomas by using monoclonal antibody to PCNA. Carcinoma in pleomorphic adenoma (n = 8), cell-rich variant (n = 6) and typical pleomorphic adenoma (n = 6) were selected in this study. The PCNA index in carcinoma in pleomorphic adenoma showed a higher index of nuclear staining (mean 22.9%, S.D. 6.2) than in typical pleomorphic adenoma (mean 6.9%, S.D. 3.4) or a cell-rich variant of pleomorphic adenoma (mean 8.8%, S.D. 3.3). A significant difference in PCNA index was found between benign and malignant pleomorphic adenoma (P < 0.05). The present study suggests that PCNA index significantly differs between pleomorphic adenoma and carcinoma in pleomorphic adenoma, but in the prediction of malignant transformation potential it should be combined with routine histopathological examination.

  20. High Grade Leiomyosarcoma Mimicking a Recurrent Angiomyxoma in the Perineum

    PubMed Central

    Sood, Neha; Swaika, Abhisek; Hanooshi, Bashar; Waldorf, James; Peterson, Jennifer; Wu, Kevin; Attia, Steven; Dinh, Tri A.

    2015-01-01

    Perineal leiomyosarcoma is an extremely rare and aggressive cancer with a high metastatic potential and no defined standard treatment. There are only a few (six) reported cases in the literature. We report the case of a 67-year-old woman with a perineal leiomyosarcoma arising at the same site of a previously resected superficial angiomyxoma. Initially, she was treated for a presumptive recurrence of angiomyxoma. As she did not respond to medical therapy, she underwent repeat surgical excision. Pathology revealed a high grade leiomyosarcoma, histologically strikingly distinct from the initial diagnosis. She received adjuvant local radiation therapy, and remains without evidence of recurrent disease 36 months after completion of all therapy. This is the first reported case of a high grade perineal leiomyosarcoma originating at the same site as a resected benign superficial angiomyxoma. Our case emphasizes the necessity of a prompt histological diagnosis in cases of presumed recurrent perineal angiomyxoma. PMID:26266017

  1. High Grade Leiomyosarcoma Mimicking a Recurrent Angiomyxoma in the Perineum.

    PubMed

    Sood, Neha; Swaika, Abhisek; Hanooshi, Bashar; Waldorf, James; Peterson, Jennifer; Wu, Kevin; Attia, Steven; Dinh, Tri A

    2015-05-01

    Perineal leiomyosarcoma is an extremely rare and aggressive cancer with a high metastatic potential and no defined standard treatment. There are only a few (six) reported cases in the literature. We report the case of a 67-year-old woman with a perineal leiomyosarcoma arising at the same site of a previously resected superficial angiomyxoma. Initially, she was treated for a presumptive recurrence of angiomyxoma. As she did not respond to medical therapy, she underwent repeat surgical excision. Pathology revealed a high grade leiomyosarcoma, histologically strikingly distinct from the initial diagnosis. She received adjuvant local radiation therapy, and remains without evidence of recurrent disease 36 months after completion of all therapy. This is the first reported case of a high grade perineal leiomyosarcoma originating at the same site as a resected benign superficial angiomyxoma. Our case emphasizes the necessity of a prompt histological diagnosis in cases of presumed recurrent perineal angiomyxoma.

  2. [Extraskeletal Ewing's sarcoma].

    PubMed

    Baram, J; Tichler, T; Nass, D; Brenner, H J

    1992-01-01

    5 patients diagnosed as having extraskeletal Ewing's sarcoma have been referred to our adult oncology unit since 1980. All were men, ranging in age from 18-57 (mean 32 years). The primary tumor was located on the trunk in 4 and in an extremity in 1. Wide tumor excision was feasible in only 2. 3 died within 27 months and 2 are alive, 13 and 67 months, respectively, following diagnosis. This study demonstrates the highly aggressive nature of extraskeletal Ewing's sarcoma and the need for early diagnosis and efficient chemotherapy.

  3. Selective nonoperative management of high grade splenic trauma.

    PubMed

    Branco, Bernardino C; Tang, Andrew L; Rhee, Peter; Fraga, Gustavo Pereira; Nascimento, Bartolomeu; Rizoli, Sandro; O'Keeffe, Terence

    2013-01-01

    The "Evidence-based Telemedicine - Trauma & Acute Care Surgery" (EBT-TACS) Journal Club performed a critical review of the literature and selected three up-to-date articles on the management of splenic trauma. Our focus was on high-grade splenic injuries, defined as AAST injury grade III-V. The first paper was an update of the 2003 Eastern Association for the Surgery of Trauma (EAST) practice management guidelines for nonoperative management of injury to the spleen. The second paper was an American Association for the Surgery of Trauma (AAST) 2012 plenary paper evaluating the predictive role of contrast blush on CT scan in AAST grade IV and V splenic injuries. Our last article was from Europe and investigates the effects of angioembolization of splenic artery on splenic function after high-grade splenic trauma (AAST grade III-V). The EBT-TACS Journal Club elaborated conclusions and recommendations for the management of high-grade splenic trauma. PMID:23912375

  4. PLAG1 gene alterations in salivary gland pleomorphic adenoma and carcinoma ex-pleomorphic adenoma: a combined study using chromosome banding, in situ hybridization and immunocytochemistry.

    PubMed

    Martins, Carmo; Fonseca, Isabel; Roque, Lúcia; Pereira, Teresa; Ribeiro, Catarina; Bullerdiek, Jörn; Soares, Jorge

    2005-08-01

    Pleomorphic adenoma is the most common benign tumor of the salivary glands. It has marked histological diversity with epithelial, myoepithelial and mesenchymal-type cells arranged in a variety of architectural and differentiation patterns. Pleomorphic adenoma gene 1 (PLAG1), shown to be consistently rearranged in pleomorphic adenomas, is activated by chromosomal translocations involving 8q12, the chromosome region that is most frequently affected in these tumors. In this study, we evaluated PLAG1 involvement in salivary gland tumorigenesis by determining the frequency of its alterations in a selected group of 20 salivary gland tumors: 16 pleomorphic adenomas and four carcinomas ex-pleomorphic adenoma, having in common the presence of karyotypic chromosome 8 deviations, either structural, with 8q12 rearrangements, or numerical, with gain of chromosome 8. PLAG1 status was analyzed using in situ hybridization techniques, on metaphase cells, by fluorescence detection and/or interphase cells in paraffin sections, by chromogenic detection. Except for one pleomorphic adenoma case (5%) that lacked PLAG1 involvement, 17 tumors (85%), (14 pleomorphic adenomas and three carcinomas ex-pleomorphic adenoma) showed intragenic rearrangements of PLAG1 and the remaining two cases (10%), (one pleomorphic adenoma and one carcinoma ex-pleomorphic adenoma), had chromosome trisomy 8 only. To further investigate the role of PLAG1 on pleomorphic adenomas tumorigenesis, as well as the putative morphogenesis mechanism, we attempted to identify the cell types (epithelial vs myoepithelial) carrying 8q12/PLAG1 abnormalities by a combined phenotypic/genotypic analysis in four cases (three pleomorphic adenoma and one carcinoma ex-pleomorphic adenoma) characterized by 8q12 translocations and PLAG1 rearrangement. In these cases, both cells populations carried PLAG1 rearrangements. This finding further supports the pluripotent single-cell theory, which postulates that the tumor-initiated, modified

  5. Use of molecular studies for treatment of metastatic pleomorphic large cell pancreatic cancers-a novel strategy.

    PubMed

    Padhi, Parikshit; Narula, Arshjyot; Balog, Anna; Christou, Antonios

    2016-04-01

    Pleomorphic large cell pancreatic cancer is a rare and more aggressive variant with no proven treatment in the metastatic setting. It constitutes about 1% of the total pancreatic cancer cases. In the absence of any standard of care, we aim to increase awareness amongst clinical practitioners that molecular level testing, using immunohistochemistry, next-generation sequencing and chromogenic in-situ hybridization can help in making chemotherapeutic decisions for this variant of pancreatic cancer. We present a 50-year-old male who presented to our hospital complaining of persistent abdominal pain. CT scan revealed a pancreatic tail mass that was invading the splenic flexure causing high-grade obstruction. There was evidence of peritoneal studding. He underwent exploratory laparotomy with biopsy of the pancreatic mass and omentum which revealed metastatic undifferentiated pleomorphic large cell pancreatic cancer. Since there is no proven treatment for this particular entity, his specimen was sent for molecular testing. The molecular studies revealed positive mutations of TLE3 gene, EGFR, KRAS, PD1 gene, TP53 and TOP2A gene. The tumor was found to be sensitive to gemcitabine, paclitaxel, docetaxel, temozolamide, dacarbazine and doxorubicin. He was initiated on gemcitabine and nab-paclitaxel. The patient was treated based on these recommendations. The patient completed 5 cycles of gemcitabine and nab-paclitaxel. Treatment had to be held because of gemcitabine induced hemolytic uremic syndrome. Serial CT scans have shown stable disease and currently it has been 10 months since his diagnosis. Molecular level testing can be an important instrument in not only diagnosing but also be an important aid in deciding about the chemotherapeutic agents to be used in cases of metastatic pleomorphic large cell pancreatic cancer. Availability a knowledge of the novel tools like immunohistochemistry, next-generation sequencing and chromogenic in-situ hybridization can be prudent and

  6. Management of Bone Sarcoma.

    PubMed

    Gutowski, Christina J; Basu-Mallick, Atrayee; Abraham, John A

    2016-10-01

    Treatment of bone sarcoma requires careful planning and involvement of an experienced multidisciplinary team. Significant advancements in systemic therapy, radiation, and surgery in recent years have contributed to improved functional and survival outcomes for patients with these difficult tumors, and emerging technologies hold promise for further advancement. PMID:27542644

  7. Leukosis/Sarcoma Group

    Technology Transfer Automated Retrieval System (TEKTRAN)

    The leukosis/sarcoma (L/S) group of diseases designates a variety of transmissible benign and malignant neoplasms of chickens caused by members that belong to the family Retroviridae. Because the expansion of the literature on this disease, it is no longer feasible to cite all relevant publications ...

  8. Leukosis/Sarcoma Group

    Technology Transfer Automated Retrieval System (TEKTRAN)

    The leukosis/sarcoma (L/S) group of diseases designates a variety of transmissible benign and malignant neoplasms of chickens caused by members that belong to the family Retroviridae. Lymphoid leukosis has been the most common form of L/S group of diseases seen in field flocks, although myeloid leuk...

  9. Immunotherapy of Childhood Sarcomas.

    PubMed

    Roberts, Stephen S; Chou, Alexander J; Cheung, Nai-Kong V

    2015-01-01

    Pediatric sarcomas are a heterogeneous group of malignant tumors of bone and soft tissue origin. Although more than 100 different histologic subtypes have been described, the majority of pediatric cases belong to the Ewing's family of tumors, rhabdomyosarcoma and osteosarcoma. Most patients that present with localized stage are curable with surgery and/or chemotherapy; however, those with metastatic disease at diagnosis or those who experience a relapse continue to have a very poor prognosis. New therapies for these patients are urgently needed. Immunotherapy is an established treatment modality for both liquid and solid tumors, and in pediatrics, most notably for neuroblastoma and osteosarcoma. In the past, immunomodulatory agents such as interferon, interleukin-2, and liposomal-muramyl tripeptide phosphatidyl-ethanolamine have been tried, with some activity seen in subsets of patients; additionally, various cancer vaccines have been studied with possible benefit. Monoclonal antibody therapies against tumor antigens such as disialoganglioside GD2 or immune checkpoint targets such as CTLA-4 and PD-1 are being actively explored in pediatric sarcomas. Building on the success of adoptive T cell therapy for EBV-related lymphoma, strategies to redirect T cells using chimeric antigen receptors and bispecific antibodies are rapidly evolving with potential for the treatment of sarcomas. This review will focus on recent preclinical and clinical developments in targeted agents for pediatric sarcomas with emphasis on the immunobiology of immune checkpoints, immunoediting, tumor microenvironment, antibody engineering, cell engineering, and tumor vaccines. The future integration of antibody-based and cell-based therapies into an overall treatment strategy of sarcoma will be discussed.

  10. Pulmonary artery intimal sarcoma diagnosed using endobronchial ultrasound-guided transbronchial needle aspiration.

    PubMed

    Caraway, Nancy P; Salina, Davide; Deavers, Michael T; Morice, Rodolfo; Landon, Gene

    2015-01-01

    Intimal sarcoma of the pulmonary artery is a rare intraluminal malignant neoplasm that has an aggressive biological behavior, and early diagnosis may improve patient outcome. We describe a case of pulmonary artery intimal sarcoma diagnosed on cytologic material obtained by endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) biopsy with rapid on-site evaluation (ROSE). The aspirate showed loosely cohesive clusters of pleomorphic malignant spindled and epithelioid cells. An immunostain panel did not demonstrate any definitive mesenchymal or epithelial differentiation. The tumor's intraluminal origin was supported by radiographic imaging studies. Subsequently, the patient received preoperative chemotherapy and underwent tumor resection with reconstruction. This report describes the cytomorphologic features of this rare intravascular tumor and demonstrates how EBUS-TBNA with ROSE was instrumental in obtaining optimal cytologic sampling for ancillary studies, thus expediting the management.

  11. Pulmonary artery intimal sarcoma diagnosed using endobronchial ultrasound-guided transbronchial needle aspiration.

    PubMed

    Caraway, Nancy P; Salina, Davide; Deavers, Michael T; Morice, Rodolfo; Landon, Gene

    2015-01-01

    Intimal sarcoma of the pulmonary artery is a rare intraluminal malignant neoplasm that has an aggressive biological behavior, and early diagnosis may improve patient outcome. We describe a case of pulmonary artery intimal sarcoma diagnosed on cytologic material obtained by endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) biopsy with rapid on-site evaluation (ROSE). The aspirate showed loosely cohesive clusters of pleomorphic malignant spindled and epithelioid cells. An immunostain panel did not demonstrate any definitive mesenchymal or epithelial differentiation. The tumor's intraluminal origin was supported by radiographic imaging studies. Subsequently, the patient received preoperative chemotherapy and underwent tumor resection with reconstruction. This report describes the cytomorphologic features of this rare intravascular tumor and demonstrates how EBUS-TBNA with ROSE was instrumental in obtaining optimal cytologic sampling for ancillary studies, thus expediting the management. PMID:25745502

  12. Irradiation of Pediatric High-Grade Spinal Cord Tumors

    SciTech Connect

    Tendulkar, Rahul D.; Pai Panandiker, Atmaram S.; Wu Shengjie; Kun, Larry E.; Broniscer, Alberto; Sanford, Robert A.; Merchant, Thomas E.

    2010-12-01

    Purpose: To report the outcome using radiation therapy (RT) for pediatric patients with high-grade spinal cord tumors. Methods and Materials: A retrospective chart review was conducted that included 17 children with high-grade spinal cord tumors treated with RT at St. Jude Children's Research Hospital between 1981 and 2007. Three patients had gross total resection, 11 had subtotal resection, and 3 underwent biopsy. The tumor diagnosis was glioblastoma multiforme (n = 7), anaplastic astrocytoma (n = 8), or anaplastic oligodendroglioma (n = 2). Seven patients received craniospinal irradiation (34.2-48.6 Gy). The median dose to the primary site was 52.2 Gy (range, 38-66 Gy). Results: The median progression-free and overall survivals were 10.8 and 13.8 months, respectively. Local tumor progression at 12 months (79% vs. 30%, p = 0.02) and median survival (13.1 vs. 27.2 months, p = 0.09) were worse for patients with glioblastoma multiforme compared with anaplastic astrocytoma or oligodendroglioma. The median overall survival was shorter for patients when failure included neuraxis dissemination (n = 8) compared with local failure alone (n = 5), 9.6 vs. 13.8 months, p = 0.08. Three long-term survivors with World Health Organization Grade III tumors were alive with follow-up, ranging from 88-239 months. Conclusions: High-grade spinal cord primary tumors in children have a poor prognosis. The propensity for neuraxis metastases as a component of progression after RT suggests the need for more aggressive therapy.

  13. [Pleomorphic adenoma of the parotid gland, rules for resection].

    PubMed

    de Ridder, Mischa; Smeele, Ludi E; Balm, Alfons J M

    2012-01-01

    The importance of complete excision of a benign pleomorphic adenoma is illustrated by two patients' histories. A 28-year-old man underwent a local excision of a nodule under the left ear without histological confirmation. Ten years later he returned to our institute with a large multilocular process and subcutaneous nodules. Cytology showed pleomorphic adenoma. Patient was treated with total facial nerve preserving parotidectomy and radiotherapy. An 81-year-old male underwent a surgical removal of a swelling under his left ear eight years before admission for a large diffusely infiltrating tumor in the neck. Repeated cytology showed carcinoma ex pleomorphic adenoma. This tumor was inoperable and he was treated by palliative irradiation. In case of incomplete resection, pleomorphic adenoma cells are spilled with an increasing chance of local recurrence. Also degeneration into carcinoma ex pleomorphic adenoma is possible after incomplete resection, with impact on survival. These risks of residual disease determine the need of centralization of diagnosis and treatment of this benign parotid tumor.

  14. Cellular pleomorphism in papillary tumors of the pineal region

    PubMed Central

    Magalhães, Juliana; Rostad, Steven; Foltz, Greg; Pytel, Peter; Rodriguez, Fausto J.

    2015-01-01

    Papillary tumor of the pineal region (PTPR) is a recently recognized entity. We present the pathologic findings of two cases of PTPR as examples, and discuss the presence of cellular pleomorphism in these tumors. Patient 1 is a 48-year-old man with a pineal region mass. The tumor had unique biphasic patterns, papillary/pseudopapillary areas, and increased mitotic activity. Juxtaposed areas had marked pleomorphism, including nuclear enlargement, smudgy chromatin, nuclear pseudoinclusions, and cytoplasmic vacuolation. Mitoses were absent in these areas. Immunohistochemical staining revealed strong S100 expression. CAM 5.2 and CK18 were strongly positive in a patchy fashion. MIB1 labeling indices were high in classic PTPR regions but very low in pleomorphic areas. Patient 2 was a 35-year-old male with a pineal region tumor characterized by papillary architecture and overall cellular monotony, rare mitoses, and pleomorphism as a more isolated finding, with associated nuclear enlargement and crowding. S100 and CAM 5.2 labeling were present, and MIB1 labeling index was very low throughout the tumor. We discuss the pathologic and phenotypic features of PTPR. Variable pleomorphism may be present, reflected in size variation and nuclear hyperchromasia, but was not accompanied by increased proliferative activity in these cases, suggesting a degenerative phenomenon. PMID:22622671

  15. Cellular pleomorphism in papillary tumors of the pineal region.

    PubMed

    Magalhães, Juliana; Rostad, Steven; Foltz, Greg; Pytel, Peter; Rodriguez, Fausto J

    2013-04-01

    Papillary tumor of the pineal region (PTPR) is a recently recognized entity. We present the pathologic findings of two cases of PTPR as examples, and discuss the presence of cellular pleomorphism in these tumors. Patient 1 is a 48-year-old man with a pineal region mass. The tumor had unique biphasic patterns, papillary/pseudopapillary areas, and increased mitotic activity. Juxtaposed areas had marked pleomorphism, including nuclear enlargement, smudgy chromatin, nuclear pseudoinclusions, and cytoplasmic vacuolation. Mitoses were absent in these areas. Immunohistochemical staining revealed strong S100 expression. CAM 5.2 and CK18 were strongly positive in a patchy fashion. MIB1 labeling indices were high in classic PTPR regions but very low in pleomorphic areas. Patient 2 was a 35-year-old male with a pineal region tumor characterized by papillary architecture and overall cellular monotony, rare mitoses, and pleomorphism as a more isolated finding, with associated nuclear enlargement and crowding. S100 and CAM 5.2 labeling were present, and MIB1 labeling index was very low throughout the tumor. We discuss the pathologic and phenotypic features of PTPR. Variable pleomorphism may be present, reflected in size variation and nuclear hyperchromasia, but was not accompanied by increased proliferative activity in these cases, suggesting a degenerative phenomenon.

  16. High grade prostatic intraepithelial neoplasia with squamous differentiation

    PubMed Central

    Melissari, M; Beltran, A Lopez; Mazzucchelli, R; Froio, E; Bostwick, D G; Montironi, R

    2006-01-01

    An unusual variant of prostatic intraepithelial neoplasia with prominent and extensive squamous differentiation is described. The lesion was identified in the transition zone of a 79 year old man with a three year history of increasing urinary obstructive symptoms and a clinical diagnosis of benign prostatic hyperplasia who underwent simple prostatectomy. Two years after surgery, prostatic biopsies showed atrophy and mild chronic inflammation, with no evidence of malignancy. This unusual intraepithelial lesion seems not to have been described before and may represent a new variant of high grade prostatic intraepithelial neoplasia (HGPIN) with squamous differentiation. PMID:16567473

  17. Histopathological case report of high grade salivary duct carcinoma.

    PubMed

    Borovec, Jiri; Cegan, Martin; Mala, Katerina; Harmash, Natalia; Chramosta, Petr; Kobierzycki, Christopher; Bobek, Vladimir

    2015-01-01

    The case of a 39-year-old man with slowly growing mass in the superior part of left parotid region is described. Patient presented neurological symptoms including hypomobility of lower left eyelid and inability of complete closure of left side eyelids resulting in conjunctivitis and hyperlacrimation. Routine physical examination supported by image and laboratory tests was performed. Pathomorphological results of hematoxylin and eosin staining as well immunohistochemical examination in view of clinical presentation pointed to diagnosis of high grade salivary duct carcinoma. Rare incidence, histological view similar to breast cancer and body localization are sufficient reasons for further analyses and descriptions of this type of lesions. PMID:26691893

  18. Drugs Approved for Soft Tissue Sarcoma

    MedlinePlus

    ... Professionals Questions to Ask about Your Treatment Research Drugs Approved for Soft Tissue Sarcoma This page lists ... soft tissue sarcoma that are not listed here. Drugs Approved for Soft Tissue Sarcoma Cosmegen (Dactinomycin) Dactinomycin ...

  19. Misdiagnosis of primary pleomorphic rhabdomyosarcoma of the right thigh in a young adult: A case report

    PubMed Central

    Yu, Xiaolong; Yang, Yong; Zhang, Bin; Liu, Hucheng; Guo, Runsheng; Dai, Min

    2016-01-01

    Pleomorphic rhabdomyosarcoma (PRMS) is a rare type of soft tissue tumor accounting for <2% of all adult sarcomas. The present study describes a case of a 28-year-old male patient with primary PRMS of the right thigh. The patient was initially diagnosed with a schwannoma and underwent conservative therapy at a local hospital. At the 6-month follow-up, the patient reported a marked increase in the size of the mass. Finally, the patient underwent fine-needle aspiration and total tumor resection. The tumor measured 11×9×5 cm3 in size and was located in the vastus intermedius muscle. According to histological and immunohistochemical findings, a diagnosis of PRMS was confirmed by an expert pathology consultant. Postoperative follow-up at 3 months revealed no evidence of recurrent disease or residual side effects from therapy. However, it is imperative that such cases are closely monitored following surgery, in order to evaluate the long-term efficacy of the procedure, since misdiagnosis may increase the risk of recurrence and metastasis. The present case is noteworthy due to the misdiagnosis of PRMS, the large size of the mass and the young age of the patient. PMID:27588141

  20. Fluorescence and image guided resection in high grade glioma.

    PubMed

    Panciani, Pier Paolo; Fontanella, Marco; Schatlo, Bawarjan; Garbossa, Diego; Agnoletti, Alessandro; Ducati, Alessandro; Lanotte, Michele

    2012-01-01

    The extent of resection in high grade glioma is increasingly been shown to positively effect survival. Nevertheless, heterogeneity and migratory behavior of glioma cells make gross total resection very challenging. Several techniques were used in order to improve the detection of residual tumor. Aim of this study was to analyze advantages and limitations of fluorescence and image guided resection. A multicentric prospective study was designed to evaluate the accuracy of each method. Furthermore, the role of 5-aminolevulinc acid and neuronavigation were reviewed. Twenty-three patients harboring suspected high grade glioma, amenable to complete resection, were enrolled. Fluorescence and image guides were used to perform surgery. Multiple samples were obtained from the resection cavity of each lesion according to 5-ALA staining positivity and boundaries as delineated by neuronavigation. All samples were analyzed by a pathologist blinded to the intra-operative labeling. Decision-making based on fluorescence showed a sensitivity of 91.1% and a specificity of 89.4% (p<0.001). On the other hand, the image-guided resection accuracy was low (sensitivity: 57.8%; specificity: 57.4%; p=0.346). We observed that the sensitivity of 5-ALA can be improved by the combined use of neuronavigation, but this leads to a significant reduction in specificity. Thus, the use of auxiliary techniques should always be subject to critical skills of the surgeon. We advocate a large-scale study to further improve the assessment of multimodal approaches.

  1. PLAG1 expression is maintained in recurrent pleomorphic adenoma.

    PubMed

    de Brito, Beatriz Samara; Gaspar, Natália Giovanelli; Egal, Erika Said Abu; Sanchez-Romero, Celeste; Martins, Antonio Santos; Tincani, Álfio José; de Oliveira Gondak, Rogério; de Almeida, Oslei Paes; Kowalski, Luiz Paulo; Altemani, Albina; Mariano, Fernanda Viviane

    2016-10-01

    The proto-oncogene (pleomorphic adenoma gene 1 (PLAG1)) is immunohistochemically overexpressed in pleomorphic adenoma (PA). Its expression in recurrent pleomorphic adenoma (RPA), however, has not been investigated. Since complex mechanisms are involved in tumor recurrence, the aim of this study was to investigate whether PLAG1 overexpression occurs in RPA. We studied PLAG1 protein expression in 40 PAs and 36 RPAs by immunohistochemistry. Cases with immunopositive cells were classified into two categories, between 10 and 50 % and >50 %. In both groups, PLAG1 expression was observed in both epithelial and myoepithelial cells. Of PAs, 37 cases (93 %) were positive, while this was the case in 34 RPA cases (94 %). Our findings suggest that in addition to morphological similarity, PA and RPA express PLAG1, which might play a role in tumor recurrence. Furthermore, as for PA, expression of PLAG1 can be considered a valuable diagnostic marker for RPA.

  2. Remarkable Triple Pleomorphic Adenoma Affecting both Parotid and Submandibular Glands

    PubMed Central

    Pingarrón-Martín, Lorena; Arias-Gallo, L. J.; Demaría-Martínez, G.; Chamorro Pons, M.

    2014-01-01

    The objective of this article is to present the first case reported in the literature of metachronous pleomorphic adenoma of bilateral parotid glands and submaxillary gland. The authors report the case of a 27-year-old female with metachronous mixed tumors in her right parotid and submandibular glands. The patient has no history of previous radiotherapy. All three lesions were diagnosed by fine-needle aspiration. The histopathologic evaluation of all three major salivary gland masses demonstrated pleomorphic adenomas, with no occult malignancy observed on serial sections. The presentation of pleomorphic adenomas in the parotids and submandibular glands probably represents three unrelated primary sites of tumor, yet the possibility of metastasis from one gland to the other cannot be excluded. PMID:26000084

  3. An Unusual Localization of a Pleomorphic Adenoma in the Rhinopharynx

    PubMed Central

    Pagella, Fabio; Chu, Francesco; Pusateri, Alessandro; Matti, Elina

    2012-01-01

    Pleomorphic adenoma is the most common benign tumor of the parotid glands. Rarely it may arise from minor salivary glands of the upper aerodigestive tract. A 57-year-old woman was admitted at our institution presenting with nasal obstruction. Endoscopic evaluation revealed a pedicled mass in the rhinopharynx. After radiological examination, we opted for a transnasal endoscopic-assisted excision of the mass under general anaesthesia. Histological evaluation deponed for pleomorphic adenoma with clear surgical margins. No endoscopic evidence of local recurrence has been shown after 48 months of followup. In the literature, few cases of pleomorphic adenoma arising in the rhinopharynx have been reported. The introduction of endoscopy, as shown by our experience, leads to important benefits in the identification, treatment, and followup of such rhinopharyngeal benign tumors. PMID:22927861

  4. [Nucleolus organizer regions (NORs) in metastasizing pleomorphic adenoma].

    PubMed

    Landini, G; Kitano, M; Urago, A

    1990-12-01

    The metastasizing pleomorphic adenoma of the salivary glands is a rare variant of pleomorphic adenoma with a benign microscopical appearance, but malignant biological behaviour and production of metastasis. The histopathological study with the routine techniques is not enough for disclosing the nature of these tumors and they are most of the times underdiagnosed as benign. The correct diagnosis, almost always too late, is evident after several recurrences and the detection of metastatic foci. The number of nucleolar organizing regions (NORs) detected with the silver colloid method can be used in histopathology to determine the degree of cell activity. We report the findings in a case of metastasizing pleomorphic adenoma of the submandibular gland. This technique demonstrated to be useful for the diagnosis and characterization of the metabolism of these tumors.

  5. Functional Histology of Salivary Gland Pleomorphic Adenoma: An Appraisal.

    PubMed

    Triantafyllou, Asterios; Thompson, Lester D R; Devaney, Kenneth O; Bell, Diana; Hunt, Jennifer L; Rinaldo, Alessandra; Vander Poorten, Vincent; Ferlito, Alfio

    2015-09-01

    The complex microstructure of salivary gland pleomorphic adenoma is examined in relation to function. Events related to secretion of macromolecules and absorption, responses to the altered microenvironment and controversies concerning epithelial-mesenchymal transition versus modified myoepithelial differentiation are explored. Their effects on tumor cell phenotypes and arrangements are emphasized. Heterotopic differentiation and attempts at organogenesis are also considered. The approach allows interpreting microstructure independently of histogenetic perceptions, envisaging the tumor cells as a continuum, endorsing luminal structures as the principal components, and defining pleomorphic adenoma as a benign epithelial tumour characterized by variable epithelial-mesenchymal transition, secretion/differentiation and metaplasia.

  6. Pleomorphic adenoma originated from the inferior nasal turbinate.

    PubMed

    Unlu, H Halis; Celik, Onur; Demir, M Akif; Eskiizmir, Gorkem

    2003-12-01

    Although pleomorphic adenoma is the most common benign neoplasm of the salivary glands, it has also been reported to be present in the neck, ear, mediastinum, external nose and nasal cavity. Intranasal localization of this lesion is very rare and mainly originates from the nasal septum. From wherever the lesion originates, the main treatment modality should be surgical. We presented a very rare case of intranasal pleomorphic adenoma originated from the inferior nasal turbinate. Due to the expansile nature of the lesion, a midfacial degloving approach was preferred.

  7. Endoscopic resection of pleomorphic adenoma arising in the nasopharynx.

    PubMed

    Roh, Jong-Lyel; Jung, Bong Jin; Rha, Ki Sang; Park, Chan Ii

    2005-08-01

    Pleomorphic adenoma is the commonest benign tumor of the salivary glands. It has rarely been reported arising in the nasopharynx. A pleomorphic adenoma originating from the left lateral wall of the nasopharynx was found in a 61-year-old female who presented with nasal obstruction and intermittent epistaxis. It was successfully removed by combined transnasal and transoral surgery under endoscopic visualization. Endoscope-guided surgery may be helpful for the complete resection of benign nasopharyngeal tumors whilst minimizing injury to the Eustachian tube openings or velopharynx.

  8. Symptoms resembling temporomandibular joint disorder caused by a pleomorphic adenoma.

    PubMed

    Marchese, Nadia; Witterick, Ian; Freeman, Bruce V

    2013-01-01

    Pleomorphic adenoma is a benign neoplasm of the salivary glands. It is the most common type of salivary gland tumour and the tumour most commonly found in the parotid gland. Clinical diagnosis of a parotid gland neoplasm can be difficult, particularly when the lesion is located deep within the gland. Although usually asymptomatic, pleomorphic adenoma may exhibit symptoms mimicking those of conditions such as temporomandibular joint disorder. This case report highlights the difficulties of diagnosing this type of tumour and the importance of communication between physicians and dentists to ensure an accurate diagnosis.

  9. Current classification, treatment options, and new perspectives in the management of adipocytic sarcomas

    PubMed Central

    De Vita, Alessandro; Mercatali, Laura; Recine, Federica; Pieri, Federica; Riva, Nada; Bongiovanni, Alberto; Liverani, Chiara; Spadazzi, Chiara; Miserocchi, Giacomo; Amadori, Dino; Ibrahim, Toni

    2016-01-01

    Sarcomas are a heterogeneous group of mesenchymal tumors arising from soft tissue or bone, with an uncertain etiology and difficult classification. Soft tissue sarcomas (STSs) account for around 1% of all adult cancers. Till date, more than 50 histologic subtypes have been identified. Adipocyte sarcoma or liposarcoma (LPS) is one of the most common STS subtypes, accounting for 15% of all sarcomas, with an incidence of 24% of all extremity STSs and 45% of all retroperitoneal STSs. The new World Health Organization classification system has divided LPS into four different subgroups: atypical lipomatous tumor/well-differentiated LPS, dedifferentiated LPS, myxoid LPS, and pleomorphic LPS. These lesions can develop at any location and exhibit different aggressive potentials reflecting their morphologic diversity and clinical behavior. Patients affected by LPS should be managed in specialized multidisciplinary cancer centers. Whereas surgical resection is the mainstay of treatment for localized disease, the benefits of adjuvant and neoadjuvant chemotherapy are still unclear. Systemic treatment, particularly chemotherapy, is still limited in metastatic disease. Despite the efforts toward a better understanding of the biology of LPS, the outcome of advanced and metastatic patients remains poor. The advent of targeted therapies may lead to an improvement of treatment options and clinical outcomes. A larger patient enrollment into translational and clinical studies will help increase the knowledge of the biological behavior of LPSs, test new drugs, and introduce new methodological studies, that is, on treatment response. PMID:27785071

  10. Microchip-Associated Sarcoma in a Shrew (Suncus murinus)

    PubMed Central

    Schutt, Leah K; Turner, Patricia V

    2010-01-01

    A 16-mo-old female house musk shrew (Suncus murinus) with a 1-wk history of a rapidly growing subcutaneous mass in the interscapsular region was euthanized and submitted for necropsy. Macroscopic examination identified an irregular, well-demarcated, solid, tan-white subcutaneous mass. A small cavity containing a microchip device was present at the center of the mass. In addition, massive splenomegaly was evident grossly. Histologically, the subcutaneous mass comprised spindle cells arranged in a storiform pattern of interweaving bundles, consistent with a high-grade soft tissue sarcoma with multifocal necrosis. Immunohistochemical investigation suggested that the neoplastic cells were positive for neuron-specific enolase and (rarely) α-smooth muscle actin and negative for cytokeratin, desmin, S100, and vimentin. In light of the mesenchymal histopathologic phenotype and the lack of specific immunoreactivity pattern, the mass was considered to be most consistent with a poorly differentiated sarcoma. To our knowledge, this is the first report of a microchip-associated soft tissue sarcoma in a shrew. PMID:20858367

  11. Recommended names for pleomorphic genera in Dothideomycetes.

    PubMed

    Rossman, Amy Y; Crous, Pedro W; Hyde, Kevin D; Hawksworth, David L; Aptroot, André; Bezerra, Jose L; Bhat, Jayarama D; Boehm, Eric; Braun, Uwe; Boonmee, Saranyaphat; Camporesi, Erio; Chomnunti, Putarak; Dai, Dong-Qin; D'souza, Melvina J; Dissanayake, Asha; Gareth Jones, E B; Groenewald, Johannes Z; Hernández-Restrepo, Margarita; Hongsanan, Sinang; Jaklitsch, Walter M; Jayawardena, Ruvishika; Jing, Li Wen; Kirk, Paul M; Lawrey, James D; Mapook, Ausana; McKenzie, Eric H C; Monkai, Jutamart; Phillips, Alan J L; Phookamsak, Rungtiwa; Raja, Huzefa A; Seifert, Keith A; Senanayake, Indunil; Slippers, Bernard; Suetrong, Satinee; Taylor, Joanne E; Thambugala, Kasun M; Tian, Qing; Tibpromma, Saowaluck; Wanasinghe, Dhanushka N; Wijayawardene, Nalin N; Wikee, Saowanee; Woudenberg, Joyce H C; Wu, Hai-Xia; Yan, Jiye; Yang, Tao; Zhang, Ying

    2015-12-01

    This paper provides recommendations of one name for use among pleomorphic genera in Dothideomycetes by the Working Group on Dothideomycetes established under the auspices of the International Commission on the Taxonomy of Fungi (ICTF). A number of these generic names are proposed for protection because they do not have priority and/or the generic name selected for use is asexually typified. These include: Acrogenospora over Farlowiella; Alternaria over Allewia, Lewia, and Crivellia; Botryosphaeria over Fusicoccum; Camarosporula over Anthracostroma; Capnodium over Polychaeton; Cladosporium over Davidiella; Corynespora over Corynesporasca; Curvularia over Pseudocochliobolus; Elsinoë over Sphaceloma; Excipulariopsis over Kentingia; Exosporiella over Anomalemma; Exserohilum over Setosphaeria; Gemmamyces over Megaloseptoria; Kellermania over Planistromella; Kirschsteiniothelia over Dendryphiopsis; Lecanosticta over Eruptio; Paranectriella over Araneomyces; Phaeosphaeria over Phaeoseptoria; Phyllosticta over Guignardia; Podonectria over Tetracrium; Polythrincium over Cymadothea; Prosthemium over Pleomassaria; Ramularia over Mycosphaerella; Sphaerellopsis over Eudarluca; Sphaeropsis over Phaeobotryosphaeria; Stemphylium over Pleospora; Teratosphaeria over Kirramyces and Colletogloeopsis; Tetraploa over Tetraplosphaeria; Venturia over Fusicladium and Pollaccia; and Zeloasperisporium over Neomicrothyrium. Twenty new combinations are made: Acrogenospora carmichaeliana (Berk.) Rossman & Crous, Alternaria scrophulariae (Desm.) Rossman & Crous, Pyrenophora catenaria (Drechsler) Rossman & K.D. Hyde, P. dematioidea (Bubák & Wróbl.) Rossman & K.D. Hyde, P. fugax (Wallr.) Rossman & K.D. Hyde, P. nobleae (McKenzie & D. Matthews) Rossman & K.D. Hyde, P. triseptata (Drechsler) Rossman & K.D. Hyde, Schizothyrium cryptogamum (Batzer & Crous) Crous & Batzer, S. cylindricum (G.Y. Sun et al.) Crous & Batzer, S. emperorae (G.Y. Sun & L. Gao) Crous & Batzer, S. inaequale (G.Y. Sun & L

  12. Terahertz reflectometry imaging for low and high grade gliomas

    PubMed Central

    Ji, Young Bin; Oh, Seung Jae; Kang, Seok-Gu; Heo, Jung; Kim, Sang-Hoon; Choi, Yuna; Song, Seungri; Son, Hye Young; Kim, Se Hoon; Lee, Ji Hyun; Haam, Seung Joo; Huh, Yong Min; Chang, Jong Hee; Joo, Chulmin; Suh, Jin-Suck

    2016-01-01

    Gross total resection (GTR) of glioma is critical for improving the survival rate of glioma patients. One of the greatest challenges for achieving GTR is the difficulty in discriminating low grade tumor or peritumor regions that have an intact blood brain barrier (BBB) from normal brain tissues and delineating glioma margins during surgery. Here we present a highly sensitive, label-free terahertz reflectometry imaging (TRI) that overcomes current key limitations for intraoperative detection of World Health Organization (WHO) grade II (low grade), and grade III and IV (high grade) gliomas. We demonstrate that TRI provides tumor discrimination and delineation of tumor margins in brain tissues with high sensitivity on the basis of Hematoxylin and eosin (H&E) stained image. TRI may help neurosurgeons to remove gliomas completely by providing visualization of tumor margins in WHO grade II, III, and IV gliomas without contrast agents, and hence, improve patient outcomes. PMID:27782153

  13. Re-irradiation alternatives for recurrent high-grade glioma

    PubMed Central

    Dong, Yuanli; Fu, Chengrui; Guan, Hui; Zhang, Tianyi; Zhang, Zicheng; Zhou, Tao; Li, Baosheng

    2016-01-01

    Despite advances in the fields of surgery, chemotherapy and radiotherapy, the prognosis for high-grade glioma (HGG) remains unsatisfactory. The majority of HGG patients experience disease recurrence. To date, no standard treatments have been established for recurrent HGG. Repeat surgery and chemotherapy demonstrate moderate efficacy. As recurrent lesions are usually located within the previously irradiated field, a second course of irradiation was once considered controversial, as it was considered to exhibit unsatisfactory efficacy and radiation-related toxicities. However, an increasing number of studies have indicated that re-irradiation may present an efficacious treatment for recurrent HGG. Re-irradiation may be delivered via conventionally fractionated stereotactic radiotherapy, hypofractionated stereotactic radiation therapy, stereotactic radiosurgery and brachytherapy techniques. In the present review, the current literature regarding re-irradiation treatment for recurrent HGG is summarized with regard to survival outcome and side effects. PMID:27703519

  14. Molecular characteristics of pediatric high-grade gliomas

    PubMed Central

    Chamdine, Omar; Gajjar, Amar

    2014-01-01

    SUMMARY High-grade gliomas (HGGs) are extremely lethal tumors. Survival has not changed significantly in the past decades. The only known prognostic factors in pediatric HGGs (pHGGs) are extent of resection and histologic grade. Treatment has historically been based on adult trials because of the rarity of pHGGs and the lack of genomic tools to explore their unique molecular characteristics. The recent advances in molecular biological data helped divide these tumors into distinct subgroups. In this review, the authors focus on major molecular targets of alterations in pHGGs: histone H3.3, telomeres, PDGFRA, IDH, BRAFV600E, ACVR1 and NTRK and briefly highlight the difference with the adult counterpart. PMID:25438814

  15. Irradiation and Bevacizumab in High-Grade Glioma Retreatment Settings

    SciTech Connect

    Niyazi, Maximilian; Ganswindt, Ute; Schwarz, Silke Birgit; Kreth, Friedrich-Wilhelm; Tonn, Joerg-Christian; Geisler, Julia; Fougere, Christian la; Ertl, Lorenz; Linn, Jennifer; Siefert, Axel; Belka, Claus

    2012-01-01

    Purpose: Reirradiation is a treatment option for recurrent high-grade glioma with proven but limited effectiveness. Therapies directed against vascular endothelial growth factor have been shown to exert certain efficacy in combination with chemotherapy and have been safely tested in combination with radiotherapy in a small cohort of patients. To study the feasibility of reirradiation combined with bevacizumab treatment, the toxicity and treatment outcomes of this approach were analyzed retrospectively. Patients and Methods: After previous treatment with standard radiotherapy (with or without temozolomide) patients with recurrent malignant glioma received bevacizumab (10 mg/kg intravenous) on Day 1 and Day 15 during radiotherapy. Maintenance therapy was selected based on individual considerations, and mainly bevacizumab-containing regimens were chosen. Patients received 36 Gy in 18 fractions. Results: The data of the medical charts of the 30 patients were analyzed retrospectively. All were irradiated in a single institution and received either bevacizumab (n = 20), no additional substance (n = 7), or temozolomide (n = 3). Reirradiation was tolerated well, regardless of the added drug. In 1 patient treated with bevacizumab, a wound dehiscence occurred. Overall survival was significantly better in patients receiving bevacizumab (p = 0.03, log-rank test). In a multivariate proportional hazards Cox model, bevacizumab, Karnovsky performance status, and World Health Organization grade at relapse turned out to be the most important predictors for overall survival. Conclusion: Reirradiation with bevacizumab is a feasible and effective treatment for patients with recurrent high-grade gliomas. A randomized trial is warranted to finally answer the question whether bevacizumab adds substantial benefit to a radiotherapeutic retreatment setting.

  16. Neoadjuvant chemotherapy for high-grade advanced gastric cancer.

    PubMed

    Yonemura, Y; Sawa, T; Kinoshita, K; Matsuki, N; Fushida, S; Tanaka, S; Ohoyama, S; Takashima, T; Kimura, H; Kamata, T

    1993-01-01

    Fifty-five patients with high-grade advanced gastric cancer in whom the presence of stage IV was confirmed by preoperative diagnostic imaging were treated with PMUE therapy by a combined use of cisplatin (CDDP) 75 mg/m2, mitomycin C (MMC) 10 mg/body, etoposide 150 mg/body, and UFT (a combination of 1-(2-tetrahydrofuryl)-5-fluorouracil and uracil in a molar ratio of 1:4) 400 mg/day. CDDP and MMC was administered intravenously on the first day, followed by etoposide 50 mg/day on the 3rd, 4th, and 5th days. All the patients had measurable lesions that were evaluated by computed tomography scanning before and after the treatments. These patients were allocated randomly to two groups. Of these cases, 29 belonged to the neoadjuvant chemotherapy (NAC) group to whom PMUE therapy was given preoperatively; the remaining 26 patients underwent operation first and received PMUE thereafter (control group). Background factors did not differ significantly between the two groups. The response rate was higher in the NAC group than in the control group (62% in the former versus 35% in the latter). The resectability rates were 79% and 88% in the NAC and control groups, respectively. However, the rate of potentially curable cases was higher in the NAC group than in the control group (38% in the former versus 15% in the latter). Among the nonresection cases, the prognosis was highly unfavorable in both groups. In the resection cases, however, the survival rate was significantly better in the NAC group than in the control group. These results may indicate that in patients with high-grade, advanced gastric cancer initial chemotherapy (neoadjuvant chemotherapy) and then surgery should be considered. PMID:8511923

  17. [Hematogenous metastases of benign pleomorphic adenomas of the salivary glands (author's transl)].

    PubMed

    Heckmayr, M; Seifert, G

    1977-01-01

    Hematogenous metastases of benign pleomorphic adenomas are exceptionally rare. In three secure reports of the world literature metastases occurred in lung, liver or bone after operation of a benign pleomorphic adenoma with the greatest interval of twenty-two years between operation and metastases. This will be interpreted as implantation-metastases. All other observations represented secondary carcinomas in pre-existing pleomorphic adenomas. The morphological criterias of an accurate classification are explained. A number of reports of the literature about metastases of benign pleomorphic adenomas deals with misinterpretations of secondary adenomas in pre-existing pleomorphic adenomas.

  18. [Current Trends in Radiotherapy Following Surgical Resection of Soft-tissue Sarcoma of the Extremities and Trunk].

    PubMed

    Kraus-Tiefenbacher, U S; Van Kampen, M

    2015-04-01

    Besides surgery, radiotherapy plays its well-established part in the multimodality treatment of soft-tissue sarcomas. It can be delivered before or after surgery with similar control rates. Adjuvant radiotherapy increases the local control rates as well as the overall survival in intermediate or high-grade soft-tissue sarcomas. Due to the complex and sophisticated nature of the treatment, patients should be referred to specialised centres where modern radiotherapeutic options like intensity modulated radiotherapy and image-guided radiotherapy can be offered.

  19. Renal metastases from osteogenic sarcoma

    SciTech Connect

    Ayres, R.; Curry, N.S.; Gordon, L.; Bradford, B.F.

    1985-01-01

    A clinically and radiographically unsuspected ossified renal metastasis from a primary osteogenic sarcoma was identified by computed tomography (CT) and radionuclide bone scan. These imaging modalities play an important adjunctive role in the evaluation and follow-up of patients with primary osteogenic sarcoma.

  20. Pleomorphic xanthoastrocytoma in a case of tuberous sclerosis

    PubMed Central

    Martin, Arvind G.; Singh, Mutum Samarendra; Idris, Badrisyah; Abdullah, Jafri Malin

    2014-01-01

    Tuberous sclerosis is a known phakomatosis and the associated finding of a subependymal giant cell astrocytoma is common with this disorder. A case of tuberous sclerosis with a finding not previously reported, i.e. that of a pleomorphic xanthoastrocytoma, is presented here. PMID:25002765

  1. Morphological and biochemical features of Borrelia burgdorferi pleomorphic forms

    PubMed Central

    Herranen, Anni; Schwarzbach, Armin; Gilbert, Leona

    2015-01-01

    The spirochaete bacterium Borrelia burgdorferi sensu lato is the causative agent of Lyme disease, the most common tick-borne infection in the northern hemisphere. There is a long-standing debate regarding the role of pleomorphic forms in Lyme disease pathogenesis, while very little is known about the characteristics of these morphological variants. Here, we present a comprehensive analysis of B. burgdorferi pleomorphic formation in different culturing conditions at physiological temperature. Interestingly, human serum induced the bacterium to change its morphology to round bodies (RBs). In addition, biofilm-like colonies in suspension were found to be part of B. burgdorferi’s normal in vitro growth. Further studies provided evidence that spherical RBs had an intact and flexible cell envelope, demonstrating that they are not cell wall deficient, or degenerative as previously implied. However, the RBs displayed lower metabolic activity compared with spirochaetes. Furthermore, our results indicated that the different pleomorphic variants were distinguishable by having unique biochemical signatures. Consequently, pleomorphic B. burgdorferi should be taken into consideration as being clinically relevant and influence the development of novel diagnostics and treatment protocols. PMID:25564498

  2. Pleomorphic Carcinoma of the Colon: Morphological and Immunohistochemical Findings

    PubMed Central

    Branca, Giovanni; Barresi, Valeria; Ieni, Antonio; Irato, Eleonora; Caruso, Rosario Alberto

    2016-01-01

    Pleomorphic carcinoma is an aggressive neoplasm defined by the World Health Organization (WHO) as a poorly differentiated (squamous cell carcinoma or adenocarcinoma) or undifferentiated carcinoma in which at least 10% spindle and/or giant cells are identified, or as a carcinoma constituted purely of spindle and giant cells. Although this entity has initially been shown in the lung, it has been described also in extrapulmonary locations, with only one report for a colonic site. A 65-year-old woman developed a caecal tumour. Gross examination revealed an endophytic/ulcerative mass 7 cm in length. Microscopically, the tumour was a poorly differentiated adenocarcinoma with a pleomorphic component that occupied more than 10% of the specimen. The tumour shared these histopathological findings with pulmonary giant cell carcinoma but differed in other clinicopathological features such as a pushing growth pattern, stage pT3N1, and an uneventful outcome 24 months after operation. The pleomorphic component showed morphological and immunohistochemical features compatible with mitotic catastrophe, a non-apoptotic cell death occurring in cycling cells after aberrant mitosis. These features included multinucleation, micronucleation, atypical mitoses, foci of geographic necrosis, as well as immunohistochemical overexpression of p53 and Ki-67. The interpretation of the pleomorphic component as morphological expression of mitotic catastrophe may be useful in comprehending the pathogenesis of this rare neoplasm, and it may have practical implications as a potential cancer therapeutic target. PMID:27462191

  3. Sebaceous carcinoma ex-pleomorphic adenoma: a rare phenotypic occurrence.

    PubMed

    Cohn, Michael L; Callender, David L; El-Naggar, Adel K

    2004-08-01

    Primary sebaceous carcinoma of salivary glands is a rare entity with approximately 22 de novo documented cases. Similar tumor arising in a benign mixed tumor has only been reported once. We report a second case of sebaceous carcinoma in a pleomorphic adenoma and discuss the clinicopathologic features, histogenesis, and the differential diagnosis of this unusual tumor.

  4. Poorly differentiated medullary carcinoma of the colon with an unusual phenotypic profile mimicking high grade large cell lymphoma – a unique case report and review of the literature

    PubMed Central

    Nguyen, Johnny; Coppola, Domenico; Shan, Yuan; Zhang, Ling

    2014-01-01

    Medullary carcinoma (MC) of the colon and rectum is a rare entity, accounting for less than 0.1% of colonic adenocarcinoma that poses a diagnostic challenge for the practicing pathologist. Poorly differentiated or undifferentiated MC with an unusual histological appearance and immunoprofile in addition to heavy lymphoid infiltrate could make it problematic when differentiating it from a high grade lymphoma, in particular anaplastic large B- or T-cell lymphoma, plasmablastic lymphoma, and other undifferentiated neoplasms. Here we reported a unique case of an 81 y/o woman presenting with a 7.0 cm colon mass detected by computed tomography (CT) scan. A partial transverse and ileum resection with appendectomy were performed. Microscopic examination revealed sheets of large, pleomorphic, mitotically-active cells with abundant eosinophilic cytoplasm and multiple prominent nucleoli, growing with a pushing border and poor glandular formation in a background of intratumoral lymphocytes. The neoplastic cells were only focally positive for keratins (<10%); diffusely and strongly positive for vimentin and CD10 with high proliferative index (Ki-67, 90%). The tumor cells were also aberrantly positive for CD30, CD79a and CD43 (diffusely or focally), resulting in a diagnostic dilemma between colonic MC and high grade lymphoma. Careful examination and additional immunohistochemical stains performed proved there was no evidence of T or B-cell lymphoma, melanoma, or other types of primary colon or metastatic carcinomas. This case highlights the difficulty in distinguishing a high grade lymphoma and poorly differentiated colonic MC, and, also the aberrant expression of CD10 and a significant loss of pancytokeratin could result in a diagnostic pitfall. PMID:24551312

  5. An intracapsular carcinoma ex pleomorphic adenoma with lung metastases composed exclusively of benign elements: histological evidence of a continuum between metastasizing pleomorphic adenoma and carcinoma ex pleomorphic adenoma.

    PubMed

    Weissferdt, Annikka; Langman, Gerald

    2010-07-15

    Malignant mixed tumors of the salivary glands, encompassing carcinoma ex pleomorphic adenoma (ca ex PA), carcinosarcoma and metastasizing pleomorphic adenoma (mPA), are rare neoplasms. Ca ex PA arises in a pre-existing pleomorphic adenoma (PA). When the malignant component does not breach the capsule of the parent PA, the lesion is termed intracapsular ca ex PA, a neoplasm which is thought to have no metastatic potential. Metastatic deposits of ca ex PA are composed exclusively of malignant elements or mixed benign and malignant components. We describe the case of a 62-year-old female with an intracapsular ca ex PA of the buccal mucosa with subsequent metastases to the lung. The metastatic deposits resembled benign PA with no histological evidence of malignancy. This pattern of spread is described with mPA, an entity that caused controversy in the past regarding its exact classification as a benign or malignant tumor. The possibility that ca ex PA originates from a mPA, with intracapsular ca ex PA representing an intermediate lesion in a histological continuum, is discussed.

  6. Proliferative activity and aneuploidy in pleomorphic adenomas of the salivary glands.

    PubMed

    Martin, A R; Mantravadi, J; Kotylo, P K; Mullins, R; Walker, S; Roth, L M

    1994-03-01

    We used flow cytometry in a retrospective study of pleomorphic adenoma and carcinoma arising in pleomorphic adenoma, using paraffin-embedded tissue, to assess the relationship among proliferative activity, ploidy, and recurrence or malignant transformation. Twenty-four specimens obtained from 22 tumors were acceptable for analysis (co-efficient of variation, < or = 7.0), including multiple samples from two tumors. Fourteen tumors (13 benign and one malignant) were diploid. Six tumors were aneuploid: four benign pleomorphic adenomas and two carcinomas arising in pleomorphic adenoma. Two tetraploid tumors were malignant recurrences from the same patient. Of the recurrent tumors (nine benign and four malignant), 54% were aneuploid. The highest S-phase fractions were observed in recurrent and malignant pleomorphic adenomas. Immunostaining with p105, a nuclear proliferation antigen, revealed increased proliferative activity in a majority of pleomorphic adenomas. Increased proliferative activity and aneuploidy occurred in benign pleomorphic adenomas.

  7. Sorafenib in Treating Patients With Soft Tissue Sarcomas (Extremity Sarcoma Closed to Entry as of 5/30/07)

    ClinicalTrials.gov

    2014-04-01

    Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Metastatic Osteosarcoma; Recurrent Adult Soft Tissue Sarcoma; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Osteosarcoma; Stage I Adult Soft Tissue Sarcoma; Stage II Adult Soft Tissue Sarcoma; Stage III Adult Soft Tissue Sarcoma; Stage IV Adult Soft Tissue Sarcoma

  8. Functional and psychosocial effects of multimodality limb-sparing therapy in patients with soft tissue sarcomas

    SciTech Connect

    Chang, A.E.; Steinberg, S.M.; Culnane, M.; Lampert, M.H.; Reggia, A.J.; Simpson, C.G.; Hicks, J.E.; White, D.E.; Yang, J.J.; Glatstein, E. )

    1989-09-01

    We have documented functional and psychosocial changes in patients with extremity soft tissue sarcomas who have undergone multimodality limb-sparing treatments. In 88 patients, parameters related to economic status, sexual activity, pain, limb function, and global quality of life (QOL) were recorded prior to surgery and every 6 months postoperatively. Changes from the preoperative assessment for every parameter were analyzed in each patient. Six months after surgery, there was a decrease in employment status, sexual activity, and in limb function in a significant number of patients. At 12 months, these decreases were still evident. Despite these changes, global QOL measured by a standardized test showed at least some improvement in a significant proportion of patients at 12 months. These findings highlight the difficulty in defining QOL. It could not be ascertained if radiation therapy and/or chemotherapy were causative factors in specific changes because of the small numbers of patients in each subgroup. However, among 60 patients with high-grade sarcomas, significant wound problems developed in 10 of 33 who received postoperative radiation therapy in combination with adjuvant doxorubicin and cyclophosphamide chemotherapy compared with one of 27 patients who received adjuvant chemotherapy alone (P = .016). Also, among high-grade sarcoma patients with 12-month follow-up, six of 19 patients who received radiation therapy and chemotherapy developed joint contractures compared with zero of 15 patients who received chemotherapy alone (P less than .04). The combination of postoperative radiation therapy and chemotherapy appeared to be associated with significantly more tissue-related injury in patients with high-grade sarcomas compared with chemotherapy alone.

  9. Diagnostic Study of Tumor Characteristics in Patients With Ewing's Sarcoma

    ClinicalTrials.gov

    2013-06-20

    Localized Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor

  10. Collecting and Storing Biological Samples From Patients With Ewing Sarcoma

    ClinicalTrials.gov

    2016-05-16

    Askin Tumor; Localized Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor

  11. The clinical outcome of pazopanib treatment in Japanese patients with relapsed soft tissue sarcoma: A Japanese Musculoskeletal Oncology Group (JMOG) study

    PubMed Central

    Nakamura, Tomoki; Kawai, Akira; Araki, Nobuhito; Goto, Takahiro; Yonemoto, Tsukasa; Sugiura, Hideshi; Nishida, Yoshihiro; Hiraga, Hiroaki; Honoki, Kanya; Yasuda, Taketoshi; Boku, Shogen; Sudo, Akihiro; Ueda, Takafumi

    2016-01-01

    BACKGROUND Because the efficacy and safety of pazopanib in Japanese patients with soft tissue sarcoma (STS) had not been evaluated previously in a large‐scale cohort, the authors investigated the efficacy and safety of pazopanib in 156 Japanese patients with relapsed STS. This was a retrospective study based on the collection of real‐life, postmarketing surveillance data. METHODS Patients received pazopanib with the objective of treating local recurrence (n = 20), metastasis (n = 104), and both (n = 32). The patient median age was 53.8 years. The primary objective of this study was to clarify the efficacy of pazopanib for patients with STS. RESULTS The median treatment duration was 28.7 weeks, and the average dose intensity of pazopanib was 609 mg. Adverse events occurred in 127 patients (81.4%). In addition to the main common toxicities, such as hypertension and liver disorder, pneumothorax (n = 11) and thrombocytopenia (n = 16) also were observed. The median progression‐free survival for all patients was 15.4 weeks. The median progression‐free survival for patients with leiomyosarcoma, synovial sarcoma, undifferentiated pleomorphic sarcoma, and liposarcoma was 18.6 weeks, 16.4 weeks, 15.3 weeks, and 8 weeks, respectively. The median survival for all patients was 11.2 months. The median survival for patients with leiomyosarcoma, synovial sarcoma, undifferentiated pleomorphic sarcoma, and liposarcoma was 20.1 months, 10.6 months, 9.5 months, and 7.3 months, respectively. CONCLUSIONS There were apparent differences in the efficacy of pazopanib treatment among histologic types of STS. Pazopanib treatment is a new treatment option; however, adverse events like pneumothorax and thrombocytopenia, which did not occur frequently in the PALETTE study (pazopanib for metastatic soft‐tissue sarcoma), should be taken into consideration. Cancer 2016;122:1408‐16. © 2016 The Authors. Cancer published by Wiley Periodicals, Inc. on behalf of American Cancer Society

  12. Barrett's esophagus: management of high-grade dysplasia and cancer.

    PubMed

    Ruol, Alberto; Zaninotto, Giovanni; Costantini, Mario; Battaglia, Giorgio; Cagol, Matteo; Alfieri, Rita; Epifani, Magdalena; Ancona, Ermanno

    2004-03-01

    Esophagectomy remains the treatment of choice for the appropriate patient with Barrett's adenocarcinoma invading beyond the mucosa, without evidence of distant metastasis or invasion of adjacent organs. On the other hand, therapeutic management of patients with Barrett's high-grade dysplasia (HGD) or mucosal adenocarcinoma should be individualized, taking into account the patient's preferences, willingness to return for frequent endoscopic biopsies, and medical fitness to undergo esophagectomy. Surgery has to be considered the best treatment for HGD or superficial carcinoma, unless contraindicated by severe comorbidities, because it has proven to be the only treatment that is successful in curing the condition and preventing recurrent HGD or the development of invasive cancer. Nonsurgical treatment by photodynamic therapy or endoscopic mucosal resection may be a less invasive and organ-sparing option for elderly, poor-risk patients but it is still to be considered an investigational therapy that should only be conducted under a clinical trial protocol. Finally, intensive endoscopic biopsy surveillance of patients with HGD is another investigational option that may allow prompt treatment of cancer if it develops. However, few data document the safety of this observational approach. PMID:15013713

  13. Concurrent thermochemoradiotherapy for brain high-grade glioma

    NASA Astrophysics Data System (ADS)

    Ryabova, A. I.; Novikov, V. A.; Choinzonov, E. L.; Gribova, O. V.; Startseva, Zh. A.; Bober, E. E.; Frolova, I. G.; Baranova, A. V.

    2016-08-01

    Despite the achievements in the current strategies for treatment, the prognosis in malignant glioma patients remains unsatisfactory. Hyperthermia is currently considered to be the most effective and universal modifier of radiotherapy and chemotherapy. Preliminary treatment outcomes for 28 patients with newly diagnosed (23) and recurrent (5) high-grade gliomas were presented. All the patients received multimodality treatment including surgery, thermoche-moradiotherapy followed by 4 cycles of adjuvant chemotherapy. All the patients endured thermochemoradiotherapy well. A complication, limited skin burn (II stage), was diagnosed in two cases and treated conservatively without treatment interruption. A month after thermochemoradiotherapy the results were as follows: complete regression was achieved in 4 cases, partial regression in 4 cases, stable disease in 14 cases and disease progression in 6 cases (one of them is pseudo-progression). After completing the adjuvant chemotherapy 2 more patients demonstrated complete response and 1 patient had disease progression. Introduction of local hyperthermia in multimodal therapy of malignant glioma does not impair the combined modality treatment tolerability of patients with malignant gliomas. A small number of studied patients and short follow-up time do not allow making reliable conclusions about the impact of local hyperthermia on the treatment outcomes; however, there is a tendency towards the increase in disease-free survival in the patients with newly diagnosed malignant gliomas.

  14. Thromboembolic disease in patients with high-grade glioma.

    PubMed

    Perry, James R

    2012-09-01

    Venous thromboembolism (VTE) is common throughout the course of disease in high-grade glioma (HGG). The interactions between the coagulation cascade, endothelium, and regulation of angiogenesis are complex and drive glioblastoma growth and invasion. We reviewed the incidence of VTE in HGG, the biology of the coagulome as related to glioblastoma progression, prevention and treatment of thrombosis, and the putative role of anticoagulants as anti-cancer therapy. VTE can be significantly reduced during the postoperative period with adherence to the use of mechanical and medical thromboprophylaxis. Activation of the coagulation cascade occurs throughout the course of disease because of a variety of complex interactions, including tumor hypoxia, upregulation of VEGR expression, and increases in both tumor cell-specific tissue factor (TF) expression and inducible TF expression in numerous intrinsic regulatory pathways. Long-term anticoagulation to prevent VTE is an attractive therapy; however, the therapeutic window is narrow and current data do not support its routine use. Most patients with proven symptomatic VTE can be safely anticoagulated, including those receiving anti-VEGF therapy, such as bevacizumab. Initial therapy should include low molecular weight heparin (LMWH), and protracted anticoagulant treatment, perhaps indefinitely, is indicated for patients with HGG because of the ongoing risk of thrombosis. A variety of coagulation- and tumor-related proteins, such as TF and circulating microparticles, may serve as potential disease-specific biomarkers in relation to disease recurrence, monitoring of therapy, and as potential therapeutic targets. PMID:23095833

  15. Adoptive cell therapy for sarcoma

    PubMed Central

    Mata, Melinda; Gottschalk, Stephen

    2015-01-01

    Current therapy for sarcomas, though effective in treating local disease, is often ineffective for patients with recurrent or metastatic disease. To improve outcomes, novel approaches are needed and cell therapy has the potential to meet this need since it does not rely on the cytotoxic mechanisms of conventional therapies. The recent successes of T-cell therapies for hematological malignancies have led to renewed interest in exploring cell therapies for solid tumors such as sarcomas. In this review, we will discuss current cell therapies for sarcoma with special emphasis on genetic approaches to improve the effector function of adoptively transferred cells. PMID:25572477

  16. Levetiracetam improves verbal memory in high-grade glioma patients

    PubMed Central

    de Groot, Marjolein; Douw, Linda; Sizoo, Eefje M.; Bosma, Ingeborg; Froklage, Femke E.; Heimans, Jan J.; Postma, Tjeerd J.; Klein, Martin; Reijneveld, Jaap C.

    2013-01-01

    Background Treatment of high-grade glioma (HGG) patients with anti-epileptic drugs (AEDs) has met with various side effects, such as cognitive deterioration. The cognitive effects of both older and newer AEDs in HGG patients are largely unknown. The aim of this study was to determine the effect of older and newer AEDs on cognitive performance in postoperative HGG patients. Methods We selected HGG patients from 3 separate cohorts for use of older, newer, or no AEDs, as they represented distinct treatment eras and provided the opportunity to compare older and newer AEDs. In all 3 cohorts, patients were included within 6 weeks following neurosurgery before the start of postoperative treatment. Cognitive functioning was evaluated by an extensive neuropsychological assessment, executed in 6 cognitive domains (attention, executive functioning, verbal memory, working memory, psychomotor functioning, and information processing speed). Results One hundred seventeen patients met the inclusion criteria; 44 patients used no AED, 35 were on monotherapy with a newer AED (all levetiracetam), and 38 were on monotherapy with an older AED (valproic acid or phenytoin). Patients on older and newer AEDs performed equally well as patients not on an AED, and patients on levetiracetam performed even better on verbal memory tests than patients not on an AED. Post-hoc analyses revealed that within the group using older AEDs, patients on valproic acid performed better than patients on phenytoin. Conclusions Neither levetiracetam nor valproic acid was associated with additional cognitive deficits in HGG patients. Both AEDs even appeared to have a beneficial effect on verbal memory in these patients. PMID:23233537

  17. Collapse in High-Grade Stenosis during Pulsatile Flow Experiments

    NASA Astrophysics Data System (ADS)

    Kobayashi, Shunichi; Tang, Dalin; Ku, David N.

    It has been hypothesized that blood flow through high grade stenotic arteries may produce conditions in which elastic flow choking may occur. The development of atherosclerotic plaque fracture may be exacerbated by the compressive stresses during collapse. This study explored the effects of pulsatile flow on stenotic flow collapse. Pulsatile flow was produced using a gear pump controlled by a digitized physiologic waveform. Upstream and downstream mean pressures and pulsatile flow rates were measured and digitized. An improved model of arterial stenosis was created using an elastomer with an incremental modulus of elasticity matched to a bovine carotid artery in the relevant range of collapse. Additionally, the model retained a very thick wall in the stenotic region similar to arterial disease. Flow choking was observed for pulsatile pressure drops close to those previously reported for steady flow. The phase difference between flow rate and pressure between upstream and downstream of the stenosis occurred by the compliance of tube and stenosis resistance. For 80% nominal stenosis by diameter and 100+/-30mmHg upstream pressure, collapse occurred for average pulsatile pressure drops of 93mmHg. Pulsatile flow experiments in this model revealed the range of conditions for the flow choking and the paradoxical collapse of the stenosis during systole with expansion during diastole. The stenosis severity was dynamic through the pulse cycle and was significantly greater under flow than the nominal severity. The results indicate that flow choking and stenotic compression may be significant in thick-walled arterial stenoses subjected to pulsatile flow.

  18. Novel oncogenic PDGFRA mutations in pediatric high-grade gliomas.

    PubMed

    Paugh, Barbara S; Zhu, Xiaoyan; Qu, Chunxu; Endersby, Raelene; Diaz, Alexander K; Zhang, Junyuan; Bax, Dorine A; Carvalho, Diana; Reis, Rui M; Onar-Thomas, Arzu; Broniscer, Alberto; Wetmore, Cynthia; Zhang, Jinghui; Jones, Chris; Ellison, David W; Baker, Suzanne J

    2013-10-15

    The outcome for children with high-grade gliomas (HGG) remains dismal, with a 2-year survival rate of only 10% to 30%. Diffuse intrinsic pontine glioma (DIPG) comprise a subset of HGG that arise in the brainstem almost exclusively in children. Genome-wide analyses of copy number imbalances previously showed that platelet-derived growth factor receptor α (PDGFRA) is the most frequent target of focal amplification in pediatric HGGs, including DIPGs. To determine whether PDGFRA is also targeted by more subtle mutations missed by copy number analysis, we sequenced all PDGFRA coding exons from a cohort of pediatric HGGs. Somatic-activating mutations were identified in 14.4% (13 of 90) of nonbrainstem pediatric HGGs and 4.7% (2 of 43) of DIPGs, including missense mutations and in-frame deletions and insertions not previously described. Forty percent of tumors with mutation showed concurrent amplification, whereas 60% carried heterozygous mutations. Six different mutations impacting different domains all resulted in ligand-independent receptor activation that was blocked by small molecule inhibitors of PDGFR. Expression of mutants in p53-null primary mouse astrocytes conferred a proliferative advantage in vitro and generated HGGs in vivo with complete penetrance when implanted into brain. The gene expression signatures of these murine HGGs reflected the spectrum of human diffuse HGGs. PDGFRA intragenic deletion of exons 8 and 9 were previously shown in adult HGG, but were not detected in 83 nonbrainstem pediatric HGG and 57 DIPGs. Thus, a distinct spectrum of mutations confers constitutive receptor activation and oncogenic activity to PDGFRα in childhood HGG. PMID:23970477

  19. Genomic characterization of recurrent high-grade astroblastoma.

    PubMed

    Bale, Tejus A; Abedalthagafi, Malak; Bi, Wenya Linda; Kang, Yun Jee; Merrill, Parker; Dunn, Ian F; Dubuc, Adrian; Charbonneau, Sarah K; Brown, Loreal; Ligon, Azra H; Ramkissoon, Shakti H; Ligon, Keith L

    2016-01-01

    Astroblastomas are rare primary brain tumors, diagnosed based on histologic features. Not currently assigned a WHO grade, they typically display indolent behavior, with occasional variants taking a more aggressive course. We characterized the immunohistochemical characteristics, copy number (high-resolution array comparative genomic hybridization, OncoCopy) and mutational profile (targeted next-generation exome sequencing, OncoPanel) of a cohort of seven biopsies from four patients to identify recurrent genomic events that may help distinguish astroblastomas from other more common high-grade gliomas. We found that tumor histology was variable across patients and between primary and recurrent tumor samples. No common molecular features were identified among the four tumors. Mutations commonly observed in astrocytic tumors (IDH1/2, TP53, ATRX, and PTEN) or ependymoma were not identified. However one case with rapid clinical progression displayed mutations more commonly associated with GBM (NF1(N1054H/K63)*, PIK3CA(R38H) and ERG(A403T)). Conversely, another case, originally classified as glioblastoma with nine-year survival before recurrence, lacked a GBM mutational profile. Other mutations frequently seen in lower grade gliomas (BCOR, BCORL1, ERBB3, MYB, ATM) were also present in several tumors. Copy number changes were variable across tumors. Our findings indicate that astroblastomas have variable growth patterns and morphologic features, posing significant challenges to accurate classification in the absence of diagnostically specific copy number alterations and molecular features. Their histopathologic overlap with glioblastoma will likely confound the observation of long-term GBM "survivors". Further genomic profiling is needed to determine whether these tumors represent a distinct entity and to guide management strategies. PMID:27425854

  20. Benign pleomorphic adenoma of the larynx. A case report.

    PubMed

    Som, P M; Nagel, B D; Feuerstein, S S; Strauss, L

    1979-01-01

    Minor salivary gland tumors of the larynx are relatively rare. Morphologically the majority of them are adenoid cystic carcinomas and the overwhelming location is the subglottic larynx. Benign pleomorphic adenomas are rare minor salivary gland tumors of the larynx which usually occur at the glottic and subglottic levels. We present only the sixth case of a supraglottic benign pleomorphic adenoma that we could find in the literature. A preoperative laryngogram localized the tumor to the supraglottic larynx and the intact mucosa suggested a specific differential diagnosis. The tumor was removed by a lateral pharyngotomy with retention of good laryngeal function. The relatively asymptomatic presentation of our case is a somewhat unusual finding which, however, was previously noted in similar case reports.

  1. Pleomorphic adenoma with extensive lipometaplasia: report of three cases.

    PubMed

    Haskell, Henry D; Butt, Khalid M; Woo, Sook-Bin

    2005-10-01

    We report a series of three cases of pleomorphic adenoma with extensive lipometaplasia, a recently described subtype of pleomorphic adenoma of salivary gland origin. Two patients were female and one male, ranging in age from 30 to 45 years. Two occurred in the minor salivary glands of the lip and palate, respectively, and one in the parotid. Typical histologic findings are presented. In addition, one case consists of a proliferation of spindle cells with an interesting combination of mature adipose tissue, hyaline cartilage, and bone in the absence of ductal structures. The differential diagnosis, as it pertains to other fat-containing tumors (such as lipoadenoma, spindle cell lipoma, interstitial lipomatosis, and benign mesenchymoma), is discussed. It is likely that the ability of myoepithelial cells to undergo various metaplasias is the cause of the unusual histologic appearances of this tumor.

  2. Drugs Approved for Kaposi Sarcoma

    Cancer.gov

    This page lists cancer drugs approved by the Food and Drug Administration (FDA) for Kaposi sarcoma. The list includes generic names and brand names. The drug names link to NCI's Cancer Drug Information summaries.

  3. Treatment Options for Kaposi Sarcoma

    MedlinePlus

    ... therapy are used to treat Kaposi sarcoma lesions . Photon radiation therapy treats lesions with high-energy light. ... complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information ...

  4. Treatment Option Overview (Kaposi Sarcoma)

    MedlinePlus

    ... therapy are used to treat Kaposi sarcoma lesions . Photon radiation therapy treats lesions with high-energy light. ... complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information ...

  5. Radiotherapy to Improve Local Control Regardless of Surgical Margin and Malignancy Grade in Extremity and Trunk Wall Soft Tissue Sarcoma: A Scandinavian Sarcoma Group Study

    SciTech Connect

    Jebsen, Nina L. Trovik, Clement S.; Bauer, Henrik C.F.; Rydholm, Anders; Monge, Odd R.; Hall, Kirsten Sundby; Alvegard, Thor; Bruland, Oyvind S.

    2008-07-15

    Purpose: Adjuvant radiotherapy has during the past decades become increasingly used in the treatment of localized soft tissue sarcoma. We evaluated the effect of radiotherapy (RT) on local recurrence rates (LRRs) in Scandinavia between 1986 and 2005. Methods and Materials: A total of 1,093 adult patients with extremity or trunk wall soft tissue sarcoma treated at four Scandinavian sarcoma centers were stratified according to the treatment period (1986-1991, 1992-1997, and 1998-2005). The use of adjuvant RT, quality of the surgical margin, interval between surgery and RT, and LRR were analyzed. The median follow-up was 5 years. Results: The use of RT (77% treated postoperatively) increased from 28% to 53%, and the 5-year LRR decreased from 27% to 15%. The rate of wide surgical margins did not increase. The risk factors for local recurrence were histologic high-grade malignancy (hazard ratio [HR], 5), an intralesional (HR, 6) or marginal (HR, 3) surgical margin, and no RT (HR, 3). The effect of RT on the LRR was also significant after a wide margin resection and in low-grade malignant tumors. The LRR was the same after preoperative and postoperative RT. The median interval from surgery to the start of RT was 7 weeks, and 98% started RT within 4 months. The LRR was the same in patients who started treatment before and after 7 weeks. Conclusion: The results of our study have shown that adjuvant RT effectively prevents local recurrence in soft tissue sarcoma, irrespective of the tumor depth, malignancy grade, and surgical margin status. The effect was most pronounced in deep-seated, high-grade tumors, even when removed with a wide surgical margin.

  6. Pleomorphic adenoma of the lacrimal gland. Case report.

    PubMed

    Ostrosky, Alejandro; Klurfan, Federico Juan; Gonzalez, Maximiliano Jorge; Camaly, Diego; Villa, Diego

    2005-01-01

    Lacrimal gland tumors are rare. We present a case of a 69 year old male patient with a one year history of an orbital tumor (Pleomorphic Adenoma). The tumor was surgically enucleated and patient recovery was very satisfactory, with no signs of recurrence after one year of follow-up. A review of the clinical symptoms, radiologic features, treatment and histopathology of this kind of tumors is also made.

  7. Intraosseous pleomorphic adenoma: case report and review of the literature.

    PubMed

    Aver-De-Araujo, L M; Chaves-Tarquinio, S B; Neuzling-Gomes, A P; Etges, A

    2002-01-01

    Pleomorphic adenoma is the most common neoplasm of the salivary glands, affecting mainly the parotid gland. The preferential intraoral site of this tumor is the palate. A case of a 31-year-old woman with an intraosseous pleomorphic adenoma located in the maxilla (left paramedian region), showing an approximate evolution of one year is reported. The present intraosseous case represents a rare location, with the tumor probably originating from glandular epithelial remnants captured during embryogenesis. In a review of the literature of 142 cases of intragnathic localization (24% in the maxilla) are identified. A slight predominance of women was observed (56%), with 55% of the patients being affected during the 5th to 7th decade of life. The tumors were malignant in 94% of the cases, with special predominance of mucoepidermoid carcinoma (65%). Intraosseous pleomorphic adenomas are rare, with the present patient being the 6th case reported in the literature and the second found in the maxilla. Mean age of the 5 previously reported cases was 58.8 years.

  8. The Role for Radiation Therapy in the Management of Sarcoma.

    PubMed

    Leachman, Brooke K; Galloway, Thomas J

    2016-10-01

    Although there is no consensus regarding the optimal sequencing of external beam radiotherapy and surgery for extremity soft tissue sarcoma, radiation therapy delivered before or after limb-sparing surgery significantly improves local control, particularly for high-grade tumors. Large database analyses suggest that improved local control may translate into an overall survival benefit. Best practices require ample communication between the radiation and surgical teams to ensure appropriate tissues are targeted, unnecessary radiation is avoided, and patients are afforded the best opportunity for cure while maintaining function. Modern experiences with intensity-modulated radiotherapy/image-guided radiation therapy suggest toxicity is reduced through field size reduction and precise targeting, improving the therapeutic ratio. PMID:27542646

  9. Not such a simple cyst: unusual presentation of sarcoma

    PubMed Central

    Cooper, Helen

    2014-01-01

    A 10-year-old girl presented with a lump on her left upper anterior thigh. She presented to accident and emergency twice and to her general practitioner once. It was provisionally diagnosed as a cyst but it continued to grow in size. The lump had been noticed for 6 months, in paediatric clinic it was 5×5 cm, firm, tense and felt cystic. Systemically she was otherwise well. The overlying skin was blueish with prominent veins. The MRI also suggested a cyst but on excision it was revealed to be a high-grade sarcoma. After surgical excision she had a skin graft. Regular follow-ups with MRI have not shown any recurrence, she also has regular chest X-rays which have all been normal. PMID:25315800

  10. Langerhan’s Cell Sarcoma: Two Case Reports

    PubMed Central

    Kaleem, Tasneem A.; Schild, Michael H.; Miller, Daniel; Jha, Asit; Cortese, Cherise; Attia, Steven; Miller, Robert C.

    2016-01-01

    Langerhan’s cell sarcoma (LCS) is a rare neoplasm with a poor prognosis. To our knowledge, only sixty-six cases have been published. We discuss two patients who presented very differently with LCS, as well as a recently published review of all sixty-six cases. Our first case had a complicated history of metastatic, high-grade myxofibrosarcomas and presented with a single skin lesion of LCS which was treated with resection to a positive margin and adjuvant radiotherapy. The LCS recurred locoregionally and was again resected. The patient is alive two years after initial diagnosis. The second case presented with bone marrow and splenic involvement, leukocytosis, and thrombocytopenia. This patient had an excellent response to etoposide, prednisone, oncovorin, cyclophosphamide, and adriamycin, with normalization of the complete blood count, negative bone marrow biopsy at follow up, and splenectomy without viable neoplasm. This patient is alive without signs of disease at 16 months after initial diagnosis. PMID:27134713

  11. Intracapsular carcinoma ex pleomorphic adenoma. Report of a case with unusual metastatic behaviour.

    PubMed

    Felix, A; Rosa-Santos, J; Mendonça, M E; Torrinha, F; Soares, J

    2002-01-01

    Intracapsular carcinomas ex pleomorphic adenoma are exceedingly rare salivary glands tumours, known to have a benign clinical behaviour with metastatic unrecognised potential. We report a case of a 57-year-old female patient presenting with a typical example of intracapsular carcinoma ex pleomorphic adenoma of the parotid gland and evidence of ipsilateral cervical lymph node metastases two years before. The behaviour of this unique case illustrates the disputed malignancy of carcinomas arising within encapsulated pleomorphic adenomas.

  12. Pleomorphic adenoma of the vulva, clinical reminder of a rare occurrence.

    PubMed

    Su, Albert; Apple, Sophia K; Moatamed, Neda A

    2012-01-01

    Pleomorphic adenoma, also known as mixed tumor, is a benign tumor which typically presents as a painless and persistent mass. The majority of pleomorphic adenomas involve the salivary glands, most commonly the parotid gland. Other sites include breast and skin. It is a rare tumor in the vulva. In this article we are reporting a case of pleomorphic adenoma of labia with characteristic pathologic and clinical findings, as reminder of a common benign neoplasm occurring with rare locality.

  13. An immunohistochemical study of bizarre neoplastic cells in pleomorphic adenoma: its cytological nature and proliferative activity.

    PubMed

    Takeda, Y

    1999-11-01

    The cytological nature and proliferative activity of bizarre neoplastic cells, widely scattered in pleomorphic adenomas of salivary gland origin were studied. Pleomorphic adenomas containing numerous bizarre neoplastic cells were found in four cases, and were equal to 2.9% of all pleomorphic adenomas examined. All four cases presented as well-circumscribed, firm masses measuring less than 1.5 cm in size, located in the palate, and were of 7 months to 4 years duration. Histopathologically, these pleomorphic adenomas were cell rich type, and were well demarcated from surrounding tissues, although their fibrous capsules were partially defective. In addition to characteristic histopathological findings of pleomorphic adenoma, numerous neoplastic cells with bizarre appearance were scattered throughout the lesion, excepting for tubuloductal structures. These bizarre neoplastic cells had irregular-shaped and large nuclei with or without hyperchromatism, although their nucleoli were small and mitotic figures were few. Furthermore, there were many multinucleated giant cells, some of which showed multilobulated nuclei. Neither necrosis nor infarct was seen in the tumors. Immunohistochemically, bizarre neoplastic cells scattered in solid-proliferating areas and myxoid areas were neoplastic myoepithelial cells in nature. There was no statistical significance of MIB-1 labeling indices between pleomorphic adenomas with bizarre neoplastic cells and usual pleomorphic adenomas. The p53 labeling indices were quite low. Although the benign nature of pleomorphic adenomas with numerous bizarre neoplastic cells and hypercellularity, distinguishing such pleomorphic adenomas from various stages of malignant transformation in pleomorphic adenomas and other carcinomas should be made by histological section of submitted biopsy specimen or aspirated content for cytological diagnosis. The present paper suggests that the term 'bizarre cell pleomorphic adenoma' is an appropriate name for this

  14. [Pleomorphic adenoma of salivary glands: diagnostic pitfalls and mimickers of malignancy].

    PubMed

    Skálová, A; Andrle, P; Hostička, L; Michal, M

    2012-10-01

    Pleomorphic adenoma is the most common salivary gland tumor, characterized by a complex biphasic proliferation of epithelial and myoepithelial cells intermingled with a mezenchymal component with frequent metaplastic changes and protean histomorphology of the cells. This review describes several unusual histological findings in pleomorphic adenoma that may mimic malignancy, and therefore they represent a diagnostic pitfall. Intravascular invasion of tumor cells is generally suspicious of malignancy; however, intravascular tumor deposits may be rarely found within the capsule of clinically benign salivary pleomorphic adenomas. It is important not to render a malignant diagnosis in such neoplasms, in the absence of other evidence of malignancy. Pleomorphic adenomas, particularly of minor glands of palate, may contain large areas of squamous and mucinous metaplasia suspicious of mucoepidermoid carcinoma (MEC). In contrast to MEC, metaplastic pleomorphic adenomas do not harbour the distinctive translocations t(11;19) and t(11;15), they are not invasive, in contrast they reveal at least focally myxochondroid stroma. Cribriform structures in pleomorphic adenoma may mimic adenoid cystic carcinoma. Oncocytic metaplasia in cellular rich pleomorphic adenoma/myoepithelioma may be associated with significant nuclear polymorphism and hyperchromasia suspicious of malignancy. The most common pitfall in diagnosis of pleomorphic adenoma is so called "atypical PA" that must be distinguished from early malignant transformation to in situ-carcinoma ex pleomorphic adenoma.

  15. [Radiotherapy of adult soft tissue sarcoma].

    PubMed

    Le Péchoux, C; Moureau-Zabotto, L; Llacer, C; Ducassou, A; Sargos, P; Sunyach, M P; Thariat, J

    2016-09-01

    Incidence of soft tissue sarcoma is low and requires multidisciplinary treatment in specialized centers. The objective of this paper is to report the state of the art regarding indications and treatment techniques of main soft tissue sarcoma localisations.

  16. General Information about Adult Soft Tissue Sarcoma

    MedlinePlus

    ... Soft Tissue Sarcoma Treatment (PDQ®)–Patient Version General Information About Adult Soft Tissue Sarcoma Go to Health ... the PDQ Adult Treatment Editorial Board . Clinical Trial Information A clinical trial is a study to answer ...

  17. General Information about Childhood Soft Tissue Sarcoma

    MedlinePlus

    ... Soft Tissue Sarcoma Treatment (PDQ®)–Patient Version General Information About Childhood Soft Tissue Sarcoma Go to Health ... the PDQ Pediatric Treatment Editorial Board . Clinical Trial Information A clinical trial is a study to answer ...

  18. Childhood Soft Tissue Sarcoma: Treatment Information

    MedlinePlus

    ... Germ Cell Tumors Kidney/Wilms Tumor Liver Cancer Neuroblastoma Osteosarcoma Rhabdomyosarcoma Skin Cancer Soft Tissue Sarcoma Thyroid ... Tumor Liver Cancer Lymphoma (Non-Hodgkin) Lymphoma (Hodgkin) Neuroblastoma Osteosarcoma Retinoblastoma Rhabdomyosarcoma Skin Cancer Soft Tissue Sarcoma ...

  19. [Radiotherapy of adult soft tissue sarcoma].

    PubMed

    Le Péchoux, C; Moureau-Zabotto, L; Llacer, C; Ducassou, A; Sargos, P; Sunyach, M P; Thariat, J

    2016-09-01

    Incidence of soft tissue sarcoma is low and requires multidisciplinary treatment in specialized centers. The objective of this paper is to report the state of the art regarding indications and treatment techniques of main soft tissue sarcoma localisations. PMID:27523415

  20. Sorafenib in Treating Patients With Metastatic, Locally Advanced, or Recurrent Sarcoma

    ClinicalTrials.gov

    2014-05-07

    Adult Angiosarcoma; Adult Epithelioid Sarcoma; Adult Leiomyosarcoma; Adult Malignant Fibrous Histiocytoma; Adult Neurofibrosarcoma; Adult Synovial Sarcoma; Ovarian Sarcoma; Recurrent Adult Soft Tissue Sarcoma; Recurrent Uterine Sarcoma; Stage III Adult Soft Tissue Sarcoma; Stage III Uterine Sarcoma; Stage IV Adult Soft Tissue Sarcoma; Stage IV Uterine Sarcoma; Uterine Carcinosarcoma; Uterine Leiomyosarcoma

  1. Synovial Sarcoma With Myoid Differentiation.

    PubMed

    Qassid, Omar; Ali, Ahmed; Thway, Khin

    2016-09-01

    Synovial sarcoma is a malignant mesenchymal tumor with variable epithelial differentiation, which is defined by the presence of a specific t(X;18)(p11.2;q11.2) chromosomal translocation that generates SS18-SSX fusion oncogenes. Synovial sarcoma typically arises within extremity deep soft tissue (particularly around large joints) of young adults, but has been shown to occur at almost any location. When it arises in more unusual sites, such as the abdomen, it can present a significant diagnostic challenge. We describe a case of intraabdominal monophasic synovial sarcoma that immunohistochemically showed strong expression of smooth muscle actin and calponin but only very scanty cytokeratin, and which showed morphologic and immunohistochemical overlap with other spindle cell neoplasms that can arise at this site, such as gastrointestinal stromal tumor and myofibrosarcoma. As correct diagnosis is of clinical and prognostic importance, surgical pathologists should be aware of the potential for synovial sarcoma to occur at a variety of anatomic sites and of its spectrum of immunoreactivity. Synovial sarcoma should be in the differential diagnosis of spindle cell neoplasms with myoid differentiation that do not fall into any definite tumor category, for which there should be a relatively low threshold for performing fluorescence in situ hybridization or reverse transcription-polymerase chain reaction to assess for the specific SS18 gene rearrangement or SS18-SSX fusion transcripts, which remain the diagnostic gold standard. PMID:27106779

  2. [Unusual lung localization of histiocytic sarcoma].

    PubMed

    Aichaouia, C; Daboussi, S; Haddaoui, A; Moatemri, Z; Farah, S; Khadraoui, M; Bouzaiene, A; Cheikh, R

    2012-10-01

    Histiocytic sarcoma, proliferation araising from immunoregulatory effector system cells, is a very rare and recently recognised tumor. Diagnosis is based on immunohistochemistry and molecular genetic techniques, which allow to distinguish histiocytic sarcoma from lymphocytic proliferation, such as non-Hodgkin's. We report this rare case of multivisceral histiocytic sarcoma revealed by lung localization and for which the evolution was fatal.

  3. Granulocytic sarcoma masquerading as Ewing's sarcoma: a diagnostic dilemma.

    PubMed

    Haresh, Kunhi Parambath; Joshi, Nikhil; Gupta, Chaitali; Prabhakar, Ramachandran; Sharma, Daya Nand; Julka, Pramod Kumar; Rath, Goura Kishor

    2008-01-01

    An eleven-year-old boy presented with a swelling in his left elbow. Radiologically the features were that of an Ewing's sarcoma involving the ulna. Histopathology showed small round cell tumor strongly positive for Monoclonal Imperial Cancer research fund 2 (MIC2) antigen. Similar cells in the bone marrow were involved with MIC2 positivity. The patient developed skin lesions, which on biopsy were found to be chloromas. The initial biopsies were reevaluated with special stains revealing granulocytic sarcomas in acute myeloid leukemia masquerading as Ewing's due to its MIC2 positivity. The possibility of myeloid neoplasms should be considered routinely with known MIC2 positive round cell tumors. PMID:18923208

  4. Pleomorphic hyalinizing angiectatic tumor of soft parts: case report with unusual ganglion-like cells and review of the literature.

    PubMed

    Changchien, Yi-Che; Bocskai, Pál; Kovács, Ilona; Hargitai, Zoltán; Kollár, Sándor; Török, Miklós

    2014-12-01

    Pleomorphic hyalinizing angiectatic tumor (PHAT) is a recently described, non-metastasizing tumor of uncertain lineage. This tumor distributes equally between the genders and has a predilection for the subcutaneous soft tissue, particularly in lower extremity, other locations are rare. Based on the recent literature, PHAT is suspected to encompass the morphological spectrum with other tumors such as myxoinflammatory fibroblastic sarcoma (MIFS) and hemosiderotic fibrolipomatous tumor (HFLT), although cytogenetic data remain inconsistent. We report a case of PHAT that arose in the upper arm with unusual morphology which showed ganglion-like cells similar to Reed-Sternberg-like cells found in MIFS. The tumor had strong immunohistochemical expression of CD34, CD99, and was negative for S-100. The ganglion-like cells were positive for both CD34 and CD68 but negative for CD30. The translocation between chromosome 1 and 10, a frequent finding of MIFS and HFLT, was not identified by FISH excluding the possibility of hybrid PHAT and MIFS. We conclude FISH can be a potential useful tool to separate PHAT with atypical morphology from hybrid tumor in doubted cases. Due to the rarity of PHAT and lack of consistent pathogenetic signatures, more cases and further studies will be needed to elucidate the pathogenesis and nature of this tumor.

  5. Novel Genes Implicated in Embryonal, Alveolar, and Pleomorphic Rhabdomyosarcoma: A Cytogenetic and Molecular Analysis of Primary Tumors1

    PubMed Central

    Goldstein, Myriam; Meller, Isaac; Issakov, Josephine; Orr-Urtreger, Avi

    2006-01-01

    Abstract Rhabdomyosarcoma, the most common pediatric soft tissue sarcoma, likely results from deregulation of the skeletal myogenesis program. Although associations between PAX3, PAX7, FOXO1A, and RMS tumorigenesis are well recognized, the entire spectrum of genetic factors underlying RMS development and progression is unclear. Using a combined approach of spectral karyotyping, array-based comparative genomic hybridization (CGH), and expression analysis, we examined 10 primary RMS tumors, including embryonal, alveolar, and the rare adult pleomorphic variant, to explore the involvement of different genes and genetic pathways in RMS tumorigenesis. A complete karyotype established for each tumor revealed a high aneuploidy level, mostly tetraploidy, with double minutes and additional structural aberrations. Quantitative expression analysis detected the overexpression of the AURKA gene in all tumors tested, suggesting a role for this mitotic regulator in the aneuploidy and chromosomal instability observed in RMS. Array-based CGH analysis in primary RMS tumors detected copy number changes of genes involved in multiple genetic pathways, including transcription factors such as MYC-related gene from lung cancer and the cytoskeleton and cell adhesion-encoding genes laminin γ-2 and p21-activated kinase-1. Our data suggest the involvement of genes encoding cell adhesion, cytoskeletal signaling, and transcriptional and cell cycle components in RMS tumorigenesis. PMID:16790082

  6. SYNOVIAL SARCOMA OF THE LARYNX.

    PubMed

    Javed, Nabila; Iqbal, Javed

    2015-01-01

    Synovial sarcoma is a mesenchymal spindle cell tumour that displays variable epithelial differentiation. It most commonly occurs in lower extremities. Head and neck is a rare site for synovial sarcoma accounting for less than 10%. Larynx is an extremely rare site and only 16 cases with laryngeal location have been reported. Immunohistochemistry is important for correct diagnosis. Surgical excision of the tumour with clear margins and local radiotherapy is effective in local control. Chemotherapy is indicated in the presence of distant metastasis. Case of a 16 years old female is presented with hoarseness of voice and mass in supraglottic region. Lateral pharangotomy and excision of mass revealed synovial sarcoma. She had been treated with adjuvant radiotherapy in September 2012. She was fine and coming for regular follow up.

  7. Testicular myeloid sarcoma: case report

    PubMed Central

    Zago, Luzia Beatriz Ribeiro; Ladeia, Antônio Alexandre Lisbôa; Etchebehere, Renata Margarida; de Oliveira, Leonardo Rodrigues

    2013-01-01

    Myeloid sarcomas are extramedullary solid tumors composed of immature granulocytic precursor cells. In association with acute myeloid leukemia and other myeloproliferative disorders, they may arise concurrently with compromised bone marrow related to acute myeloid leukemia, as a relapsed presentation, or occur as the first manifestation. The testicles are considered to be an uncommon site for myeloid sarcomas. No therapeutic strategy has been defined as best but may include chemotherapy, radiotherapy and/or hematopoietic stem cell transplantation. This study reports the evolution of a patient with testicular myeloid sarcoma as the first manifestation of acute myeloid leukemia. The patient initially refused medical treatment and died five months after the clinical condition started. PMID:23580888

  8. Lethal mass mimicking myxoma in the heart.

    PubMed

    Soo, Wern Miin; Pang, Yin Huei; Poh, Kian-Keong

    2014-10-01

    A 70-year-old man presented in advanced heart failure with jaundice. Transthoracic echocardiography revealed a 21 × 24-mm mass in the left atrium attached to the posterior mitral valve leaflet. Surgical excision was attempted, but the tumor had infiltrated the entire left atrial wall and was deemed too extensive to be resectable. Histology confirmed a high-grade pleomorphic sarcoma with malignant fibrous histiocytoma-like features. Liver biopsy revealed a high-grade liver sarcoma.

  9. Pleomorphic adenoma of the buccal salivary gland: magnetic resonance imaging findings with differential diagnoses.

    PubMed

    Sharma, Neeraj

    2012-08-01

    The majority of minor salivary gland tumors are malignant. Of the benign tumors, pleomorphic adenomas are most common. The cheeks, lips, and gingiva are rarely sites of occurrence. A case of pleomorphic adenoma of the buccal salivary gland is presented here with a discussion of the importance of magnetic resonance imaging in demonstrating the extent of the lesion and establishing a differential diagnoses.

  10. Pleomorphic rhabdomyosarcoma of the left atrium mimicking myxoma.

    PubMed

    Aggarwal, Tanvi; Goyal, Surbhi; Zaheer, Sufian

    2016-01-01

    Primary rhabdomyosarcoma (RMS) of the heart is a rare malignant tumor which has poor prognosis and survival despite surgery and adjuvant chemotherapy. The preoperative diagnosis is often difficult in view of nonspecific clinicoradiological findings. This report describes a case of a 60-year-old woman who was clinically diagnosed as left atrial myxoma. A diagnosis of pleomorphic RMS was made on histopathology after excision. Our case discusses the clinicopathological features and treatment options of cardiac RMS emphasizing the fact that histopathology and immunohistochemistry are essential to confirm the diagnosis of such an aggressive malignant tumor. PMID:27510683

  11. Pleomorphic Adenoma of the External Auditory Canal: A Rare Presentation

    PubMed Central

    Jaber, Samir; Rudic, Milan; Keogh, Ivan James

    2015-01-01

    A 55-year-old male presented with a nine-month history of gradually enlarging, painless mass in the right external auditory canal associated with hearing loss and occasional bleeding. Examination demonstrated complete obstruction of the outer 1/3 of the external auditory canal by a firm, pink, rubbery mass. CT scan of the temporal bone showed tumor mass with no evidence of bone destruction. The tumor was excised and histopathology confirmed a diagnosis of ceruminous pleomorphic adenoma of the external auditory canal. Six months following the surgery, patient is free of any recurrent disease. PMID:26106498

  12. Retroauricular Pleomorphic Adenoma Arising from Heterotopic Salivary Gland Tissue

    PubMed Central

    Bacaj, Patrick; Borah, Gregory

    2016-01-01

    Summary: A 38-year-old woman is described who presented with a slowly growing mass on the posterior aspect of the left ear. Excision and histopathologic evaluation revealed a pleomorphic adenoma (PA) originating from heterotopic salivary gland tissue. Many authors have presented cases of PAs originating from ceruminous glands in the external auditory canal or of so-called chondroid syringoma originating from apocrine and eccrine sweat glands. This is the only case in the recent literature of a PA originating from a heterotopic rest of salivary gland tissue in the retroauricular region. The 3 main sources of PAs, their embryologic derivation, and treatment are described.

  13. [Pleomorphic adenoma of the lung; report of a case].

    PubMed

    Noda, M; Tabata, T; Yamane, Y

    2002-11-01

    A 67-year-old woman was admitted because of an abnormal shadow on the chest X-ray film. Chest computed tomography (CT) film revealed a peripheral mass in the middle lower lobe of the lung. We performed open lung biopsy and diagnosed as a benign epithelial adenoma but not ruled out adenocarcinoma completely by intraoperative examination of frozen sections. We operated right middle lobectomy and mediastinal lymph nodes dissections. Histological examination confirmed pleomorphic adenoma. We examined salivary glands, but didn't find the focus of that. The patient had been well for three years postoperatively.

  14. Carcinoma ex pleomorphic adenoma of the upper lip.

    PubMed

    Dyalram, D; Huebner, T; Papadimitriou, J C; Lubek, J

    2012-03-01

    Carcinoma ex pleomorphic adenoma (CXPA) is a rare salivary gland malignancy most often reported within the parotid gland. Of the salivary gland tumours that occur within the minor salivary glands at least 50% are reported to be malignant. This proves to be inaccurate when describing salivary gland tumours within the upper lip which are usually benign. A Medline search of the English language literature yields only one case report of a CXPA located within the upper lip. The authors present a second case report of CXPA within the upper lip and a review of its pathologic features and management.

  15. Metastasizing pleomorphic adenoma of the submandibular gland: a case report.

    PubMed

    Miladi, S; Mestiri, S; Kermani, W; Ziadi, S; Sriha, B; Bouzouita, K; Mokni, M

    2014-03-01

    Pleomorphic adenoma (PA), originally called mixed tumour, is the most common neoplasm of the salivary glands. It is usually a benign, slow-growing and well-circumscribed tumour. However, PA may occasionally give rise to metastases that usually occur after a previous recurrence. These tumours display benign histological features in both primary tumours and metastases. Such tumours have been termed metastatic PA or metastatic mixed tumours. We report a case of metastatic PA of the submandibular gland with metastasis to the cervical lymph nodes.

  16. Pleomorphic adenoma of the pterygopalatine fossa: a case report.

    PubMed

    Kanazawa, T; Nishino, H; Ichimura, K

    2000-01-01

    Pleomorphic adenoma (PA) is the most common benign tumor of the major salivary glands. It can also Occur in the minor salivary glands, mainly in the oral cavity, and in other sites in the head and neck region. We present a very rare case of PA in the pterygopalatine fossa. Surgical resection of the tumor was performed via the transmaxillary approach. The patient has experienced neither surgical complications nor recurrence in the past 3 years. This case suggests that a localized benign tumor in the pterygopalatine fossa can be removed safely and efficaciously via a transmaxillary approach.

  17. Pleomorphic adenoma originates from inferior nasal turbinate causing epiphora.

    PubMed

    Erol, Bekir; Selçuk, Ömer Tarik; Gürses, Cemil; Osma, Üstün; Köroğlu, Mert; Süren, Dinç

    2013-01-01

    Pleomorphic adenoma is the most common benign tumor of the salivary glands. A 62-year-old female patient presented with epiphora and was suffering from breathing difficulties. With the diagnostic nasal endoscopy, a mass, originating from right inferior nasal turbinate and filling the entire nasal cavity, was seen. Originating from the inferior nasal turbinate is a very rare entity. Paranasal sinus computed tomography and magnetic resonance images revealed a mass that fills and expands the right nasal cavity. Mass was hypoechoic in B-mode ultrasonography and hypovascular in color Doppler ultrasonography, and rate of tissue stiffness was high in sonoelastography. These were helpful for the diagnosis.

  18. Mast cell sarcoma: clinical management.

    PubMed

    Weiler, Catherine R; Butterfield, Joseph

    2014-05-01

    Mast cell sarcoma is a disorder that results in abnormal mast cells as identified by morphology, special stains, and in some publications, c-kit mutation analysis. It affects animal species such as canines more commonly than humans. In humans it is a very rare condition, with variable clinical presentation. There is no standard therapy for the disorder. It can affect any age group. It is occasionally associated with systemic mastocytosis and/or urticaria pigmentosa. The prognosis of mast cell sarcoma in published literature is very poor in humans.

  19. Extra osseous primary Ewing's sarcoma.

    PubMed

    Ali, Syed Asad; Muhammad, Agha Taj; Soomro, Abdul Ghani; Siddiqui, Akmal Jamal

    2010-01-01

    The case of 20 years old boy with an extra osseous Ewing's sarcoma is described. He was initially diagnosed as a case of infiltrative malignant tumour of left suprarenal gland on the basis of preoperative workup but postoperative biopsy of surgically excised specimen confirmed Extra-osseous Ewing's Sarcoma (EES) suprarenal gland with no evidence of malignancy on skeletal scintiscan, bone marrow aspirate and histopathology Suprarenal location of primary EES is unknown and probably has not been reported in literature. We report a unique case of EES.

  20. Primary histiocytic sarcoma arising in the head and neck with predominant spindle cell component

    PubMed Central

    Alexiev, Borislav A; Sailey, Charles J; McClure, Shawn A; Ord, Robert A; Zhao, XF; Papadimitriou, John C

    2007-01-01

    This is the first case report of Histiocytic Sarcoma (HS) with predominant spindle cell component occurring in the head and neck region of a 41-year-old man. The tumor was composed of sheets of large round to oval cells with pleomorphic vesicular nuclei, prominent nucleoli and abundant eosinophilic cytoplasm. Multinucleated forms, numerous mitoses, and tumor necrosis were also noted. Sheets, fascicles, and whorls of spindle cells with spindled to ovoid vesicular nuclei, small to medium-sized distinct nucleoli, and eosinophilic cytoplasm were frequently observed. Immunohistochemical staining in the tumor cells was positive for CD163, CD68, lysozyme, CD45, and NSE. Focal expression of CD4 and S-100 was also noted. Electron microscopy demonstrated an abundance of lysosomes in the cytoplasm of tumor cells. Chromosome study revealed a 57–80 hyperdiploid [7]/46, XY [13] karyotype, including 3 to 4 copies of various chromosomes. The immunohistochemical and ultrastructural findings confirmed the diagnosis of HS. PMID:17324277

  1. Role of fine needle aspiration cytology in diagnosis of pleomorphic adenomas.

    PubMed

    Verma, Kusum; Kapila, Kusum

    2002-04-01

    This retrospective study was carried out to review the cases diagnosed as pleomorphic adenoma in major or minor salivary glands and determine the difficulties encountered on typing this tumour on fine needle aspiration cytology (FNAC). Over a 19-year period (1982-2000) 488 pleomorphic adenomas were diagnosed on FNAC from different sites (parotid - 372 cases, submandibular - 95 cases; oral cavity - 21 cases). Histology was available in 232 cases. Twenty-nine cases where a histological diagnosis of pleomorphic adenoma was made but the cytological diagnosis was variable were also reviewed. In 216 of the 232 cases a good cytohistological correlation was available. On review only 4 of the 16 cases initially diagnosed as pleomorphic adenoma on FNAC where the histology revealed a different tumour were categorized as pleomorphic adenoma, while 3 each were classified as adenoid cystic carcinoma and benign tumour ?type, and 2 each were diagnosed to be muco-epidermoid carcinoma, monomorphic adenoma and acinic cell carcinoma. On review of the FNAC smears from 29 cases where a histological diagnosis of pleomorphic adenoma was available while the cytological diagnosis was variable, only 11 (38%) were categorized as pleomorphic adenoma. In the majority of the remaining cases the cytological diagnosis did not alter markedly, 7 of 10 cases where the tumour could not be typed on cytology initially could not be typed even on review. In conclusion, FNAC is an ideal, fairly accurate preoperative procedure for the diagnosis of pleomorphic adenomas. Certain diagnostic problems occur in differentiating pleomorphic adenomas from adenoid cystic carcinoma, monomorphic adenoma and mucoepidermoid carcinoma. Carcinoma ex-pleomorphic adenoma is difficult to identify on FNAC and in our series all 4 such cases on histology were considered benign on cytology. PMID:11952751

  2. Role of fine needle aspiration cytology in diagnosis of pleomorphic adenomas.

    PubMed

    Verma, Kusum; Kapila, Kusum

    2002-04-01

    This retrospective study was carried out to review the cases diagnosed as pleomorphic adenoma in major or minor salivary glands and determine the difficulties encountered on typing this tumour on fine needle aspiration cytology (FNAC). Over a 19-year period (1982-2000) 488 pleomorphic adenomas were diagnosed on FNAC from different sites (parotid - 372 cases, submandibular - 95 cases; oral cavity - 21 cases). Histology was available in 232 cases. Twenty-nine cases where a histological diagnosis of pleomorphic adenoma was made but the cytological diagnosis was variable were also reviewed. In 216 of the 232 cases a good cytohistological correlation was available. On review only 4 of the 16 cases initially diagnosed as pleomorphic adenoma on FNAC where the histology revealed a different tumour were categorized as pleomorphic adenoma, while 3 each were classified as adenoid cystic carcinoma and benign tumour ?type, and 2 each were diagnosed to be muco-epidermoid carcinoma, monomorphic adenoma and acinic cell carcinoma. On review of the FNAC smears from 29 cases where a histological diagnosis of pleomorphic adenoma was available while the cytological diagnosis was variable, only 11 (38%) were categorized as pleomorphic adenoma. In the majority of the remaining cases the cytological diagnosis did not alter markedly, 7 of 10 cases where the tumour could not be typed on cytology initially could not be typed even on review. In conclusion, FNAC is an ideal, fairly accurate preoperative procedure for the diagnosis of pleomorphic adenomas. Certain diagnostic problems occur in differentiating pleomorphic adenomas from adenoid cystic carcinoma, monomorphic adenoma and mucoepidermoid carcinoma. Carcinoma ex-pleomorphic adenoma is difficult to identify on FNAC and in our series all 4 such cases on histology were considered benign on cytology.

  3. Prognostic features of renal sarcomas (Review)

    PubMed Central

    ÖZTÜRK, HAKAN

    2015-01-01

    The aim of the present review was to evaluate the prognostic features of primary sarcomas of the kidney. A literature review was conducted using a number of databases, including Medline (PubMed) and Scopus, for studies published between January 1992 and December 2013. Of the studies published in English, those describing the prognostic features of primary sarcomas of the kidney were recorded. The electronic search was limited to the following keywords: Sarcoma, renal sarcoma, prognosis, diagnosis, immunohistochemistry, genetic and survey. Subsequent to the search, no review articles and/or meta-analyses associated with the prognosis of primary sarcomas of the kidney were identified. In total, 31 studies, which consisted of case studies, case series and studies concerned with the overall prognosis of urological soft-tissue sarcomas, were reviewed. Primary sarcoma of the kidney has a poor prognosis compared with other sarcomas of the urogenital system. In addition to the surgical excision of renal sarcomas, pathological, molecular and genetic prognostic factors are also considered. Due to the small number of cases, previous studies have not randomized the prognostic features of primary sarcomas of the kidney. The elucidation of the so-called ‘chaotic’ genetic and molecular basis of renal sarcomas will help to predict patient prognoses. Surgical excision is the most significant parameter for determining the prognosis of sarcomas of the kidney. However, sarcomas also exhibit prognostic features that are based upon pathological, genetic and molecular factors. The present review suggests that additional factors may be important in predicting the prognosis of patients with renal sarcomas, and that clinicians should plan treatment and follow-up regimens according to these factors. PMID:25663853

  4. Pleomorphic adenoma of a deep orbital ectopic lacrimal gland.

    PubMed

    Misra, Somen; Bhandari, Akshay; Misra, Neeta; Gogri, Pratik; Mahajan, Shruti

    2016-10-01

    Ectopic lacrimal gland, being one of the choristomas, is comprised of lacrimal gland tissue outside the lacrimal gland fossa in the fronto-lateral part of the orbital roof. Ectopic lacrimal gland is a rare condition where the gland may be found in the orbit, eyelids, ocular adnexa or within the globe. Neoplastic transformation of such tissue may occur. A sixty-two-year old male patient presented with right eye proptosis and slight nasal displacement of the globe. Computerized tomography scan revealed a well-defined hypodense lesion of size 19 x 18 x 20 mm supero-lateral to lateral rectus muscle, with mild proptosis and thinning of the right lateral orbital wall. Excisional biopsy was performed through a lateral orbitotomy approach. A well circumscribed globular mass was removed from the right orbit, well behind the fossa for the lacrimal gland in the retrobulbar space. Histopathology was suggestive of pleomorphic adenoma of lacrimal gland. Pleomorphic adenoma is an epithelial tumor of the lacrimal gland which is extremely rare from an ectopic lacrimal gland and only few cases have been reported in literature till date.

  5. Pleomorphic adenoma of a deep orbital ectopic lacrimal gland.

    PubMed

    Misra, Somen; Bhandari, Akshay; Misra, Neeta; Gogri, Pratik; Mahajan, Shruti

    2016-10-01

    Ectopic lacrimal gland, being one of the choristomas, is comprised of lacrimal gland tissue outside the lacrimal gland fossa in the fronto-lateral part of the orbital roof. Ectopic lacrimal gland is a rare condition where the gland may be found in the orbit, eyelids, ocular adnexa or within the globe. Neoplastic transformation of such tissue may occur. A sixty-two-year old male patient presented with right eye proptosis and slight nasal displacement of the globe. Computerized tomography scan revealed a well-defined hypodense lesion of size 19 x 18 x 20 mm supero-lateral to lateral rectus muscle, with mild proptosis and thinning of the right lateral orbital wall. Excisional biopsy was performed through a lateral orbitotomy approach. A well circumscribed globular mass was removed from the right orbit, well behind the fossa for the lacrimal gland in the retrobulbar space. Histopathology was suggestive of pleomorphic adenoma of lacrimal gland. Pleomorphic adenoma is an epithelial tumor of the lacrimal gland which is extremely rare from an ectopic lacrimal gland and only few cases have been reported in literature till date. PMID:27541944

  6. Oral rehabilitation after surgical removal of pleomorphic adenoma.

    PubMed

    Goiato, Marcelo Coelho; Tamae, Adriano Caires; Silva, Pedro Ivo Santos; dos Santos, Daniela Micheline; Iyda, Mariana Garib; Moreno, Amalia; Magro-Filho, Osvaldo; Bertoz, André Pinheiro Magalhães

    2011-11-01

    Although tumors of minor salivary glands are rare, the pleomorphic adenoma is the most common pathology among the benign neoplasm and can be found with high prevalence in the junction between hard palate and soft palate. The treatment of choice for most of maxillary tumors is surgical through either a total or partial maxillectomy. However, surgical defects caused by such type of treatment lead to both clinical and psychologic disorders for the patient. The immediate oral rehabilitation using interim palate obturator after maxillectomy provides optimization on the healing process, recovers the stomatognathic functions after surgery, and avoids psychosocial sequelae for the patients. This clinical report aimed to present the rehabilitation with immediate palate obturator of a patient who underwent a partial maxillectomy due to a hard palate pleomorphic adenoma of minor salivary glands. We report the clinical importance of the prosthetic rehabilitation and the improvements on both quality of life and stomatognathic functions of this patient. It can be concluded that the immediate rehabilitation of the patient after partial maxillectomy by using an interim palate obturator was a great option and provided clinical benefits in the immediate postoperative period, improving the patient’s quality of life, allowing the patient’s reinsertion into society, and reducing the surgical treatment sequelae.

  7. Carcinomas ex pleomorphic adenoma and malignant mixed tumors. Histomorphologic indexes.

    PubMed

    Tortoledo, M E; Luna, M A; Batsakis, J G

    1984-03-01

    Clinical and pathologic differences exist between the several neoplasms encompassed by the term malignant mixed tumors of salivary glands. The majority of the neoplasms are carcinomas ex pleomorphic adenoma. True malignant mixed tumors (carcinosarcomas) are rare, and even more rare are the benign metastasizing mixed tumors. This study of 40 malignant mixed tumors indicates that two previously unreported variables, measured invasion in millimeters and histologic subclassifications of the malignant neoplasm, are valuable guides to prognosis and biologic behavior. All patients whose malignant neoplasm extended for more than 8 mm beyond residual capsule or benign residual tumor died of their disease. The extent of invasion also correlated with perineurial invasion, involvement of bone, and metastases to lymph nodes. Histologic subclassification points out that there is no prototypical carcinoma ex pleomorphic adenoma and that high- and low-grade carcinomas can be found. Only one of the patients with low-grade (terminal duct) carcinomas died of his disease during follow-up periods extending to over 20 years.

  8. View looking northwest toward HIghGrade Ore Bin and Concentrate Bin ...

    Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

    View looking northwest toward HIgh-Grade Ore Bin and Concentrate Bin - Kennecott Copper Corporation, Concentration Mill, On Copper River & Northwestern Railroad, Kennicott, Valdez-Cordova Census Area, AK

  9. Pneumothorax as adverse event in patients with lung metastases of soft tissue sarcoma treated with pazopanib: a single reference centre case series

    PubMed Central

    2014-01-01

    Background Recently, the phase III PALETTE study introduced pazopanib (Votrient®) as treatment for adult patients with locally advanced or metastatic non-liposarcoma soft tissue sarcoma after prior treatment with doxorubicin and/or ifosfamide. Pneumothorax was reported as adverse event in 8 of 246 treated patients (3.3%) in that study. This case series presents the incidence and clinic of this complication in the Leiden University Medical Centre. Cases Forty-three patients were treated with pazopanib of which six patients (14.0%) developed a pneumothorax. These six patients were treated for malignant peripheral nerve sheath tumour, angiosarcoma, synovial sarcoma, fibromyxomatoid sarcoma, pleomorphic sarcoma and endometrial stromal sarcoma. All six patients had subpleural pulmonary or pleural metastases at the start of pazopanib and the pneumothorax developed during or shortly after treatment with pazopanib and was difficult to treat. Discussion The incidence reported by us is higher than the incidence in the PALETTE study. Trials with pazopanib in renal cell carcinoma, urothelial carcinoma and cervix carcinoma did not report pneumothorax as an adverse event, suggesting pneumothorax as a specific adverse event in soft tissue sarcoma patients treated with pazopanib. This may be related to the fact that there is often pleural metastatic involvement and cystic degeneration due to pazopanib treatment may add to the risk. Conclusion The risk of an, often difficult to treat, pneumothorax during pazopanib therapy should be discussed with the patient before initiation of treatment for a pulmonary metastasized sarcoma and physicians should be alert to the occurrence of such an event. PMID:25302110

  10. Gemcitabine Hydrochloride, Docetaxel, and Radiation Therapy in Treating Patients With Uterine Sarcoma That Has Been Removed By Surgery

    ClinicalTrials.gov

    2015-01-16

    Stage IA Uterine Sarcoma; Stage IB Uterine Sarcoma; Stage IC Uterine Sarcoma; Stage IIA Uterine Sarcoma; Stage IIB Uterine Sarcoma; Stage IIIA Uterine Sarcoma; Stage IIIB Uterine Sarcoma; Stage IIIC Uterine Sarcoma; Stage IVA Uterine Sarcoma; Stage IVB Uterine Sarcoma; Uterine Corpus Leiomyosarcoma

  11. Kaposi's sarcoma of the penis.

    PubMed

    Houston, W; Pontin, A; Kuhn, T; Mambo, N

    1975-06-01

    The 11th case of Kaposi's sarcoma of the glans penis is reported. Aetiology and treatment are discussed and evidence adduced to support a conservative surgical approach by local excision, supplemented if necessary by low dosage irradiation and possibly methotrexate infusion.

  12. Molecular Approaches to Sarcoma Therapy

    PubMed Central

    Olsen, R. J.; Tarantolo, S. R.

    2002-01-01

    Soft tissue sarcomas comprise a heterogeneous group of aggressive tumors that have a relatively poor prognosis. Although conventional therapeutic regimens can effectively cytoreduce the overall tumor mass, they fail to consistently achieve a curative outcome. Alternative gene-based approaches that counteract the underlying neoplastic process by eliminating the clonal aberrations that potentiate malignant behavior have been proposed. As compared to the accumulation of gene alterations associated with epithelial carcinomas, sarcomas are frequently characterized by the unique presence of a single chromosomal translocation in each histological subtype. Similar to the Philadelphia chromosome associated with CML, these clonal abnormalities result in the fusion of two independent unrelated genes to generate a unique chimeric protein that displays aberrant activity believed to initiate cellular transformation. Secondary gene mutations may provide an additional growth advantage that further contributes to malignant progression. The recent clinical success of the tyrosine kinase inhibitor, STI571, suggests that therapeutic approaches specifically directed against essential survival factors in sarcoma cells may be effective. This review summarizes published approaches targeting a specific molecular mechanism associated with sarcomagenesis. The strategy and significance of published translational studies in six distinct areas are presented. These include: (1) the disruption of chimeric transcription factor activity; (2) inhibition of growth stimulatory post-translational modifications; (3) restoration of tumor suppressor function; (4) interference with angiogenesis; (5) induction of apoptotic pathways; and (6) introduction of toxic gene products. The potential for improving outcomes in sarcoma patients and the conceptual obstacles to be overcome are discussed. PMID:18521343

  13. Armodafinil in Reducing Cancer-Related Fatigue in Patients With High Grade Glioma | Division of Cancer Prevention

    Cancer.gov

    This randomized phase III trial studies armodafinil to see how well it works in reducing cancer-related fatigue in patients with high grade glioma. Armodafinil may help relieve fatigue in patients with high grade glioma. |

  14. High-grade ovarian cancer secreting effective exosomes in tumor angiogenesis.

    PubMed

    Yi, Huan; Ye, Jun; Yang, Xiao-Mei; Zhang, Li-Wen; Zhang, Zhi-Gang; Chen, Ya-Ping

    2015-01-01

    Ovarian cancer, the most lethal gynecological cancer, related closely to tumor stage. High-grade ovarian cancer always results in a late diagnose and high recurrence, which reduce survival within five years. Until recently, curable therapy is still under research and anti-angiogenesis proves a promising way. Tumor-derived exosomes are essential in tumor migration and metastases such as angiogenesis is enhanced by exosomes. In our study, we have made comparison between high-grade and unlikely high-grade serous ovarian cancer cells on exosomal function of endothelial cells proliferation, migration and tube formation. Exosomes derived from high-grade ovarian cancer have a profound impact on angiogenesis with comparison to unlikely high-grade ovarian cancer. Proteomic profiles revealed some potential proteins involved in exosomal function of angiogenesis such as ATF2, MTA1, ROCK1/2 and so on. Therefore, exosomes plays an influential role in angiogenesis in ovarian serous cancer and also function more effectively in high-grade ovarian cancer cells.

  15. Kaposi's sarcoma in South Africa.

    PubMed

    Sitas, F; Newton, R

    2001-01-01

    Kaposi's sarcoma was endemic in South Africa even before the advent of the human immunodeficiency virus (HIV). Between 1988 and 1996, the incidence of Kaposi's sarcoma in South Africa has risen at least threefold and continues to increase as the HIV epidemic grows. Research from South Africa has shown that infection with human herpesvirus 8 (HHV8) is associated with Kaposi's sarcoma but not with any other major cancer site or type. In addition, the risk of Kaposi's sarcoma increases with increasing antibody titer to HHV8, but, for a given titer, the risk is greater in HIV-seropositive compared with HIV-seronegative individuals. The age- and sex-standardized seroprevalence of HHV8 in black South African hospital patients was found to be slightly more than 30%; the seroprevalence of HHV8 increased with age and was similar in men and in women. The modes of transmission of HHV8 are yet to be fully elucidated. Limited evidence exists for sexual transmission in black South African adults, but mother-to-child and person-to-person transmission in childhood is also likely. Furthermore, the seroprevalence of HHV8 decreases with increasing levels of education and is lower in whites than in blacks, suggesting that factors associated with poverty may be important determinants of transmission. Future research should focus on risk factors for Kaposi's sarcoma in HHV8-infected individuals, on determinants and mode of transmission of HHV8, and on the elucidation of the effect of primary HHV8 infection in adults and in children.

  16. Kaposi's sarcoma in South Africa.

    PubMed

    Sitas, F; Newton, R

    2001-01-01

    Kaposi's sarcoma was endemic in South Africa even before the advent of the human immunodeficiency virus (HIV). Between 1988 and 1996, the incidence of Kaposi's sarcoma in South Africa has risen at least threefold and continues to increase as the HIV epidemic grows. Research from South Africa has shown that infection with human herpesvirus 8 (HHV8) is associated with Kaposi's sarcoma but not with any other major cancer site or type. In addition, the risk of Kaposi's sarcoma increases with increasing antibody titer to HHV8, but, for a given titer, the risk is greater in HIV-seropositive compared with HIV-seronegative individuals. The age- and sex-standardized seroprevalence of HHV8 in black South African hospital patients was found to be slightly more than 30%; the seroprevalence of HHV8 increased with age and was similar in men and in women. The modes of transmission of HHV8 are yet to be fully elucidated. Limited evidence exists for sexual transmission in black South African adults, but mother-to-child and person-to-person transmission in childhood is also likely. Furthermore, the seroprevalence of HHV8 decreases with increasing levels of education and is lower in whites than in blacks, suggesting that factors associated with poverty may be important determinants of transmission. Future research should focus on risk factors for Kaposi's sarcoma in HHV8-infected individuals, on determinants and mode of transmission of HHV8, and on the elucidation of the effect of primary HHV8 infection in adults and in children. PMID:11158199

  17. Inhibition of SP1 by the mithramycin analog EC-8042 efficiently targets tumor initiating cells in sarcoma.

    PubMed

    Tornin, Juan; Martinez-Cruzado, Lucia; Santos, Laura; Rodriguez, Aida; Núñez, Luz-Elena; Oro, Patricia; Hermosilla, Maria Ana; Allonca, Eva; Fernández-García, Maria Teresa; Astudillo, Aurora; Suarez, Carlos; Morís, Francisco; Rodriguez, Rene

    2016-05-24

    Tumor initiating cells (TICs), responsible for tumor initiation, and cancer stem cells (CSCs), responsible for tumor expansion and propagation, are often resistant to chemotherapeutic agents. To find therapeutic targets against sarcoma initiating and propagating cells we used models of myxoid liposarcoma (MLS) and undifferentiated pleomorphic sarcoma (UPS) developed from human mesenchymal stromal/stem cells (hMSCs), which constitute the most likely cell-of-origin for sarcoma. We found that SP1-mediated transcription was among the most significantly altered signaling. To inhibit SP1 activity, we used EC-8042, a mithramycin (MTM) analog (mithralog) with enhanced anti-tumor activity and highly improved safety. EC-8042 inhibited the growth of TIC cultures, induced cell cycle arrest and apoptosis and upregulated the adipogenic factor CEBPα. SP1 knockdown was able to mimic the anti-proliferative effects induced by EC-8042. Importantly, EC-8042 was not recognized as a substrate by several ABC efflux pumps involved in drug resistance, and, opposite to the chemotherapeutic drug doxorubicin, repressed the expression of many genes responsible for the TIC/CSC phenotype, including SOX2, C-MYC, NOTCH1 and NFκB1. Accordingly, EC-8042, but not doxorubicin, efficiently reduced the survival of CSC-enriched tumorsphere sarcoma cultures. In vivo, EC-8042 induced a profound inhibition of tumor growth associated to a strong reduction of the mitotic index and the induction of adipogenic differentiation and senescence. Finally, EC-8042 reduced the ability of tumor cells to reinitiate tumor growth. These data suggest that EC-8042 could constitute an effective treatment against both TIC and CSC subpopulations in sarcoma. PMID:27105533

  18. Inhibition of SP1 by the mithramycin analog EC-8042 efficiently targets tumor initiating cells in sarcoma

    PubMed Central

    Tornin, Juan; Martinez-Cruzado, Lucia; Santos, Laura; Rodriguez, Aida; Núñez, Luz-Elena; Oro, Patricia; Hermosilla, Maria Ana; Allonca, Eva; Fernández-García, Maria Teresa; Astudillo, Aurora; Suarez, Carlos; Morís, Francisco; Rodriguez, Rene

    2016-01-01

    Tumor initiating cells (TICs), responsible for tumor initiation, and cancer stem cells (CSCs), responsible for tumor expansion and propagation, are often resistant to chemotherapeutic agents. To find therapeutic targets against sarcoma initiating and propagating cells we used models of myxoid liposarcoma (MLS) and undifferentiated pleomorphic sarcoma (UPS) developed from human mesenchymal stromal/stem cells (hMSCs), which constitute the most likely cell-of-origin for sarcoma. We found that SP1-mediated transcription was among the most significantly altered signaling. To inhibit SP1 activity, we used EC-8042, a mithramycin (MTM) analog (mithralog) with enhanced anti-tumor activity and highly improved safety. EC-8042 inhibited the growth of TIC cultures, induced cell cycle arrest and apoptosis and upregulated the adipogenic factor CEBPα. SP1 knockdown was able to mimic the anti-proliferative effects induced by EC-8042. Importantly, EC-8042 was not recognized as a substrate by several ABC efflux pumps involved in drug resistance, and, opposite to the chemotherapeutic drug doxorubicin, repressed the expression of many genes responsible for the TIC/CSC phenotype, including SOX2, C-MYC, NOTCH1 and NFκB1. Accordingly, EC-8042, but not doxorubicin, efficiently reduced the survival of CSC-enriched tumorsphere sarcoma cultures. In vivo, EC-8042 induced a profound inhibition of tumor growth associated to a strong reduction of the mitotic index and the induction of adipogenic differentiation and senescence. Finally, EC-8042 reduced the ability of tumor cells to reinitiate tumor growth. These data suggest that EC-8042 could constitute an effective treatment against both TIC and CSC subpopulations in sarcoma. PMID:27105533

  19. Inhibition of SP1 by the mithramycin analog EC-8042 efficiently targets tumor initiating cells in sarcoma.

    PubMed

    Tornin, Juan; Martinez-Cruzado, Lucia; Santos, Laura; Rodriguez, Aida; Núñez, Luz-Elena; Oro, Patricia; Hermosilla, Maria Ana; Allonca, Eva; Fernández-García, Maria Teresa; Astudillo, Aurora; Suarez, Carlos; Morís, Francisco; Rodriguez, Rene

    2016-05-24

    Tumor initiating cells (TICs), responsible for tumor initiation, and cancer stem cells (CSCs), responsible for tumor expansion and propagation, are often resistant to chemotherapeutic agents. To find therapeutic targets against sarcoma initiating and propagating cells we used models of myxoid liposarcoma (MLS) and undifferentiated pleomorphic sarcoma (UPS) developed from human mesenchymal stromal/stem cells (hMSCs), which constitute the most likely cell-of-origin for sarcoma. We found that SP1-mediated transcription was among the most significantly altered signaling. To inhibit SP1 activity, we used EC-8042, a mithramycin (MTM) analog (mithralog) with enhanced anti-tumor activity and highly improved safety. EC-8042 inhibited the growth of TIC cultures, induced cell cycle arrest and apoptosis and upregulated the adipogenic factor CEBPα. SP1 knockdown was able to mimic the anti-proliferative effects induced by EC-8042. Importantly, EC-8042 was not recognized as a substrate by several ABC efflux pumps involved in drug resistance, and, opposite to the chemotherapeutic drug doxorubicin, repressed the expression of many genes responsible for the TIC/CSC phenotype, including SOX2, C-MYC, NOTCH1 and NFκB1. Accordingly, EC-8042, but not doxorubicin, efficiently reduced the survival of CSC-enriched tumorsphere sarcoma cultures. In vivo, EC-8042 induced a profound inhibition of tumor growth associated to a strong reduction of the mitotic index and the induction of adipogenic differentiation and senescence. Finally, EC-8042 reduced the ability of tumor cells to reinitiate tumor growth. These data suggest that EC-8042 could constitute an effective treatment against both TIC and CSC subpopulations in sarcoma.

  20. Can proliferation biomarkers reliably predict recurrence in World Health Organization 2003 defined endometrial stromal sarcoma, low grade?

    PubMed

    Feng, Weiwei; Malpica, Anais; Skaland, Ivar; Gudlaugsson, Einar; Robboy, Stanley J; Dalen, Ingvild; Hua, Keqin; Zhou, Xianrong; Baak, Jan P A

    2013-01-01

    An estimated 1500-3000 invasive Endometrial Stromal Sarcomas (ESS) cases annually occur worldwide. Before 2003, ESS was divided as low and high grade ESS based on mitotic activity. In 2003 the WHO changed the names, excluded mitoses and made nuclear atypia and necrosis the essential diagnostic criteria to distinguish ESS, Low Grade (ESS-LG, recurrence-free survival >90%) and Undifferentiated Endometrial Sarcoma (UES, poor prognosis). We have evaluated in WHO2003 defined ESS-LG whether proliferation biomarkers predict recurrence. Using survival analysis, the prognostic value of classical mitosis counts (Mitotic Activity Index, MAI) in haematoxyllin-eosin (H&E) sections, and immunohistochemical proliferation biomarkers (Ki-67 and PhosphoHistone-3 (PPH3)) were examined in 24 invasive endometrial stromal sarcomas. Three of 24 (12.5%) ESS-LG recurred. The MAI, PPH3 and Ki-67 were all prognostic (P = 0.001, 0.002 and 0.03). MAI values were >3 in the recurrent cases, but never exceeded 10 (the classical threshold for low and high grade). Non-recurrent cases had 0 ≤ MAI ≤ 3. PPH3 and Ki67 counts can be easier to perform than MAI and therefore helpful in the diagnosis of ESS, Low Grade. In conclusion, in this small study of WHO2003 defined ESS-LG, high levels of proliferation as measured by MAI, PPH3 and Ki-67 are predictive of recurrence. Larger studies are required to confirm these results.

  1. What's New in Soft Tissue Sarcomas Research and Treatment?

    MedlinePlus

    ... Topic Additional resources for soft tissue sarcoma What`s new in soft tissue sarcoma research and treatment? Research ... develop. This information is already being applied to new tests to diagnose and classify sarcomas. This is ...

  2. Pleomorphic Adenoma in Retromolar Area: A Very Rare Case Report and Review of Literature

    PubMed Central

    Khan, Tahseen Ali; Dhurjati, Venkata Naga Nalini; Gaddikeri, Kavitha; Khany, MD Zainuddin E.

    2016-01-01

    Among all neoplasms affecting head and neck region, salivary gland neoplasms are rare. Pleomorphic adenomas are the most common benign salivary gland tumours making up to 50% of major and minor salivary gland tumours. Intraorally pleomorphic adenoma is mostly found on palate and lips and very rarely in retromolar area. Here we are reporting a rare case of pleomorphic adenoma in right lower retromolar area in a 31-year-old female, the lesion was excised in toto with safety margins under local anaesthesia and postoperative follow up after six months didn’t showed any recurrence. PMID:26894184

  3. Pleomorphic adenoma of the cheek in a child: A case report

    PubMed Central

    Jagadishkumar, Kalenahalli; Anilkumar, Mathod Ganeshrao; Krishna Kumar, Halasahalli Chowdegowda; Maggad, Rangaswamy

    2014-01-01

    Salivary gland tumors are rare in children and, when they do arise, they mainly affect the major salivary glands. Minor salivary gland tumors are rare in children and are responsible for less than 10% of the cases. Pleomorphic adenoma is the most common tumor of the salivary glands. The most common sites of pleomorphic adenoma of the minor salivary glands are the palates, followed by the lips and the cheeks. Pleomorphic adenoma of the cheek is rare in children and only few cases have been reported so far. PMID:25225569

  4. Intracapsular (in situ) carcinoma ex pleomorphic adenoma with unusual clinical and histological features.

    PubMed

    Logasundaram, Rajesh; Amarawickrama, Himan; Premachandra, Don; Hellquist, Henrik

    2008-12-01

    Carcinoma ex pleomorphic adenoma poses a challenge to diagnosis and treatment. Herein we describe an extremely unusual case, which presented initially as an intracapsular carcinoma ex pleomorphic adenoma in the right parotid gland. The first recurrence, after an interval of 8 years, showed only recurrence of the benign component. Five years later, a myoepithelial carcinoma arose from the same site. No ductal carcinoma as seen in the initial intracapsular carcinoma ex pleomorphic adenoma was identified. This case report elucidates the atypical clinical behaviour and interesting histological features encountered within this group of salivary neoplasm.

  5. "Benign" metastasizing pleomorphic adenoma. A case report and review of literature.

    PubMed

    Qureshi, A A; Gitelis, S; Templeton, A A; Piasecki, P A

    1994-11-01

    A case of a 34-year-old man with a pathologic fracture through a lytic lesion in the left glenoid is presented. The results of a needle biopsy of the lesion showed a benign pleomorphic adenoma. Sixteen years previously, the patient had a pleomorphic adenoma of the parotid gland. Histology of the initial tissue and the needle biopsy specimen were identical. The patient developed metastasis without any local recurrence of the initial tumor. When evaluating a patient with a lytic lesion of bone with a history of a benign pleomorphic adenoma of the salivary gland, a metastasis should be considered.

  6. Pleomorphic adenoma in an atypical location near the temporomandibular joint: a case report.

    PubMed

    Smolka, Wenko; Eggensperger, Nicole; Stauffer-Brauch, Edouard Jean; von Bredow, Ferdinand; Lizuka, Tateyuki

    2007-05-01

    Pleomorphic adenomas primarily arise in the major salivary glands, especially in the parotid. The most common area is the lower pole superficial to the plane of the facial nerve. In this report, a pleomorphic adenoma in an atypical location--the region of the temporomandibular joint (TMJ)--is presented. The tumor was solitary, closely attached to the capsule of the TMJ and superior to the parotid gland, with clear demarcation. Clinically, the tumor resembled TMJ pathology, but MRI examination led to diagnosis of a benign tumor attached to the TMJ. This report shows that pleomorphic adenoma can be a possible diagnosis for lesions at the joint capsule.

  7. Pleomorphic Adenoma in Retromolar Area: A Very Rare Case Report and Review of Literature.

    PubMed

    Qureshi, Md Yousuf; Khan, Tahseen Ali; Dhurjati, Venkata Naga Nalini; Gaddikeri, Kavitha; Khany, Md Zainuddin E

    2016-01-01

    Among all neoplasms affecting head and neck region, salivary gland neoplasms are rare. Pleomorphic adenomas are the most common benign salivary gland tumours making up to 50% of major and minor salivary gland tumours. Intraorally pleomorphic adenoma is mostly found on palate and lips and very rarely in retromolar area. Here we are reporting a rare case of pleomorphic adenoma in right lower retromolar area in a 31-year-old female, the lesion was excised in toto with safety margins under local anaesthesia and postoperative follow up after six months didn't showed any recurrence.

  8. Metastasizing "benign" pleomorphic salivary adenoma: a dramatic case-report and literature review.

    PubMed

    Tarsitano, Achille; Foschini, Maria Pia; Farneti, Paolo; Pasquini, Ernesto; Marchetti, Claudio

    2014-12-01

    Pleomorphic salivary adenomas are the most common benign neoplasms affecting the salivary glands. Very occasionally however, metastatic lesions are identified in patients with a history of PSA, which, on detailed pathological evaluation, are found to exhibit all the histological hallmarks of the preceding benign lesions. Diagnosis of benign metastasizing pleomorphic adenoma of the salivary gland is extremely rare and still under debate. We present the first case-report in literature of multiple metachronous nasal cavity, scalp and encephalic metastases of a pleomorphic adenoma of the parotid gland in a young girl.

  9. Immunohistochemical patterns in different stromal variants of pleomorphic adenomas: literature review.

    PubMed

    Patrón-Bolaños, Clara; Acosta-Torres, Laura; Tenorio-Rocha, Fernando; Jacinto-Alemán, Luis Fernando; Leyva-Huerta, Elba

    2016-03-01

    Pleomorphic adenoma is the most frequent type of benign salivary neoplasm located in the mouth and is characterized by its significant histopathological diversity. The histogenesis of the pleomorphic adenoma is uncertain; so far several studies suggest that myoepithelial cells are responsible for the variable histomorphology of this type of neoplasm. At times, stroma is the predominant element. The purpose of the present review is to analyze the results reported in the scientific literature concerning immunomarkers expressed in the different stromal elements of pleomorphic adenoma.

  10. Overdiagnosis of high-grade dysplasia in Barrett's esophagus: a multicenter, international study.

    PubMed

    Sangle, Nikhil A; Taylor, Shari L; Emond, Mary J; Depot, Michelle; Overholt, Bergein F; Bronner, Mary P

    2015-06-01

    Numerous histological mimics of high-grade dysplasia in Barrett's esophagus predispose to overdiagnosis and potential serious mismanagement, including unnecessary esophagectomy. This study investigates the prevalence and sources of this problem. Biopsies from 485 patients diagnosed with Barrett's high-grade dysplasia were screened for a multi-institutional, international Barrett's endoscopic ablation trial. Screening included review of the original diagnostic slides and an additional protocol endoscopy with an extensive biopsy sampling. Observer variability by the study pathologists was assessed through two blinded diagnostic rounds on 437 biopsies from 26 random study endoscopies. Study diagnostic reassessments revealed significantly lower rates of high-grade dysplasia. Only 248 patients (51%) were confirmed to have high-grade dysplasia. The remaining patients had inflamed gastric cardia without Barrett's (n=18; 7%), Barrett's without dysplasia (n=35; 15%), indefinite change (n=61; 26%), low-grade dysplasia (n=79; 33%), adenocarcinoma (n=43; 18%), and other (n=1; <1%), yielding an alarming total of 194 or 40% of patients who were overdiagnosed with Barrett's high-grade dysplasia. Study pathologists achieved a high-level agreement (90% three-way inter-observer agreement per biopsy, Kappa value 0.77) for high-grade dysplasia. Confounding factors promoting overdiagnosis included Barrett's inflammatory atypia (n=182), atypia limited to the basal metaplastic glands (n=147), imprecise criteria for low grade neoplasia (n=102), tangential sectioning artifact (n=59), and reactive gastric cardiac mucosa (n=38). A total of 194 patients (40%) were overdiagnosed with Barrett's high-grade dysplasia, as affirmed by the extensive screening process and high-level study pathologist agreement. The multiple diagnostic pitfalls uncovered should help raise pathologists' awareness of this problem and improve diagnostic accuracy.

  11. Diagnosis and Management of Hereditary Sarcoma.

    PubMed

    Thomas, David M; Ballinger, Mandy L

    2016-01-01

    Sarcomas are rare and heterogeneous diseases that affect a younger population than most epithelial cancers. Epidemiologic studies suggest a strong genetic component to sarcomas, and many familial cancer syndromes have been described, in which sarcomas are a feature. The best known of these are the Li-Fraumeni and retinoblastoma syndromes, study of which has been pivotal to elucidating the molecular basis for the cell response to DNA damage and the cell division. Although much has been learnt about cancer biology from the study of sarcoma families, in general clinical management of increased sarcoma risk has lagged behind other cancer predisposition syndromes. With the advent of genomic tools for genetic testing, it is likely that a substantial fraction of sarcoma patients will be identified as carriers of known risk alleles. The translation of this knowledge into effective risk management programs and cancer treatments will be essential to changes in routine clinical practice. PMID:27075354

  12. [Radiation therapy for pleomorphic adenoma of the parotid].

    PubMed

    Jardel, P; Fakhry, N; Makeieff, M; Ferrie, J-C; Milin, S; Righini, C; Lacout, A; Costes, V; Malard, O; Marcy, P-Y; Guevara, N; Odin, G; Bensadoun, R-J; Thariat, J

    2014-01-01

    Parotid pleomorphic adenoma is the most frequent tumor of salivary glands. The prognosis depends on the recurrences because they could lead to iatrogenic events (facial paralysis). Moreover the risk of malignant transformation increases with the number of local relapses. This article aims at reviewing histological and radiological criteria and the surgical techniques. To improve local control, adjuvant irradiation (in first intention or after recurrence) may be useful but is still controversial for benign tumors in young patients with a risk of radio-induced cancer. We listed studies in which adjuvant radiotherapy was used so as to define its place in the treatment strategy. Prognostic factors were found by some authors. Other studies have to be done before strong evidence-based recommendations are issued. PMID:24387927

  13. Giant mitochondria in a pleomorphic adenoma of the submandibular gland.

    PubMed

    Tandler, B; Erlandson, R A

    1983-01-01

    A benign pleomorphic adenoma of the submandibular gland was examined by electron microscopy. In some areas, the epithelial cells comprising the tumor formed ductlike structures surrounding a lumen filled with membrane vesicles. The cells actually abutting the lumens had giant mitochondria measuring up to 8 micrometers in diameter; such enlarged organelles were absent from immediately subjacent cells. The giant mitochondria exhibited a variety of cristal arrangements, the most common being a quasireticulate one. They often contained expanded cristae that enclosed a number of helical filaments. Bundles of 14-nm tubules with faintly discernible axial periodicity were frequently present in the matrix compartment, as were amorphous dense inclusions. The basis for the occurrence of giant mitochondria only in duct cells may reside in microenvironmental factors rather than in altered nuclear or mitochondrial genomes.

  14. Oncocytic myoepithelioma and pleomorphic adenoma of the salivary glands.

    PubMed

    Skálová, A; Michal, M; Ryska, A; Simpson, R H; Kinkor, Z; Walter, J; Leivo, I

    1999-06-01

    Twenty oncocytic myoepitheliomas (MEs) and pleomorphic adenomas (PAs) were composed of interlacing fascicles of swollen spindle-shaped or/and epithelioid oncocytic myoepithelial cells showing intense finely granular immunoreactivity with anti-mitochondrial antibody. Focal vacuolation of the cytoplasm of oncocytic myoepithelial cells and their gradual transition into sebaceous metaplasia were observed in 3 cases. Another unusual feature found in 5 cases was the presence of slit-like adenomatoid spaces lined with double-layered oncocytic myoepithelium closely resembling Warthin's tumour. The nuclei of oncocytic cells were characterized by enlargement, hyperchromasia and polymorphism, which should not be confused with malignancy. Oncocytic change in myoepithelial cells in MEs and PAs can cause pitfalls in the differential diagnosis of salivary gland tumours. We describe some unusual histological features associated with onococytic metaplasia in benign myoepithelial cell-derived salivary gland tumours, hoping to help to avoid the overdiagnosis of malignancy.

  15. Giant presentation of pleomorphic adenoma in major salivary gland.

    PubMed

    Gupta, Manish; Chaudhary, Neena; Gupta, Monica

    2011-01-01

    Pleomorphic adenoma (PA) is the most common benign salivary gland tumour, accounting for as many as 80% of all such tumours. Although PA most commonly occurs in the parotid gland (85% cases), it may involve submandibular (8%), lingual and minor salivary glands. It is a benign tumour with a slow and continuous growth and without treatment can assume enormous dimensions. This paper describes a giant PA's arising in the submandibular gland and treated by complete surgical excision without sequel. In our case, a male patient presented with a tumour history of more than 4 years and weight of the resected mass was 2.24 kg. Giant PA's of the submandibular gland are very rare in medical literature.

  16. Juvenile pleomorphic adenoma of masticator space: The first case report.

    PubMed

    Panigrahi, Rajat G; Sahoo, Sujit Ranjan; Panda, Swagatika; Lenka, Sthitaprajna; Padhiary, Subrat Kumar; Bhuyan, Ruchi; Bhuyan, Sanat

    2013-10-01

    Pleomorphic adenoma (PA), also called benign mixed tumor, is the most common tumor of the salivary glands. About 90% of these tumors occur in the parotid gland and 10% in the minor salivary glands. Juvenile PAs are uncommon and about 5-10% of minor salivary gland PA affects patients aged 20 years and under. The most common sites of PA of the minor salivary glands are the palate followed by lips and cheek. Other rare reported sites include the throat, floor of the mouth, tongue, tonsil, pharynx, retromolar area and nasal cavity. The masticator space is a deep facial space with a complex anatomical structure where PA is not known to occur. Here, we report an unusual case of PA of left masticator space in a 16-year-old girl patient, which to the best of our knowledge is the first reported case in English language literature.

  17. Radiation-induced sarcoma of the thyroid

    SciTech Connect

    Griem, K.L.; Robb, P.K.; Caldarelli, D.D.; Templeton, A.C. )

    1989-08-01

    A 23-year-old white man presented with a thyroid mass 12 years after receiving high-dose radiotherapy for a T2 and N1 lymphoepithelioma of the nasopharynx. Following subtotal thyroidectomy, a histopathologic examination revealed liposarcoma of the thyroid gland. The relationship between sarcomas and irradiation is described and Cahan and colleagues' criteria for radiation-induced sarcomas are reviewed. To our knowledge, we are presenting the first such case of a radiation-induced sarcoma of the thyroid gland.

  18. Prolonged clinical benefit of everolimus therapy in the management of high-grade pancreatic neuroendocrine carcinoma.

    PubMed

    Fonseca, Paula J; Uriol, Esther; Galván, José A; Alvarez, Carlos; Pérez, Quionia; Villanueva, Noemi; Berros, José P; Izquierdo, Marta; Viéitez, José M

    2013-01-01

    Treatment options for patients with high-grade pancreatic neuroendocrine tumors (pNET) are limited, especially for those with progressive disease and for those who experience treatment failure. Everolimus, an oral inhibitor of mammalian target of rapamycin (mTOR), has been approved for the treatment of patients with low- or intermediate-grade advanced pNET. In the randomized phase III RADIANT-3 study in patients with low- or intermediate-grade advanced pNET, everolimus significantly increased progression-free survival (PFS) and decreased the relative risk for disease progression by 65% over placebo. This case report describes a heavily pretreated patient with high-grade pNET and liver and peritoneal metastases who achieved prolonged PFS, clinically relevant partial radiologic tumor response, and resolution of constitutional symptoms with improvement in Karnofsky performance status while receiving a combination of everolimus and octreotide long-acting repeatable (LAR). Radiologic and clinical responses were maintained for 19 months, with minimal toxicity over the course of treatment. This case supports the findings that the combination of everolimus plus octreotide LAR may be considered for use in patients with high-grade pNET and progressive disease. Although behavior and aggressiveness are different between low- or intermediate-grade and high-grade pNET, some high-grade pNET may express mTOR; hence, everolimus should be considered in a clinical trial.

  19. Prolonged Clinical Benefit of Everolimus Therapy in the Management of High-Grade Pancreatic Neuroendocrine Carcinoma

    PubMed Central

    Fonseca, Paula J.; Uriol, Esther; Galván, José A.; Álvarez, Carlos; Pérez, Quionia; Villanueva, Noemi; Berros, José P.; Izquierdo, Marta; Viéitez, José M.

    2013-01-01

    Treatment options for patients with high-grade pancreatic neuroendocrine tumors (pNET) are limited, especially for those with progressive disease and for those who experience treatment failure. Everolimus, an oral inhibitor of mammalian target of rapamycin (mTOR), has been approved for the treatment of patients with low- or intermediate-grade advanced pNET. In the randomized phase III RADIANT-3 study in patients with low- or intermediate-grade advanced pNET, everolimus significantly increased progression-free survival (PFS) and decreased the relative risk for disease progression by 65% over placebo. This case report describes a heavily pretreated patient with high-grade pNET and liver and peritoneal metastases who achieved prolonged PFS, clinically relevant partial radiologic tumor response, and resolution of constitutional symptoms with improvement in Karnofsky performance status while receiving a combination of everolimus and octreotide long-acting repeatable (LAR). Radiologic and clinical responses were maintained for 19 months, with minimal toxicity over the course of treatment. This case supports the findings that the combination of everolimus plus octreotide LAR may be considered for use in patients with high-grade pNET and progressive disease. Although behavior and aggressiveness are different between low- or intermediate-grade and high-grade pNET, some high-grade pNET may express mTOR; hence, everolimus should be considered in a clinical trial. PMID:24019785

  20. [Breast hematoma masking a sarcoma].

    PubMed

    Relea Sarabia, A; Bahamonde Cabria, S; González Rebollo, M; Encinas Gaspar, M B

    2015-01-01

    Sarcomas account for less than 1% of malignant breast tumors. We present the case of a sarcoma (malignant fibrous histiocytoma) of the breast that debuted as a lump. The patient associated the lump with trauma, and the clinical, sonographic, and cytological findings were suggestive of a hematoma. The lump grew rapidly, and the follow-up sonogram one month later clearly revealed hypervascular solid poles. This atypical presentation is useful for reviewing the management of lesions suggestive of hematomas, which are often associated with nonspecific findings and occasionally with signs that raise suspicion of malignancy. The emergence of what appears to be a hematoma in the breast, even when accompanied by a history of trauma or a tendency toward bleeding, calls for prudence: very short-term follow-up and biopsy, even excisional biopsy, are recommended if the lesion does not evolve like a hematoma. PMID:26160042

  1. Ewing's sarcoma of proximal humeral epiphysis.

    PubMed

    Esmaili, Heydar Ali; Niknejad, Mohammad Taghi; Mohajeri, Shiva

    2015-02-01

    Ewing's sarcoma is one of the most common primary bone tumors of childhood. The tumor is almost always metaphyseal or diaphyseal, within long bones. In children, lesions of the epiphysis are often benign, with the most common diagnosis being chondroblastoma. Rarely, 1%-2% of Ewing sarcomas may involve epiphysis. We present a case of Ewing's sarcoma of the proximal humeral epiphysis in a 13-year-old boy. This case adds to previously reported cases of epiphyseal Ewing sarcoma and suggests that the diagnosis should be considered for pediatric epiphyseal lesions. PMID:25644805

  2. Ewing sarcoma: a chronicle of molecular pathogenesis.

    PubMed

    Kim, Sang Kyum; Park, Yong-Koo

    2016-09-01

    Sarcomas have traditionally been classified according to their chromosomal alterations regardless of whether they accompany simple or complex genetic changes. Ewing sarcoma, a classic small round cell bone tumor, is a well-known mesenchymal malignancy that results from simple sarcoma-specific genetic alterations. The genetic alterations are translocations between genes of the TET/FET family (TLS/FUS, EWSR1, and TAF15) and genes of the E26 transformation-specific (ETS) family. In this review, we intend to summarize a chronicle of molecular findings of Ewing sarcoma including recent advances and explain resultant molecular pathogenesis. PMID:27246176

  3. Primary pleuropulmonary synovial sarcoma: a case report.

    PubMed

    Yuan, Lianfang; Guan, Zhiyu; Dai, Xuan; Xu, Jie

    2015-01-01

    Pleuropulmonary synovial sarcoma (PPSS) is an extremely rare malignant tumor, which is increasingly recognized as a subtype of sarcoma with a distinctive chromosomal translocation specific to synovial sarcoma. It is often presents like any thoracic tumor with symptoms such as chest pain or cough. Here we report a case of PPSS in a 49-year-old woman presenting with cough, shortness of breath and chest pain. And who were found upon histologic examination of the resection specimen to have cystic primary pleuropulmonary synovial sarcoma. PMID:26823907

  4. [Molecular biology for sarcoma: useful or necessary?].

    PubMed

    Neuville, Agnès; Coindre, Jean-Michel; Chibon, Frédéric

    2015-01-01

    Sarcomas are a heterogeneous group of tumors. Their diagnosis is based on morphology and immunohistochemical profile, with categories of tumors according to the type of tissue that they resemble. Nevertheless, for several tumors, cellular origin is unknown. Molecular analysis performed in recent years allowed, combining histophenotype and genomics, better classifying such sarcomas, individualizing new entities and grouping some tumors. Simple and recurrent genetic alterations, such as translocation, mutation, amplification, can be identified in one of two sarcomas and appear as new diagnostic markers. Their identification in specialized laboratories in molecular pathology of sarcomas is often useful and sometimes necessary for a good diagnosis, leading to a heavy and multidisciplinary multi-step treatment.

  5. Synovial Sarcoma Mimicking Myositis Ossificans

    PubMed Central

    Erkut, Adem; Guvercin, Yılmaz; Bedir, Recep

    2016-01-01

    A calcification mass was incidentally found in the soft tissue of a patient who had a history of trauma to the extremity during examination. The patient had no symptom. The pathological analysis of the mass revealed it was an early-phase synovial sarcoma (SS). The diagnosis was made before the onset of symptoms and proper surgical intervention was performed. Therefore, in case of a <1 cm lesion clinically suspicious of myositis ossificans, SS should be taken into consideration as a possible diagnosis.

  6. Carcinoma ex pleomorphic adenoma originating from ectopic salivary gland in the neck region: case report.

    PubMed

    Ismı, Onur; Vayısoğlu, Yusuf; Arpaci, Rabia Bozdogan; Eti, Can; Pütürgeli, Tuğçe; Gorur, Kemal; Ozcan, Cengiz

    2015-12-01

    Carcinoma ex pleomorphic adenoma (CEPA) is the malignant salivary gland tumor originating from primary (de novo) or recurrent pleomorphic adenoma. Although parotid gland is the most common site, it can also be seen in submandibular gland or minor salivary glands. It can be seen rarely in head and neck region, such as oral cavity, trachea, nasal cavity and lacrimal gland. Although pleomorphic adenoma cases originating from ectopic salivary gland tissue in the neck region are present in the English literature, there is no published pleomorphic adenoma ex carcinoma case. In this case report we presented a CEPA as a 7.5 cm long neck mass in a 72-year-old woman originating from the submandibular region apart from submandibular gland. Difficulties in diagnosis and way to appropriate treatment are discussed with current literature.

  7. Carcinoma ex pleomorphic adenoma originating from ectopic salivary gland in the neck region: case report

    PubMed Central

    Vayısoğlu, Yusuf; Arpaci, Rabia Bozdogan; Eti, Can; Pütürgeli, Tuğçe; Gorur, Kemal; Ozcan, Cengiz

    2015-01-01

    Carcinoma ex pleomorphic adenoma (CEPA) is the malignant salivary gland tumor originating from primary (de novo) or recurrent pleomorphic adenoma. Although parotid gland is the most common site, it can also be seen in submandibular gland or minor salivary glands. It can be seen rarely in head and neck region, such as oral cavity, trachea, nasal cavity and lacrimal gland. Although pleomorphic adenoma cases originating from ectopic salivary gland tissue in the neck region are present in the English literature, there is no published pleomorphic adenoma ex carcinoma case. In this case report we presented a CEPA as a 7.5 cm long neck mass in a 72-year-old woman originating from the submandibular region apart from submandibular gland. Difficulties in diagnosis and way to appropriate treatment are discussed with current literature. PMID:26645012

  8. [Pleo-morphic adenoma of the salivary glands: clinico-pathological study of 175 cases].

    PubMed

    García-Pola Vallejo, M J; Bagán Sebastián, J V; García Martín, J M; López Arranz, J S

    1990-10-01

    In this report we analyzed 175 benign pleomorphic adenomas from the salivary glands. We described epidemiologic, clinical aspects and light microscopic features. We connected the sized, evolution time and their microscopically findings.

  9. Schwannoma-like pleomorphic adenoma: a case report with review of the literature.

    PubMed

    Lombardi, Mariangela; Socciarelli, Fabio; Fini, Giuseppina; Leonardi, Alessandra; Bartolazzi, Armando

    2014-06-01

    Pleomorphic adenoma is a common benign salivary gland tumor, which represents about 66 % of benign neoplasms of the salivary glands. Although it can occur in any salivary gland, it is most frequently found in the parotid. Pleomorphic adenomas are renowned for their cytomorphological and architectural heterogeneity that are characterized by intermixed epithelial and mesenchymal-like components. We report a rare case of pleomorphic adenoma of the parotid gland with prevalent schwannoma-like features mimicking a benign schwannoma. Microscopically the tumor showed a prevalence (about 95 %) of schwannoma-like areas with focal (about 5 %) epithelial component with tubular organization. The tumor showed positive immunoexpression for cytokeratin, S-100 protein, and focal expression of p63, CD10 and smooth muscle actin. To the best of our knowledge only six cases of schwannoma-like pleomorphic adenoma have been reported in the literature. The differential diagnosis between this entity and neurogenic and myogenic tumors is discussed.

  10. Malignant myoepithelioma arising from recurrent pleomorphic adenoma of minor salivary gland.

    PubMed

    Yoshizaki, Tomokazu; Himi, Yuji; Minato, Hiroshi; Ogawa, Ikuko; Nikai, Hiromasa; Furukawa, Mitsuru

    2002-01-01

    Malignant myoepitheliomas of the salivary gland are very rare tumors which may either arise de novo or develop in a pre-existing pleomorphic adenoma. We report a case of malignant myoepithelioma of the minor salivary gland that progressed from benign pleomorphic adenoma. The original pleomorphic adenoma was resected in 1979 (the '79 tumor). The first recurrent tumor was operated in 1995 (the '95 tumor). The '95 tumor was diagnosed as pleomorphic adenoma. Although the myoepithelial tumor component was more prevalent in the '95 tumor, histological features of the first recurrent tumor were similar to the '79 tumor. The second recurrent tumor showed more aggressive clinical features (the '98 tumor). It also showed massive growth of myoepithelial tumor cells with bone invasion, which led to the diagnosis of the '98 tumor as malignant myoepithelioma. With adequate surgical margin, the patient has been free from tumor recurrence for 20 months.

  11. Low grade mucoepidermoid carcinoma ex pleomorphic adenoma. A diagnostic problem in fine needle aspiration biopsy.

    PubMed

    Jacobs, J C

    1994-01-01

    We report a case of low grade mucoepidermoid carcinoma arising in a pleomorphic adenoma (ex pleomorphic adenoma) in the parotid salivary gland of a 32-year-old woman. Fine needle aspiration biopsy showed the typical biphasic pattern of pleomorphic adenoma: groups of benign-appearing epithelial cells and chondromyxoid stroma. In addition, features of low grade mucoepidermoid carcinoma were identified retrospectively, consisting of background mucin and rare mucin-containing cells. This case illustrates that the presence of background mucin and mucin-containing cells in an otherwise usual pleomorphic adenoma may indicate the presence of a well-differentiated mucoepidermoid carcinoma. In cases such as this, a definitive diagnosis should be postponed until the lesion is examined histologically.

  12. Research and development of intelligent controller for high-grade sanitary ware

    NASA Astrophysics Data System (ADS)

    Bao, Kongjun; Shen, Qingping

    2013-03-01

    With the social and economic development and people's living standards improve, more and more emphasis on modern society, people improve the quality of family life, the use of intelligent controller applications in high-grade sanitary ware physiotherapy students. Analysis of high-grade sanitary ware physiotherapy common functions pointed out in the production and use of the possible risks, proposed implementation of the system hardware and matching, given the system software implementation process. High-grade sanitary ware physiotherapy intelligent controller not only to achieve elegant and beautiful, simple, physical therapy, water power, deodorant, multi-function, intelligent control, to meet the consumers, the high-end sanitary ware market, strong demand, Accelerate the enterprise product Upgrade and improve the competitiveness of enterprises.

  13. Molecular Biology in Pediatric High-Grade Glioma: Impact on Prognosis and Treatment

    PubMed Central

    Rizzo, Daniela; Ruggiero, Antonio; Martini, Maurizio; Rizzo, Valentina; Maurizi, Palma; Riccardi, Riccardo

    2015-01-01

    High-grade gliomas are the main cause of death in children with brain tumours. Despite recent advances in cancer therapy, their prognosis remains poor and the treatment is still challenging. To date, surgery followed by radiotherapy and temozolomide is the standard therapy. However, increasing knowledge of glioma biology is starting to impact drug development towards targeted therapies. The identification of agents directed against molecular targets aims at going beyond the traditional therapeutic approach in order to develop a personalized therapy and improve the outcome of pediatric high-grade gliomas. In this paper, we critically review the literature regarding the genetic abnormalities implicated in the pathogenesis of pediatric malignant gliomas and the current development of molecularly targeted therapies. In particular, we analyse the impact of molecular biology on the prognosis and treatment of pediatric high-grade glioma, comparing it to that of adult gliomas. PMID:26448930

  14. Pleomorphic adenoma of the lacrimal gland in an eleven years old girl.

    PubMed

    Vijayakumar, Ananthalakshmi

    2013-04-01

    We are presenting a case of pleomorphic adenoma of the lacrimal gland in a 11 years old girl. This is a rare presentation. Pleomorphic adenoma is the most common epithelial tumour of the lacrimal gland, which represents 12% of all the lacrimal fossa lesions. It typically presents in middle age and is rare in children who are under the age of sixteen years, with only a few previously reported cases.

  15. Ultrastructure of Candida albicans pleomorphic forms: phase-contrast microscopy, scanning and transmission electron microscopy.

    PubMed

    Staniszewska, Monika; Bondaryk, Małgorzata; Siennicka, Katarzyna; Kurzatkowski, Wiesław

    2012-01-01

    A modified method of glutaraldeyde-osmium tetroxide fixation was adjusted to characterize the ultrastructure of Candida albicans pleomorphic forms, using phase-contrast microscopy, scanning electron microscopy and transmission electron microscopy. The discovered morphological criteria defining the individual morphotypes are discussed in terms of mycological and histopathological diagnostics of candidiasis. The relations are discussed between fungal pleomorphism, virulence and susceptibility of different morphotypes to fungicides.

  16. Benign pleomorphic adenoma of minor salivary gland showing perineural invasion: a rare entity.

    PubMed

    Jayaram, Rahul; Patel, Dipen; Santhanam, Vijay

    2015-01-01

    Perineural invasion is a rare and sporadically reported histological finding in relation to benign lesions. We present a case of a benign pleomorphic adenoma of a minor salivary gland of the cheek, exhibiting perineural involvement. There have been no previously reported cases of minor salivary gland pleomorphic adenomas exhibiting this phenomenon. This is also the first report of this rare feature in surgical literature pertaining to the head and neck region.

  17. Tumoral mass presenting in the nasomalar region arising from the lateral nasal wall: pleomorphic adenoma.

    PubMed

    Uğuz, Mustafa Zafer; Onal, Kazim; Demiray, Utku; Ekinci, Neşe

    2007-11-01

    Pleomorphic adenoma is the most common benign salivary gland tumor. Although the major salivary glands are the most common sites of its origin, it can also occur in the minor salivary glands of the oral cavity and rarely in the neck, ear, nasal cavity and larynx. We report a rare case of intranasal pleomorphic adenoma arising from lateral nasal wall and discuss the clinical presentation, diagnosis and treatment approaches with the review of the literature.

  18. A hepatic metastasis from pleomorphic adenoma of salivary gland: an unusual presentation.

    PubMed

    Singhal, Ashish; Shrago, Stan S; Li, Shi Feng; Huang, Yi; Kohli, Vivek

    2010-01-01

    Metastasizing pleomorphic adenoma (PA) is a rare condition displaying benign histological features as of PA in the metastatic lesion. The present study report a case of 46 years old male presented with space occupying lesion in the right lobe of liver. Patient underwent extended right hepatectomy for space occupying lesion in liver and the histological features were suggestive of metastatic benign pleomorphic adenoma. Surgical resection is the treatment of choice for both primary and metastatic lesion.

  19. Genetic Analysis of Diffuse High-Grade Astrocytomas in Infancy Defines a Novel Molecular Entity.

    PubMed

    Gielen, Gerrit H; Gessi, Marco; Buttarelli, Francesca R; Baldi, Caterina; Hammes, Jennifer; zur Muehlen, Anja; Doerner, Evelyn; Denkhaus, Dorota; Warmuth-Metz, Monika; Giangaspero, Felice; Lauriola, Libero; von Bueren, André O; Kramm, Christof M; Waha, Andreas; Pietsch, Torsten

    2015-07-01

    Pediatric high-grade gliomas are considered to be different when compared to adult high-grade gliomas in their pathogenesis and biological behavior. Recently, common genetic alterations, including mutations in the H3F3A/ATRX/DAXX pathway, have been described in approximately 30% of the pediatric cases. However, only few cases of infant high-grade gliomas have been analyzed so far. We investigated the molecular features of 35 infants with diffuse high-grade astrocytomas, including 8 anaplastic astrocytomas [World Health Organization (WHO) grade III] and 27 glioblastomas (WHO grade IV) by immunohistochemistry, multiplex ligation probe-dependent amplification (MLPA), pyrosequencing of glioma-associated genes and molecular inversion probe (MIP) assay. MIP and MLPA analyses showed that chromosomal alterations are significantly less frequent in infants compared with high-grade gliomas in older children and adults. We only identified H3F3A K27M in 2 of 34 cases (5.9%), with both tumors located in the posterior fossa. PDGFRA amplifications were absent, and CDKN2A loss could be observed only in two cases. Conversely, 1q gain (22.7%) and 6q loss (18.2%) were identified in a subgroup of tumors. Loss of SNORD located on chromosome 14q32 was observed in 27.3% of the infant tumors, a focal copy number change not previously described in gliomas. Our findings indicate that infant high-grade gliomas appear to represent a distinct genetic entity suggesting a different pathogenesis and biological behavior. PMID:25231549

  20. High-grade adenocarcinoma, (ductal type) arising in unilateral Warthin tumor of the parotid gland.

    PubMed

    Deodhar, Kedar K; Shah, Milap; Chaturvedi, Pankaj

    2011-01-01

    Warthin tumor is a well-recognized benign salivary gland neoplasm consisting of an epithelial as well as a lymphoid component. Malignant transformation in Warthin tumor is rare and its reported incidence is up to 1%. The more common types of carcinomas described in Warthin tumor are the squamous and mucoepidermoid types, with high-grade adenocarcinoma being extremely rare. A high-grade adenocarcinoma (ductal type) arising in the Warthin tumor in a 72-year-old man is presented for its rarity and diagnostic difficulties.

  1. A case report of CIC-rearranged undifferentiated small round cell sarcoma in the cerebrum.

    PubMed

    Ito, Mayumi; Ishikawa, Misawo; Kitajima, Masateru; Narita, Jun; Hattori, Shinya; Endo, Otone; Goto, Keisuke

    2016-10-01

    CIC-rearranged undifferentiated small round cell sarcoma (CIC-rearranged USRCS) is a recently established type of Ewing-like small round cell sarcomas, characterized by CIC gene rearrangement, most commonly CIC-DUX4 fusion. This report presents the second case of CIC-rearranged USRCS arising primarily in the cerebrum. A 64-year-old otherwise healthy woman presented with a 1 × 1 cm sized hemorrhagic subcortical tumor in the left temporo-parietal lobe. The tumor repeatedly recurred, and the patient underwent three surgeries, chemotherapy with doxorubicin and ifosfamide, and radiotherapy, as well as gamma knife surgery. Systemic examination revealed no other extracranial masses. Imprint cytology revealed small to moderate-sized round-to-ovoid tumor cells with mild pleomorphism and variations in size and shape. The nuclei contained finely granular chromatin, and some had easily-recognizable nucleoli. The tumor exhibited a mainly cytoplasmic pattern of CD99 immunostaining, rather than a diffuse membranous pattern. The tumor also exhibited diffuse positivity for calretinin and p16, as well as partial positivity for WT1 (nuclear and cytoplasmic staining pattern) and D2-40. FISH assessment showed CIC split signals. In conclusion, CIC-rearranged USRCSs can occur primarily in the cerebrum. It would be impossible to diagnose them through cytology alone, but cytology would be useful to rule out other small round cell brain tumors including gliomas, lymphomas, carcinomas, and germinoma. Immunohistochemical analysis including tests for CD99, calretinin, and WT1 would help to suggest CIC-rearranged USRCSs and distinguish them from Ewing sarcomas. Additionally, immunohistochemistry for p16 might be useful in the diagnosis. Diagn. Cytopathol. 2016;44:828-832. © 2016 Wiley Periodicals, Inc.

  2. A case report of CIC-rearranged undifferentiated small round cell sarcoma in the cerebrum.

    PubMed

    Ito, Mayumi; Ishikawa, Misawo; Kitajima, Masateru; Narita, Jun; Hattori, Shinya; Endo, Otone; Goto, Keisuke

    2016-10-01

    CIC-rearranged undifferentiated small round cell sarcoma (CIC-rearranged USRCS) is a recently established type of Ewing-like small round cell sarcomas, characterized by CIC gene rearrangement, most commonly CIC-DUX4 fusion. This report presents the second case of CIC-rearranged USRCS arising primarily in the cerebrum. A 64-year-old otherwise healthy woman presented with a 1 × 1 cm sized hemorrhagic subcortical tumor in the left temporo-parietal lobe. The tumor repeatedly recurred, and the patient underwent three surgeries, chemotherapy with doxorubicin and ifosfamide, and radiotherapy, as well as gamma knife surgery. Systemic examination revealed no other extracranial masses. Imprint cytology revealed small to moderate-sized round-to-ovoid tumor cells with mild pleomorphism and variations in size and shape. The nuclei contained finely granular chromatin, and some had easily-recognizable nucleoli. The tumor exhibited a mainly cytoplasmic pattern of CD99 immunostaining, rather than a diffuse membranous pattern. The tumor also exhibited diffuse positivity for calretinin and p16, as well as partial positivity for WT1 (nuclear and cytoplasmic staining pattern) and D2-40. FISH assessment showed CIC split signals. In conclusion, CIC-rearranged USRCSs can occur primarily in the cerebrum. It would be impossible to diagnose them through cytology alone, but cytology would be useful to rule out other small round cell brain tumors including gliomas, lymphomas, carcinomas, and germinoma. Immunohistochemical analysis including tests for CD99, calretinin, and WT1 would help to suggest CIC-rearranged USRCSs and distinguish them from Ewing sarcomas. Additionally, immunohistochemistry for p16 might be useful in the diagnosis. Diagn. Cytopathol. 2016;44:828-832. © 2016 Wiley Periodicals, Inc. PMID:27324529

  3. Nonconventional papillary thyroid carcinomas with pleomorphic tumor giant cells: a diagnostic pitfall with anaplastic carcinoma.

    PubMed

    Hommell-Fontaine, Juliette; Borda, Angela; Ragage, Florence; Berger, Nicole; Decaussin-Petrucci, Myriam

    2010-06-01

    The presence of pleomorphic tumor giant cells in thyroid carcinomas of follicular cell origin is always worrisome for the pathologist as they first of all refer to anaplastic carcinoma, one of the most aggressive human malignancies. However, non-anaplastic pleomorphic giant cells are well described in other thyroid diseases, most often benign. In this paper, we describe four cases of papillary thyroid carcinoma displaying pleomorphic tumor giant cells with features that differ from those of anaplastic carcinoma. Pleomorphic giant cells were admixed with the underlying thyroid carcinoma and constituted from 5% to 25% of the tumor. Cytologically, they had an abundant eosinophilic cytoplasm with large and irregular nuclei. Compared to pleomorphic giant cells of anaplastic carcinoma, they reproduced the growth pattern of the underlying carcinoma, had a low mitotic index without necrosis or inflammation, and were reactive with thyroglobulin and thyroid-specific transcription factor-1 and strongly and diffusely positive for cytokeratin AE1/AE3. After 16-84 months of follow-up, patients are relapse-free and still alive. These cases show that pleomorphic tumor giant cells arising in papillary thyroid carcinomas do not always represent dedifferentiation and progression to anaplastic carcinoma. Distinction among these processes is critical as their treatment and prognosis are very different.

  4. Agreement Among RTOG Sarcoma Radiation Oncologists in Contouring Suspicious Peritumoral Edema for Preoperative Radiation Therapy of Soft Tissue Sarcoma of the Extremity

    SciTech Connect

    Bahig, Houda; Roberge, David; Bosch, Walter; Levin, William; Petersen, Ivy; Haddock, Michael; Freeman, Carolyn; DeLaney, Thomas F.; Abrams, Ross A.; Indelicato, Danny J.; Baldini, Elizabeth H.; Hitchcock, Ying; Kirsch, David G.; Kozak, Kevin R.; Wolfson, Aaron; and others

    2013-06-01

    Purpose: Peritumoral edema may harbor sarcoma cells. The extent of suspicious edema (SE) included in the treatment volume is subject to clinical judgment, balancing the risk of missing tumor cells with excess toxicity. Our goal was to determine variability in SE delineation by sarcoma radiation oncologists (RO). Methods and Materials: Twelve expert ROs were provided with T1 gadolinium and T2-weighted MR images of 10 patients with high-grade extremity soft-tissue sarcoma. Gross tumor volume, clinical target volume (CTV)3cm (3 cm longitudinal and 1.5 cm radial margin), and CTV2cm (2 cm longitudinal and 1 cm radial margin) were contoured by a single observer. Suspicious peritumoral edema, defined as abnormal signal on T2 images, was independently delineated by all 12 ROs. Contouring agreement was analyzed using the simultaneous truth and performance level estimation (STAPLE) algorithm and kappa statistics. Results: The mean volumes of GTV, CTV2cm, and CTV3cm were, respectively, 130 cm{sup 3} (7-413 cm{sup 3}), 280 cm{sup 3} and 360 cm{sup 3}. The mean consensus volume computed using the STAPLE algorithm at 95% confidence interval was 188 cm{sup 3} (24-565 cm{sup 3}) with a substantial overall agreement corrected for chance (mean kappa = 0.71; range: 0.32-0.87). The minimum, maximum, and mean volume of SE (excluding the GTV) were 4, 182, and 58 cm{sup 3} (representing a median of 29% of the GTV volume). The median volume of SE not included in the CTV2cm and in the CTV3cm was 5 and 0.3 cm{sup 3}, respectively. There were 3 large tumors with >30 cm{sup 3} of SE not included in the CTV3cm volume. Conclusion: Despite the fact that SE would empirically seem to be a more subjective volume, a substantial or near-perfect interobserver agreement was observed in SE delineation in most cases with high-grade soft-tissue sarcomas of the extremity. A median of 97% of the consensus SE is within the CTV2cm (99.8% within the CTV3cm). In a minority of cases, however, significant

  5. Targeting epigenetic misregulation in synovial sarcoma.

    PubMed

    Waterfall, Joshua J; Meltzer, Paul S

    2012-03-20

    Like many sarcomas, synovial sarcoma is driven by a characteristic oncogenic transcription factor fusion, SS18-SSX. In this issue of Cancer Cell, Su et al. elucidate the protein partners necessary for target gene misregulation and demonstrate a direct effect of histone deacetylase inhibitors on the SS18-SSX complex composition, expression misregulation, and apoptosis.

  6. AZD0530 in Treating Patients With Recurrent Locally Advanced or Metastatic Soft Tissue Sarcoma

    ClinicalTrials.gov

    2015-07-02

    Adult Fibrosarcoma; Adult Leiomyosarcoma; Adult Liposarcoma; Adult Malignant Fibrous Histiocytoma; Adult Rhabdomyosarcoma; Dermatofibrosarcoma Protuberans; Endometrial Stromal Sarcoma; Recurrent Adult Soft Tissue Sarcoma; Recurrent Uterine Sarcoma; Stage III Adult Soft Tissue Sarcoma; Stage III Uterine Sarcoma; Stage IV Adult Soft Tissue Sarcoma; Stage IV Uterine Sarcoma; Uterine Carcinosarcoma; Uterine Leiomyosarcoma

  7. Ewing Sarcoma in the Right Ventricle

    PubMed Central

    Zhao, Bihong; Thangam, Manoj; Loyalka, Pranav; Buja, L. Maximilian; Kar, Biswajit; Gregoric, Igor D.

    2016-01-01

    Ewing sarcoma is the second most prevalent malignant primary bone tumor but constitutes only a small proportion of cardiac metastases. We present a case of asymptomatic Ewing sarcoma metastatic to the right ventricle. A 36-year-old man presented for evaluation and resection of a pedunculated right ventricular cardiac tumor. Three years before, he had been diagnosed with translocation-negative Ewing sarcoma, for which he had undergone chemotherapy and amputation of the left leg below the knee. We resected the right ventricular tumor. Analysis of the resected mass supported the diagnosis of metastatic Ewing sarcoma. Postoperative transthoracic echocardiograms showed normal biventricular size and function. One year later, the patient had no recurrence of the sarcoma. In addition to discussing this case, we review the relevant medical literature. PMID:27777536

  8. [Alveolar sarcoma. Report of a case].

    PubMed

    Devisme, L; Mensier, E; Bisiau, S; Bloget, F; Gosselin, B

    1996-01-01

    Alveolar soft part sarcoma occurs mostly in the deep soft tissues. An unusual case of primary pulmonary alveolar soft part sarcoma is reported. A 39-year-old woman presented with thoracic pain revealing the tumor. The left lower lobe was surgically resected. The microscopic features of this tumor, including characteristic alveolar pattern and the PAS-positive crystals were typical of alveolar soft part sarcoma. Immunohistochemically, granular cytoplasmic reactivities were observed with antibodies against vimentin, myoglobin, methionine-enkephalin, S100 protein and neuron-specific-enolase. Electron microscopic study demonstrated numerous crystallized structures in the tumor cell cytoplasm. This is the third case of pulmonary alveolar soft part sarcoma, one arising from the pulmonary vein. The histogenesis of alveolar soft part sarcoma is still debated. Our case does not allow distinction between myogenic or neural origin of this tumor.

  9. Connective tissue growth factor as a novel therapeutic target in high grade serous ovarian cancer

    PubMed Central

    Moran-Jones, Kim; Gloss, Brian S.; Murali, Rajmohan; Chang, David K.; Colvin, Emily K.; Jones, Marc D.; Yuen, Samuel; Howell, Viive M.; Brown, Laura M.; Wong, Carol W.; Spong, Suzanne M.; Scarlett, Christopher J.; Hacker, Neville F.; Ghosh, Sue; Mok, Samuel C.; Birrer, Michael J.; Samimi, Goli

    2015-01-01

    Ovarian cancer is the most common cause of death among women with gynecologic cancer. We examined molecular profiles of fibroblasts from normal ovary and high-grade serous ovarian tumors to identify novel therapeutic targets involved in tumor progression. We identified 2,300 genes that are significantly differentially expressed in tumor-associated fibroblasts. Fibroblast expression of one of these genes, connective tissue growth factor (CTGF), was confirmed by immunohistochemistry. CTGF protein expression in ovarian tumor fibroblasts significantly correlated with gene expression levels. CTGF is a secreted component of the tumor microenvironment and is being pursued as a therapeutic target in pancreatic cancer. We examined its effect in in vitro and ex vivo ovarian cancer models, and examined associations between CTGF expression and clinico-pathologic characteristics in patients. CTGF promotes migration and peritoneal adhesion of ovarian cancer cells. These effects are abrogated by FG-3019, a human monoclonal antibody against CTGF, currently under clinical investigation as a therapeutic agent. Immunohistochemical analyses of high-grade serous ovarian tumors reveal that the highest level of tumor stromal CTGF expression was correlated with the poorest prognosis. Our findings identify CTGF as a promoter of peritoneal adhesion, likely to mediate metastasis, and a potential therapeutic target in high-grade serous ovarian cancer. These results warrant further studies into the therapeutic efficacy of FG-3019 in high-grade serous ovarian cancer. PMID:26575166

  10. Connective tissue growth factor as a novel therapeutic target in high grade serous ovarian cancer.

    PubMed

    Moran-Jones, Kim; Gloss, Brian S; Murali, Rajmohan; Chang, David K; Colvin, Emily K; Jones, Marc D; Yuen, Samuel; Howell, Viive M; Brown, Laura M; Wong, Carol W; Spong, Suzanne M; Scarlett, Christopher J; Hacker, Neville F; Ghosh, Sue; Mok, Samuel C; Birrer, Michael J; Samimi, Goli

    2015-12-29

    Ovarian cancer is the most common cause of death among women with gynecologic cancer. We examined molecular profiles of fibroblasts from normal ovary and high-grade serous ovarian tumors to identify novel therapeutic targets involved in tumor progression. We identified 2,300 genes that are significantly differentially expressed in tumor-associated fibroblasts. Fibroblast expression of one of these genes, connective tissue growth factor (CTGF), was confirmed by immunohistochemistry. CTGF protein expression in ovarian tumor fibroblasts significantly correlated with gene expression levels. CTGF is a secreted component of the tumor microenvironment and is being pursued as a therapeutic target in pancreatic cancer. We examined its effect in in vitro and ex vivo ovarian cancer models, and examined associations between CTGF expression and clinico-pathologic characteristics in patients. CTGF promotes migration and peritoneal adhesion of ovarian cancer cells. These effects are abrogated by FG-3019, a human monoclonal antibody against CTGF, currently under clinical investigation as a therapeutic agent. Immunohistochemical analyses of high-grade serous ovarian tumors reveal that the highest level of tumor stromal CTGF expression was correlated with the poorest prognosis. Our findings identify CTGF as a promoter of peritoneal adhesion, likely to mediate metastasis, and a potential therapeutic target in high-grade serous ovarian cancer. These results warrant further studies into the therapeutic efficacy of FG-3019 in high-grade serous ovarian cancer.

  11. Kaposi sarcoma in unusual locations

    PubMed Central

    Pantanowitz, Liron; Dezube, Bruce J

    2008-01-01

    Kaposi sarcoma (KS) is a multifocal, vascular lesion of low-grade malignant potential that presents most frequently in mucocutaneous sites. KS also commonly involves lymph nodes and visceral organs. This article deals with the manifestation of KS in unusual anatomic regions. Unusual locations of KS involvement include the musculoskeletal system, central and peripheral nervous system, larynx, eye, major salivary glands, endocrine organs, heart, thoracic duct, urinary system and breast. The development of KS within wounds and blood clots is also presented. KS in these atypical sites may prove difficult to diagnose, resulting in patient mismanagement. Theories to explain the rarity and development of KS in these unusual sites are discussed. PMID:18605999

  12. Synovial Sarcoma Mimicking Myositis Ossificans

    PubMed Central

    Erkut, Adem; Guvercin, Yılmaz; Bedir, Recep

    2016-01-01

    A calcification mass was incidentally found in the soft tissue of a patient who had a history of trauma to the extremity during examination. The patient had no symptom. The pathological analysis of the mass revealed it was an early-phase synovial sarcoma (SS). The diagnosis was made before the onset of symptoms and proper surgical intervention was performed. Therefore, in case of a <1 cm lesion clinically suspicious of myositis ossificans, SS should be taken into consideration as a possible diagnosis. PMID:27595081

  13. Synovial Sarcoma Mimicking Myositis Ossificans.

    PubMed

    Balik, Mehmet Sabri; Erkut, Adem; Guvercin, Yılmaz; Bedir, Recep

    2016-09-01

    A calcification mass was incidentally found in the soft tissue of a patient who had a history of trauma to the extremity during examination. The patient had no symptom. The pathological analysis of the mass revealed it was an early-phase synovial sarcoma (SS). The diagnosis was made before the onset of symptoms and proper surgical intervention was performed. Therefore, in case of a <1 cm lesion clinically suspicious of myositis ossificans, SS should be taken into consideration as a possible diagnosis. PMID:27595081

  14. Therapeutic Trial for Patients With Ewing Sarcoma Family of Tumor and Desmoplastic Small Round Cell Tumors

    ClinicalTrials.gov

    2015-12-01

    Desmoplastic Small Round Cell Tumor; Ewing Sarcoma of Bone or Soft Tissue; Localized Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor

  15. Reporting a Rare Case of Pleomorphic Adenoma of the Breast

    PubMed Central

    Khamechian, Tahere; Alizargar, Javad; Mazoochi, Tahere

    2014-01-01

    Pleomorphic adenoma (PA) is the most common tumor type in the salivary gland. PA is uncommon in the breast tissue. Only 73 cases of PA of the breast have been reported in the world literature. We are reporting the 74th case of PA of the breast. A 61-year-old woman was referred to Shahid Beheshti Hospital Obstetric Clinic with bloody painless discharge from the right nipple. A bean size mass was detected immediately below the right nipple. After an excisional biopsy, the pathologist found proliferation in epithelial and myoepithelial cells that had small and multiple nuclei, myxoid and chondroid stroma. Immunohistochemistry stain was positive for S-100 and patchy for GFAP in tumor cells and for SMA around the tubule-glandular and tumor cell aggregates and suggested PA of the breast. It is essential for the pathologists to consider PA of the breast as a differential diagnosis of a rounded circumscribed mass in the juxta-areolar areas. Careful paraffin sections should be performed to avoid an unnecessary mastectomy. PMID:24899901

  16. Notch as a Possible Cell Differentiation Factor in Pleomorphic Adenomas

    PubMed Central

    Takamine, Keisuke; Ueda, Yukiko; Nakano, Keisuke; Ochiai, Takanaga; Sugita, Yoshihiko; Kubo, Katsutoshi; Maeda, Hatsuhiko; Hasegawa, Hiromasa; Kawakami, Toshiyuki

    2015-01-01

    The expression of Notch in 30 cases of pleomorphic adenoma was examined by immunohistochemistry. Comparing the results of our study with previous literatures, from the partial CK7 expression and substantial Notch expression in ductal epithelial cells as well as the Notch expression in solid tumor nests, it can be inferred that Notch is involved in cell differentiation. CK13 expression was observed in cells undergoing squamous metaplasia and Notch expression was seen in the nucleus of basal and squamous cells. The intense Notch expression in basal cells and weak expression in squamous cells suggests that Notch is involved in the differentiation from basal to squamous cell. Moreover, the loss of nuclear expression on the inner layer would signify that differentiation is about to end or has been terminated. Notch was expressed in the cytoplasm of cartilage cells and in the cell membrane of mucous cells but not in the nucleus indicating that differentiation has been concluded. Notch involvement is suspected in cell differentiation in areas showing ductal structures and squamous metaplasia. In summary, Notch is involved in cell differentiation of ductal cells in PA. Nuclear expression was shown in tumor cells in solid nests and surrounding structures. Moreover, Notch is expressed by basal cells undergoing squamous metaplasia suggesting the participation of Notch in cell differentiation in PA. PMID:26516303

  17. Atypical Presentation of an Upper Lip Pleomorphic Adenoma: Case Report

    PubMed Central

    Chatzichalepli, Chara; Cocos, Alina; Kleftogiannis, Manthos; Zarakas, Marissa; Chrysomali, Evanthia

    2014-01-01

    The pleomorphic adenoma (PA) or mixed tumor is the most common neoplasm of the salivary glands, usually presenting with a non-specific clinical manifestation and a diverse histopathological pattern. The region of the lips is the second most common site for minor gland neoplasms. The aim of this paper is to report the case of a 39 year old caucasian woman presenting with a swelling on the right side of the upper lip combined with a history of trauma in the region of the upper right central incisor, eight years ago. The swelling was attributed to the periapical lesion of the upper right central incisor that was observed on the orthopantomography. Intraoperatively the surgeon came upon a nodule of firm consistency in the mucolabial fold. The histopathologic diagnosis of this lesion was benign mixed tumor of salivary gland. This report discusses the deviation in frequency of mixed salivary gland tumor between upper and lower lip, the clinical differential diagnosis, the histopathological pattern and the appropriate treatment.

  18. Giant parotid pleomorphic adenoma in a Nigerian male.

    PubMed

    Akintububo, O B; Ogundipe, O K; Kaltungo, Z Y; Guduf, M I; Pindiga, U H; Abdullahi, Y M

    2016-01-01

    Pleomorphic adenoma (PA) is the most common benign salivary gland tumor, accounting for up to 60% of all tumors of the salivary gland and 75-80% of all parotid tumors. It has a slow but progressive growth pattern and if untreated can become greatly enlarged in size. However, giant PA is uncommon. We report a case of giant parotid PA of more than 10 years duration in a 60-year-old male Nigerian. A 60-year-old male patient with 10-year history of painless, massive, and pedunculated swelling in the left side of lower face measuring 25 cm × 23 cm × 17 cm in dimension. Computerized tomographic scan and percutaneous fine needle aspiration cytology returned a preliminary diagnosis of PA. Superficial parotidectomy with facial nerve preservation via the transcervical approach was performed. The excised mass weighed 5.5 kg while the postoperative healing was satisfactory. Benign PAs may attain a giant size if left untreated. Socioeconomic problems are some of the reasons for late presentation. Adequate excision of the tumor sparing the facial nerve is possible but intraoperative and reactionary hemorrhage are likely complications. PMID:27538561

  19. Giant parotid pleomorphic adenoma in a Nigerian male.

    PubMed

    Akintububo, O B; Ogundipe, O K; Kaltungo, Z Y; Guduf, M I; Pindiga, U H; Abdullahi, Y M

    2016-01-01

    Pleomorphic adenoma (PA) is the most common benign salivary gland tumor, accounting for up to 60% of all tumors of the salivary gland and 75-80% of all parotid tumors. It has a slow but progressive growth pattern and if untreated can become greatly enlarged in size. However, giant PA is uncommon. We report a case of giant parotid PA of more than 10 years duration in a 60-year-old male Nigerian. A 60-year-old male patient with 10-year history of painless, massive, and pedunculated swelling in the left side of lower face measuring 25 cm × 23 cm × 17 cm in dimension. Computerized tomographic scan and percutaneous fine needle aspiration cytology returned a preliminary diagnosis of PA. Superficial parotidectomy with facial nerve preservation via the transcervical approach was performed. The excised mass weighed 5.5 kg while the postoperative healing was satisfactory. Benign PAs may attain a giant size if left untreated. Socioeconomic problems are some of the reasons for late presentation. Adequate excision of the tumor sparing the facial nerve is possible but intraoperative and reactionary hemorrhage are likely complications.

  20. Multiple Ewing Sarcoma/Primitive Neuroectodermal Tumors in the Mediastinum

    PubMed Central

    Bae, Sung Hwan; Hwang, Jung Hwa; Da Nam, Bo; Kim, Hyun Jo; Kim, Ki-Up; Kim, Dong Won; Choi, In Ho

    2016-01-01

    Abstract Ewing sarcoma/primitive neuroectodermal tumors (ES/PNET) are high-grade malignant neoplasms. These malignancies present very rare tumors of thoracopulmonary area and even rarer in the mediastinum. In our knowledge, ES/PNET presented with multiple mediastinal masses has not been reported previously. We experienced a case of a 42-year-old man presented with gradual onset of left-side pleuritic chest pain. A contrast-enhanced chest computed tomography (CT) scan showed separate 2 large heterogeneously enhancing masses in each anterior and middle mediastinum of the left hemithorax. Positron emission tomography-computed tomography (PET-CT) scan revealed high fluorodeoxyglucose (FDG) uptake in the mediastinal masses. After surgical excision for the mediastinal masses, both of the masses were diagnosed as the ES/PNET group of tumors on the histopathologic examination. The patient refused postoperative adjuvant chemotherapy and came back with local tumor recurrence and distant metastasis on 4-month follow-up after surgical resection. We report this uncommon form of ES/PNET. We are to raise awareness that this rare malignancy should be considered as a differential diagnosis of the malignant mediastinal tumors and which can be manifested as multiple masses in a patient. Understanding this rare entity of extra-skeletal ES/PNET and characteristic imaging findings can help radiologists and clinicians to approach proper diagnosis and better management for this highly malignant tumor. PMID:26886614

  1. Body mass index, serum total cholesterol, and risk of gastric high-grade dysplasia

    PubMed Central

    Huang, Ya-Kai; Kang, Wei-Ming; Ma, Zhi-Qiang; Liu, Yu-Qin; Zhou, Li; Yu, Jian-Chun

    2016-01-01

    Abstract Obesity is related to an increased risk of gastric cardia cancer. However, the influences of excess body weight and serum total cholesterol on the risk of gastric high-grade dysplasia have not been fully characterized. A case–control study was conducted to explore the relationships between body mass index (BMI), serum total cholesterol level, and the risk of gastric high-grade dysplasia in Chinese adults. A total of 893 consecutive patients with gastric high-grade dysplasia (537 men and 356 women) and 902 controls (543 men and 359 women) were enrolled from January 2000 to October 2015. Odds ratios (ORs) with 95% confidence intervals (CIs) were estimated, and a multivariate analysis was conducted. After adjusting for age, alcohol consumption, smoking status, family history of gastric cancer or esophageal cancer, and serum total cholesterol level, a BMI ranging from 27.5 to 29.9 was significantly related to an increased risk of gastric high-grade dysplasia in both men (adjusted OR = 1.87, 95% CI = 1.24–2.81) and women (adjusted OR = 2.72, 95% CI = 1.44–5.16). The 2 highest BMI categories (27.5–29.9 and ≥30.0) were identified as risk factors for gastric cardia high-grade dysplasia in both men (BMI = 27.5–29.9: adjusted OR = 1.78, 95% CI = 1.02–3.10; BMI ≥ 30.0: adjusted OR = 2.54, 95% CI = 1.27–5.08) and women (BMI = 27.5–29.9: adjusted OR = 2.88, 95% CI = 1.27–6.55; BMI ≥ 30.0: adjusted OR = 2.77, 95% CI = 1.36–5.64), whereas only a BMI ranging from 27.5 to 29.9 was a risk factor for gastric noncardia high-grade dysplasia in both men (adjusted OR = 1.98, 95% CI = 1.25–3.14) and women (adjusted OR = 2.88, 95% CI = 1.43–5.81). In addition, higher serum total cholesterol was associated with an increased risk of gastric noncardia high-grade dysplasia (adjusted OR = 1.83, 95% CI = 1.25–2.69) in women. Increased BMI was associated with an increased risk

  2. Uterine sarcomas: clinical presentation and MRI features

    PubMed Central

    Santos, Pedro; Cunha, Teresa Margarida

    2015-01-01

    Uterine sarcomas are a rare heterogeneous group of tumors of mesenchymal origin, accounting for approximately 8% of uterine malignancies. They comprise leiomyosarcoma, endometrial stromal sarcoma, undifferentiated endometrial sarcoma, and adenosarcoma. Compared with the more common endometrial carcinomas, uterine sarcomas behave more aggressively and are associated with a poorer prognosis. Due to their distinct clinical and biological behavior, the International Federation of Gynecology and Obstetrics introduced a new staging system for uterine sarcomas in 2009, categorizing uterine carcinosarcoma as a variant of endometrial carcinoma, rather than a pure sarcoma. Magnetic resonance imaging (MRI) has a developing role in the assessment of these malignancies. Features such as tumor localization, irregular or nodular margins, necrosis, rapid growth, intense contrast enhancement, and restriction at diffusion-weighted imaging can suggest the diagnosis and help differentiate from more common leiomyomas and endometrial carcinoma. MRI is therefore extremely useful in preoperative detection and staging and, consequently, in determination of appropriate management. This pictorial review aims to discuss the clinical features of uterine sarcomas, as well as their most common appearances and distinct characteristics in MRI. PMID:25347940

  3. Atypical pleomorphic neoplasms of the pineal gland: Case report and review of the literature

    PubMed Central

    Praver, M.; D’Amico, R.; Arraez, C.; Zacharia, B. E.; Varma, H.; Goldman, J. E.; Bruce, J. N.; Canoll, P.

    2015-01-01

    Background: Pineal region tumors are rare and diverse. Among them exist reports of pleomorphic xanthroastrocytoma (PXA) and pleomorphic granular cell astrocytoma (PGCA) of the pineal gland. These related tumors are remarkably similar sharing pleomorphic histologic features with only minor immunohistochemical and ultrastructural differences. Case Description: We present a case of a 42-year old right-handed woman presented with a longstanding history of migraine headaches which had worsened over the two months leading up to her hospitalization. MRI revealed a 1.7 × 1.3 × 1.6 cm intensely enhancing lesion originating in the pineal gland. The tumor closely resembled PGCA but did not strictly fit the diagnostic requirements of either PGCA or PXA. Conclusion: The present case highlights the exotic nature of pineal region tumors with pleomorphic cell histology. Given the diverse range of tumors encountered in the pineal region, pathological confirmation is mandatory. Favorable clinical outcomes demonstrate that surgical resection alone can yield excellent long-term results for tumors falling within the spectrum of pleomorphic lesions of the pineal gland. PMID:26257987

  4. Sustained response of carcinoma ex pleomorphic adenoma treated with trastuzumab and capecitabine

    PubMed Central

    2010-01-01

    Background Carcinoma ex pleomorphic adenoma is a rare histologic subtype of salivary gland cancer with an overall poor prognosis. Limited histopathologic analyses have shown that some such tumors exhibit significant HER2/neu immunoreactivity, suggesting a potential role for HER2-based therapy. We report here a case of a 58-year old man with metastatic carcinoma ex pleomorphic adenoma who achieved a sustained long term response to combination therapy with trastuzumab and capecitabine. Case presentation A 58 year old man presented with T1N2bM0 carcinoma ex pleomorphic adenoma and underwent surgery followed by adjuvant radiation therapy. Multiple metastases to bone were documented one year later. Since the original tumor was strongly HER2/neu positive by immunohistochemistry, the patient was treated with trastuzumab, capecitabine, and zoledronic acid. He experienced total resolution of symptoms and repeat FDG-PET scan after three cycles revealed interval disease resolution. Continued treatment has resulted in maintenance of disease control for over 2 years. Conclusion This case illustrates the successful long term treatment of carcinoma ex pleomorphic adenoma with targeted therapy with trastuzumab in combination with chemotherapy. In the absence of definitive clinical trials which are unlikely to be performed due to the rarity of this tumor, case reports such as this one suggest potential utility for trastuzumab in combination with chemotherapy in the treatment of HER2/neu-overexpressing carcinoma ex pleomorphic adenoma. PMID:20504363

  5. Atypical features in pleomorphic adenoma--a clinicopathologic study and implications for management.

    PubMed

    Ethunandan, M; Witton, R; Hoffman, G; Spedding, A; Brennan, P A

    2006-07-01

    Pleomorphic adenoma is the most common salivary gland neoplasm and infrequently undergoes malignant transformation. Carcinoma ex pleomorphic adenoma is typically an infiltrative neoplasm with features of cellular pleomorphism, high mitotic activity, peri-neural and vascular invasion. More recently, sub-groups of pleomorphic adenoma have been described exhibiting vascular invasion and features of malignancy without evidence of extra-capsular extension. There is little information in the literature regarding how these different histological variants influence clinical presentation and outcome following primary treatment. Following a review of 100 consecutive pleomorphic adenomas removed from the major salivary glands, 4 cases with atypical histological features were found. Three tumours exhibited features of dysplasia/carcinoma without evidence of extra-capsular invasion and a further case demonstrated benign vascular invasion. There were no clinical features suggestive of the atypical nature of these neoplasms, though fine needle aspiration cytology (FNAC) was suspicious of a malignancy in 2 cases and CT scan in 1 case. Patients underwent a superficial parotidectomy or submandibular gland excision based on the location of the lesion. All lesions were completely excised and there were no recurrences in this series.

  6. [Some problems of clinical characteristics, diagnostics and treatment of pleomorphic adenoma of salivary glands].

    PubMed

    Stojnev, I

    1989-01-01

    The author evaluates the clinical characteristics in the light of the concepts proceeding from the International histological classification of tumours of the salivary glands on the base of his own experience in the treatment of 208 patients with pleomorphic adenoma of the salivary glands. The existing hospital documentation of the treated patients was analyzed as well as a repeated evaluation of the histological diagnosis made during the treatment and control of the therapeutic results via periodical inquiry of the treated patients. The author confirms the ratified unified nomenclature of the International classification and the benign nature of the pleomorphic adenoma. In that aspect he defends the surgical treatment of the pleomorphic adenoma as the method of choice and the high diagnostic value of the urgent intraoperative histological study. He advances the thesis for the application of more radical methods in the surgical treatment of recurrent pleomorphic adenoma of the parotid gland. According to the author, the morphological characteristics of the pleomorphic adenoma have no effect on the choice of the surgical methods for the treatment of that tumour.

  7. Therapeutic Angiotensin-(1-7) in Treating Patients With Metastatic Sarcoma That Cannot Be Removed By Surgery

    ClinicalTrials.gov

    2013-12-10

    Bone Cancer; Chondrosarcoma; Clear Cell Sarcoma of the Kidney; Metastatic Osteosarcoma; Ovarian Sarcoma; Recurrent Adult Soft Tissue Sarcoma; Recurrent Osteosarcoma; Recurrent Uterine Sarcoma; Stage III Adult Soft Tissue Sarcoma; Stage III Uterine Sarcoma; Stage IV Adult Soft Tissue Sarcoma; Stage IV Uterine Sarcoma

  8. High-grade astrocytoma (Glioblastoma Multiforme) in an Atlantic spotted dolphin (Stenella frontalis).

    PubMed

    Díaz-Delgado, J; Sacchini, S; Suárez-Bonnet, A; Sierra, E; Arbelo, M; Espinosa, A; Rodríguez-Grau Bassas, E; Mompeo, B; Pérez, L; Fernández, A

    2015-01-01

    This report describes the gross, microscopical and immunohistochemical features of a high-grade astrocytoma (glioblastoma multiforme) in an adult male Atlantic spotted dolphin (Stenella frontalis). On necropsy examination, a 5 × 2.5 × 2 cm, poorly demarcated, red, friable and locally expansile mass effaced the thalamus and the left periventricular region and extended to the left lateral ventricle of the brain. Microscopically, the mass consisted of haphazardly arranged bundles and rows of interweaving polygonal to spindle-shaped cells. These often palisaded along serpentine foci of necrosis and were surrounded by prominent vessels. Immunohistochemically, the neoplastic cells expressed glial fibrillary acidic protein, but not vimentin, S100 protein, neuron-specific enolase or neurofilament protein. A diagnosis of high-grade astrocytoma was made and this represents the first description of a glioma in a cetacean species. PMID:25728810

  9. Rare earth element patterns in Archean high-grade metasediments and their tectonic significance

    NASA Technical Reports Server (NTRS)

    Taylor, Stuart Ross; Rudnick, Roberta L.; Mclennan, Scott M.; Eriksson, Kenneth A.

    1986-01-01

    REE data on metasedimentary rocks from two different types of high-grade Archean terrains are presented and analyzed. The value of REEs as indicators of crustal evolution is explained; the three geologic settings (in North America, Southern Africa, and Australia) from which the samples were obtained are described; and the data are presented in extensive tables and graphs and discussed in terms of metamorphic effects, the role of accessory phases, provenance, and tectonic implications (recycling, the previous extent of high-grade terrains, and a model of Archean crustal growth). The diversity of REE patterns in shallow-shelf metasediments is attributed to local provenance, while the Eu-depleted post-Archean patterns are associated with K-rich plutons from small, stable early Archean terrains.

  10. Response Assessment and Magnetic Resonance Imaging Issues for Clinical Trials Involving High-Grade Gliomas.

    PubMed

    Boxerman, Jerrold L; Ellingson, Benjamin M

    2015-06-01

    There exist multiple challenges associated with the current response assessment criteria for high-grade gliomas, including the uncertain role of changes in nonenhancing T2 hyperintensity, and the phenomena of pseudoresponse and pseudoprogression in the setting of antiangiogenic and chemoradiation therapies, respectively. Advanced physiological magnetic resonance imaging (MRI), including diffusion and perfusion (dynamic susceptibility contrast MRI and dynamic contrast-enhanced MRI) sensitive techniques for overcoming response assessment challenges, has been proposed, with their own potential advantages and inherent shortcomings. Measurement variability exists for conventional and advanced MRI techniques, necessitating the standardization of image acquisition parameters in order to establish the utility of these imaging methods in multicenter trials for high-grade gliomas. This review chapter highlights the important features of MRI in clinical brain tumor trials, focusing on the current state of response assessment in brain tumors, advanced imaging techniques that may provide additional value for determining response, and imaging issues to be considered for multicenter trials.

  11. Immunotherapy for high-grade glioma: how to go beyond Phase I/II clinical trials.

    PubMed

    van Gool, Stefaan

    2013-10-01

    Evaluation of: Lasky JL 3rd, Panosyan EH, Plant A et al. Autologous tumor lysate-pulsed dendritic cell immunotherapy for pediatric patients with newly diagnosed or recurrent high-grade gliomas. Anticancer Res. 33, 2047-2056 (2013). Immunotherapy for children and adults with high-grade glioma (HGG) is an emerging innovative treatment approach, which aims at stimulating the body's own immune system against HGG by using autologous dendritic cells pulsed with autologous tumor lysate as a therapeutic vaccine. This is the third report on immunotherapy for HGG in children, bringing additional knowledge and experience to the scientific community. However, at the same time, this and other manuscripts urge for the next step in treatment development.

  12. Malignant transformation of a high-grade osteoblastoma of the petrous apex with subcutaneous metastasis.

    PubMed

    Kraft, Casey T; Morrison, Robert J; Arts, H Alexander

    2016-06-01

    We describe the clinical presentation, management, and pathologic findings in a case of osteosarcoma of the petrous apex with an atypical metastasis to the lower abdominal wall. We retrospectively reviewed the record of a 49-year-old man who was diagnosed with a right petrous apex lesion, which biopsy identified as a high-grade osteoblastoma. After two attempts at en bloc resection were not curative, radiation and chemotherapy were recommended. The patient subsequently developed a cutaneous lower abdominal mass that was diagnosed as an osteosarcoma. Meanwhile, the petrous apex tumor continued to grow despite treatment until the patient died from the burden of disease. Temporal bone osteoblastomas and osteosarcomas are both extremely rare, and they can be difficult to differentiate histologically. Our case illustrates this difficulty and demonstrates the possibility of a high-grade osteoblastoma's malignant conversion to an osteosarcoma. PMID:27304442

  13. Cilengitide in Treating Younger Patients With Recurrent or Progressive High-Grade Glioma That Has Not Responded to Standard Therapy

    ClinicalTrials.gov

    2014-05-05

    Childhood High-grade Cerebellar Astrocytoma; Childhood High-grade Cerebral Astrocytoma; Recurrent Childhood Anaplastic Astrocytoma; Recurrent Childhood Anaplastic Oligoastrocytoma; Recurrent Childhood Anaplastic Oligodendroglioma; Recurrent Childhood Brain Tumor; Recurrent Childhood Cerebellar Astrocytoma; Recurrent Childhood Cerebral Astrocytoma; Recurrent Childhood Glioblastoma; Recurrent Childhood Visual Pathway and Hypothalamic Glioma

  14. Toppling high-grade pulmonary neuroendocrine tumors with a DLL3-targeted trojan horse.

    PubMed

    Dylla, Scott J

    2016-03-01

    Delta-like protein 3 (DLL3) is a novel and tractable tumor-initiating cell-associated target for the antibody-drug conjugate SC16LD6.5 in high-grade pulmonary neuroendocrine tumors. Elevated expression of DLL3, an inhibitor of Notch pathway activation, marks the second recent observation that impairment of Notch receptor signaling may play a critical role in neuroendocrine tumorigenesis. PMID:27308627

  15. Feasibility of Using Bevacizumab With Radiation Therapy and Temozolomide in Newly Diagnosed High-Grade Glioma

    SciTech Connect

    Narayana, Ashwatha Golfinos, John G.; Fischer, Ingeborg; Raza, Shahzad; Kelly, Patrick M.D.; Parker, Erik; Knopp, Edmond A.; Medabalmi, Praveen; Zagzag, David; Eagan, Patricia; Gruber, Michael L.

    2008-10-01

    Introduction: Bevacizumab, a monoclonal antibody against vascular endothelial growth factor (VEGF), has shown promise in the treatment of patients with recurrent high-grade glioma. The purpose of this study is to test the feasibility of using bevacizumab with chemoradiation in the primary management of high-grade glioma. Methods and Materials: Fifteen patients with high-grade glioma were treated with involved field radiation therapy to a dose of 59.4 Gy at 1.8 Gy/fraction with bevacizumab 10 mg/kg on Days 14 and 28 and temozolomide 75 mg/m{sup 2}. Subsequently, bevacizumab 10 mg/kg was continued every 2 weeks with temozolomide 150 mg/m{sup 2} for 12 months. Changes in relative cerebral blood volume, perfusion-permeability index, and tumor volume measurement were measured to assess the therapeutic response. Immunohistochemistry for phosphorylated VEGF receptor 2 (pVEGFR2) was performed. Results: Thirteen patients (86.6%) completed the planned bevacizumab and chemoradiation therapy. Four Grade III/IV nonhematologic toxicities were seen. Radiographic responses were noted in 13 of 14 assessable patients (92.8%). The pVEGFR2 staining was seen in 7 of 8 patients (87.5%) at the time of initial diagnosis. Six patients have experienced relapse, 3 at the primary site and 3 as diffuse disease. One patient showed loss of pVEGFR2 expression at relapse. One-year progression-free survival and overall survival rates were 59.3% and 86.7%, respectively. Conclusion: Use of antiangiogenic therapy with radiation and temozolomide in the primary management of high-grade glioma is feasible. Perfusion imaging with relative cerebral blood volume, perfusion-permeability index, and pVEGFR2 expression may be used as a potential predictor of therapeutic response. Toxicities and patterns of relapse need to be monitored closely.

  16. Specific localization of thallium 201 in human high-grade astrocytoma by microautoradiography.

    PubMed

    Mountz, J M; Raymond, P A; McKeever, P E; Modell, J G; Hood, T W; Barthel, L K; Stafford-Schuck, K A

    1989-07-15

    The ability to accurately distinguish remaining or recurrent high-grade astrocytoma from necrosis or edema following treatment is essential to optimal patient management. Thallium 201 planar gamma-camera imaging has been shown to be helpful in detecting recurrent high-grade astrocytoma; however, due to tissue heterogeneity adjacent to and within tumor, the cellular specificity and quantification of 201Tl uptake are largely unknown. In order to determine which tissues are responsible for the radioisotope uptake, microautoradiographic techniques were used to examine multiple tissue sections from five patients with high-grade astrocytoma. Each patient received 5 mCi of 201Tl i.v. 1 h prior to tumor removal. Additionally, all patients received computerized tomographic and 201Tl planar gamma-camera scans prior to surgery. Following surgery, the excised tissue specimens were tentatively classified by gross pathological examination and then immediately processed for dry mount autoradiography; grain density was determined over regions containing tumor, adjacent and uninvolved brain tissue, necrotic tissue, and background. Highly significant differences were found in grain densities (201Tl uptake) between tumor and uninvolved brain tissue, as well as between uninvolved brain tissue and necrotic tissue; there was no significant difference between background grain density and that in necrotic tissue. Mean grain densities (grains/cm2 +/- 1 SD) across patients were: tumor, 102 +/- 23; adjacent, uninvolved brain tissue, 29 +/- 11; necrotic tissue, 6.2 +/- 1.1; and background, 7.0 +/- 4.1. We conclude that the ability of 201Tl to selectively image high-grade astrocytoma is due to its preferential uptake into tumor cells.

  17. Total enbloc spondylectomy for metastatic high grade spinal tumors: Early results

    PubMed Central

    Patil, Sanganagouda S; Nene, Abhay M

    2016-01-01

    Background: High grade metastatic spinal tumors are most common and are invasive. These patients can succumb to disease progression if not treated timely. Although considered as invasive and morbid, total enbloc spondylectomy (TES) in selected cases has better survival rates. The authors describe the results of TES for high grade metastatic spinal tumors. Materials and Methods: Five patients (four females and one male) underwent TES for solitary metastatic vertebral lesion between November 2012 and January 2014. These patients presented to us with spinal instability, unrelenting severe spinal pain and/or with severe progressive radiculopathy. Average age was 46.2 years (range 39–62 years). After complete investigations, computed tomography scan, magnetic resonance imaging scan and positron emission tomography (PET) scan, it was confirmed that these patients had high grade solitary vertebral metastatic tumor. Results: Average duration of followup was 18 months (range 16–20 months). The average preoperative visual analog scale score of 9.4 (range 9–10) improved to 2 (range 1–4) at last followup. Average blood loss was 1440 mL (range 1000–2000 mL). Average duration of surgery was 198 min (range 180–240 min). Significant pain relief was noticed in each patient in the immediate postoperative period and during followups. These patients attained complete functional activities of daily living with in a month. The imaging showed implants in situ, no recurrence of tumor, and no activity on PET scan at the final followup. Conclusion: The present series shows favorable short term results of TES for solitary, metastatic, high grade vertebral body tumors by a team approach. PMID:27512215

  18. High-grade metamafic blocks in Franciscan mélanges--olistostromal and/or tectonic?

    NASA Astrophysics Data System (ADS)

    Ernst, W. G.

    2013-12-01

    At ~175 Ma, onset of transpressive plate underflow generated an Andean-type arc along the Californian margin. The east-descending oceanic crust was metamorphosed to eclogite, garnet-blueschist, and amphibolite facies high-pressure (HP) assemblages in an inboard subduction zone at ~165-150 Ma. Except for the fragmentary Red Ant blueschists that crop out in the northern Sierran Foothills, most such metamafic rocks apparently were stored in a low-T environment well into mid- and Late Cretaceous time. Many of the high-grade metamafic rocks returned surfaceward transported as HP tectonic blocks in buoyant Franciscan serpentinite or in subducting, circulating, low-density mud-matrix mélange. However, the rare occurrence of high-grade clasts in relatively feebly recrystallized Franciscan metaconglomerates reflects a sedimentary (including olistostromal) deposition for some HP metamafic blocks. Actinolitic rinds partially encircle many high-grade blocks of either tectonic or olistostromal putative origin. These rinds are only very slightly younger than the subduction-zone metamorphism, suggesting that an early stage of metasomatic exchange took place between HP metabasalt and serpentinized harzburgite along the dynamic oceanic crust-mantle hanging-wall junction, probably while the metamafic rocks were sequestered at moderately shallow mantle depths. For HP metabasaltic rocks to have been added to the Cretaceous Franciscan clastic section as olistoliths, they must first have been carried surfaceward as tectonic blocks immersed in a low-density lithology--probably serpentinite diapirs--then eroded and transported into the trench. Whatever their origin, due to widespread post-depositional convergent-margin shearing, the original natures of many such HP metamafic blocks have been obliterated. In any case, these dense, high-grade rocks of latest Jurassic recrystallization age must have been supplied to the Cretaceous Franciscan accretionary prism by a stage of entrainment in a

  19. Predictors of Progression to High-Grade Dysplasia or Adenocarcinoma in Barrett's Esophagus.

    PubMed

    Whitson, Matthew J; Falk, Gary W

    2015-06-01

    The prevalence of esophageal adenocarcinoma is increasing dramatically. Barrett's esophagus remains the most well-established risk factor for the development of esophageal adenocarcinoma. There are multiple clinical, endoscopic, and pathologic factors that increase the risk of neoplastic progression to high-grade dysplasia or esophageal adenocarcinoma in Barrett's esophagus. This article reviews both risk and protective factors for neoplastic progression in patients with Barrett's esophagus.

  20. Ziv-aflibercept in Treating Patients With Locally Advanced, Unresectable, or Metastatic Gynecologic Soft Tissue Sarcoma

    ClinicalTrials.gov

    2015-12-03

    Fallopian Tube Cancer; Female Reproductive Cancer; Ovarian Carcinosarcoma; Ovarian Sarcoma; Recurrent Ovarian Epithelial Cancer; Recurrent Uterine Sarcoma; Stage III Ovarian Epithelial Cancer; Stage III Uterine Sarcoma; Stage IV Ovarian Epithelial Cancer; Stage IV Uterine Sarcoma; Uterine Carcinosarcoma; Uterine Leiomyosarcoma

  1. Patient and caregiver perceptions of communication of prognosis in high grade glioma.

    PubMed

    Lobb, E A; Halkett, G K B; Nowak, A K

    2011-08-01

    This study sought the views of patients and their caregivers on their experience of being diagnosed with high grade glioma. Purposive sampling was used to recruit 19 patients and 21 caregivers from the medical oncology unit of a tertiary hospital. A semi-structured face-to-face interview was conducted. Interviews were audio-taped and transcribed verbatim. Data was analysed based on Grounded Theory and using the constant comparison method. This paper focuses on patient and carer perceptions of the initial communication about the diagnosis of high grade glioma and its prognosis. Themes identified included: (a) shock at hearing the diagnosis; (b) trying to understand and process prognostic information when still in shock; (c) the perception of hope being taken away; (d) individualizing prognostic information; and (e) clinicians' lack of communication skills. This study shows that the first communication of prognosis to patients with high grade glioma and their caregivers requires careful negotiation. It illustrates the inability of individuals to process detailed prognostic information when in a state of initial shock and distress. The importance of balancing honesty with hope in the communication of a poor prognosis is highlighted. We recommend that clinicians seek patient preferences for the amount and type of information they require and that prognostic information be individualized. Detailed discussions of prognosis should only take place with senior medical staff, or advanced trainees who have demonstrated acceptable communication skills.

  2. PARP inhibition sensitizes childhood high grade glioma, medulloblastoma and ependymoma to radiation

    PubMed Central

    van Vuurden, Dannis G.; Hulleman, Esther; Meijer, Olga L.M.; Wedekind, Laurine E.; Kool, Marcel; Witt, Hendrik; Vandertop, Peter W. Peter; Würdinger, Thomas; Noske, David P.; Kaspers, Gertjan J.L.; Cloos, Jacqueline

    2011-01-01

    Poly ADP-ribose polymerase (PARP) is a protein involved in single strand break repair. Recently, PARP inhibitors have shown considerable promise in the treatment of several cancers, both in monotherapy and in combination with cytotoxic agents. Synthetic lethal action of PARP inhibitors has been observed in tumors with mutations in double strand break repair pathways. In addition, PARP inhibition potentially enhances sensitivity of tumor cells to DNA damaging agents, including radiotherapy. Aim of this study is to determine the radiosensitizing properties of the PARP inhibitor Olaparib in childhood medulloblastoma, ependymoma and high grade glioma (HGG). Increased PARP1 expression was observed in medulloblastoma, ependymoma and HGG, as compared to non-neoplastic brain tissue. Pediatric high grade glioma, medulloblastoma and ependymoma gene expression profiling revealed that high PARP1 expression is associated with poor prognosis. Cell growth inhibition assays with Olaparib resulted in differential sensitivity, with IC50 values ranging from 1.4 to 8.4 μM, irrespective of tumor type and PARP1 protein expression. Sensitization to radiation was observed in medulloblastoma, ependymoma and HGG cell lines with subcytotoxic concentrations of Olaparib, which coincided with persistence of double strand breaks. Combining PARP inhibitors with radiotherapy in clinical studies in childhood high grade brain tumors may improve therapeutic outcome. PMID:22184287

  3. Phase I and pharmacokinetic study of COL-3 in patients with recurrent high-grade gliomas.

    PubMed

    Rudek, Michelle A; New, Pamela; Mikkelsen, Tom; Phuphanich, Surasak; Alavi, Jane B; Nabors, Louis B; Piantadosi, Steven; Fisher, Joy D; Grossman, Stuart A

    2011-11-01

    COL-3 is a chemically modified tetracycline that targets multiple aspects of matrix metalloproteinase regulation. This phase I clinical trial was conducted to determine the maximum tolerated dose (MTD) of COL-3 in adults with recurrent high-grade glioma, to describe the effects of enzyme-inducing antiseizure drugs (EIADs) on its pharmacokinetics, and to obtain preliminary evidence of activity. Adults with recurrent high-grade glioma were stratified by EIAD use. COL-3 was given orally daily without interruption until disease progression or treatment-related dose-limiting toxicity (DLT). Three patients in each EIAD group were evaluated at each dose level beginning with 25 mg/m(2)/day and escalated by 25 mg/m(2)/day. Toxicity, response, and pharmacokinetics were assessed. Thirty-three patients were evaluated. The MTD was 75 mg/m(2)/day in the -EIAD patients while one was not determined in +EIAD patients. The common toxicities observed were anemia, ataxia, diarrhea, hypokalemia, CNS hemorrhage, and myalgia. One partial response was observed. -EIAD patients tended to have a higher steady-state trough concentration that was apparent only at the 100 mg/m(2)/day dose level (P = 0.01). This study suggests that: (a) EIAD use does affect the pharmacokinetics of COL-3 at higher doses; and (b) there was not enough suggestion of single-agent activity to warrant further study in recurrent high-grade gliomas.

  4. Reproducibility of histological cell type in high-grade endometrial carcinoma.

    PubMed

    Han, Guangming; Sidhu, Davinder; Duggan, Máire A; Arseneau, Jocelyne; Cesari, Matthew; Clement, Philip B; Ewanowich, Carol A; Kalloger, Steve E; Köbel, Martin

    2013-12-01

    Subclassification of endometrial carcinoma according to histological type shows variable interobserver agreement. The aim of this study was to assess specifically the interobserver agreement of histological type in high-grade endometrial carcinomas, recorded by gynecological pathologists from five academic centers across Canada. In a secondary aim, the agreement of consensus diagnosis with immunohistochemical marker combinations was assessed including six routine (TP53, CDKN2A (p16), ER, PGR, Ki67, and VIM) and six experimental immunohistochemical markers (PTEN, ARID1A, CTNNB1, IGF2BP3, HNF1B, and TFF3). The paired interobserver agreement ranged from κ 0.50 to 0.63 (median 0.58) and the intraobserver agreement from κ 0.49 to 0.67 (median 0.61). Consensus about histological type based on morphological assessment was reached in 72% of high-grade endometrial carcinomas. A seven-marker immunohistochemical panel differentiated FIGO grade 3 endometrioid from serous carcinoma with a 100% concordance rate compared with the consensus diagnosis. More practically, a three-marker panel including TP53, ER, and CDKN2A (p16) can aid in the differential diagnosis of FIGO grade 3 endometrioid from endometrial serous carcinoma. Our study demonstrates that the inter- and intraobserver reproducibility of histological type based on morphology alone are mostly moderate. Ancillary techniques such as immunohistochemical marker panels are likely needed to improve diagnostic reproducibility of histological types within high-grade endometrial carcinomas.

  5. Rare carcinoma ex pleomorphic adenoma of the buccal minor salivary gland causing a therapeutic dilemma.

    PubMed

    Kini, Yogesh; Desai, Chirag; Mahindra, Uma; Kalburge, Jitendra

    2012-04-01

    Carcinoma ex pleomorphic adenoma (CXPA), as a group, constitutes 12% of malignant salivary gland tumors. We present a case of CXPA of the buccal mucosa in a 17-year-old patient. The buccal mass was of a size of 3.0 cm located in the right cheek. Pleomorphic adenoma was the provisional diagnosis. The tumor was excised under local anesthesia. Histopathological evaluation revealed a pre-existing pleomorphic adenoma. However, on magnification, certain areas showed islands of dysplastic epithelial cells' invading the fibrous capsule and CXPA was diagnosed. The patient was recalled and secondary surgery of the site performed. No tumor tissue could be detected in the secondary resection specimen. There is no sign of recurrence since 2 years. PMID:22919226

  6. Fine-needle aspiration cytology of pleomorphic hyalinized angiectatic tumor: A case report.

    PubMed

    Lin, Oscar; Crapanzano, John P

    2005-04-01

    Pleomorphic hyalinized angiectatic tumor (PHAT) of soft parts is a neoplasm characterized by spindle and pleomorphic cells associated with an angiectatic vasculature. We describe the cytological findings of a fine-needle aspiration biopsy (FNAB) from the right medial knee of a 45-yr-old woman. The aspirate material was entirely submitted in Cytolit solution. The specimen was moderately cellular and was comprised of spindle cells in a background of fibrinous material. The cells varied from small, bland spindle cells with a fine chromatin pattern and inconspicuous nucleoli to larger pleomorphic cells with coarser chromatin and occasional intranuclear inclusions. Most of the cells were arranged singly with sporadic small cluster formation with indistinct cell borders. Rare mononuclear inflammatory cells morphologically compatible with mast cells were identified. The differential diagnosis include solitary fibrous tumor (SFT) and ancient schwannoma, which also shows fibrous-like material and spindle cells that may have intranuclear inclusions.

  7. Electrical stimulation treatment for facial palsy after revision pleomorphic adenoma surgery

    PubMed Central

    Goldie, Simon; Sandeman, Jack; Cole, Richard; Dennis, Simon; Swain, Ian

    2016-01-01

    Surgery for pleomorphic adenoma recurrence presents a significant risk of facial nerve damage that can result in facial weakness effecting patients’ ability to communicate, mental health and self-image. We report two case studies that had marked facial weakness after resection of recurrent pleomorphic adenoma and their progress with electrical stimulation. Subjects received electrical stimulation twice daily for 24 weeks during which photographs of expressions, facial measurements and Sunnybrook scores were recorded. Both subjects recovered good facial function demonstrating Sunnybrook scores of 54 and 64 that improved to 88 and 96, respectively. Neither subjects demonstrated adverse effects of treatment. We conclude that electrical stimulation is a safe treatment and may improve facial palsy in patients after resection of recurrent pleomorphic adenoma. Larger studies would be difficult to pursue due to the low incidence of cases. PMID:27106613

  8. Immunohistochemical expression of tenascin in normal human salivary glands and in pleomorphic adenomas.

    PubMed

    Sunardhi-Widyaputra, S; Van Damme, B

    1993-03-01

    The presence of the extracellular matrix glycoprotein tenascin was studied immunohistochemically in normal human salivary glands and in pleomorphic adenomas. Its expression was compared to that of fibronectin, and type IV collagen. In the normal salivary gland, tenascin was found with interruptions in periductal tissues, and continuously in blood vessels, fat cells and around nerve bundles. In pleomorphic adenoma, tenascin was detected surrounding the clusters of epithelioid cells, in areas with a myxoid and a chondroid matrix, and around some myoepithelial cells as a halo. As compared to fibronectin, there is a similar location of tenascin and fibronectin around tumor cell clusters but not in myxoid and chondroid matrices. Fibronectin was found around the cells in chondroid matrix. In conclusion, tenascin is not only found in malignant tumors but also in benign tumors such as pleomorphic adenoma. The presence of tenascin as a halo around myoepithelial cells suggests a role of these cells in development of myxoid and chondroid matrices.

  9. Immunoprofile of reactive salivary myoepithelial cells in intraductal areas of carcinoma ex-pleomorphic adenoma.

    PubMed

    de Araújo, Vera Cavalcanti; Altemani, Albina; Furuse, Cristiane; Martins, Marília Trierveiler; de Araújo, Ney Soares

    2006-11-01

    The myoepithelial cell (MC) is a component of various secretory glands, including salivary glands. Besides its function, a tumor suppressor and a tumor facilitating functions have been attributed to this cell. We investigated the immunoprofile of benign MC in intraductal areas of carcinoma ex-pleomorphic adenoma (CXPA), comparing them with the MC in duct-like areas of pleomorphic adenoma, origin of the malignant tumor. Antibodies against myoepithelial markers-CK14, alpha-SMA, calponin, P63, CD10, and D2-40-plus laminin and maspin was applied in four selected cases of intracapsular and minimal invasive CXPA with only luminal differentiation presenting areas of intraductal carcinoma. The immunohistochemical reactions of all the antibodies showed stronger staining in benign MC surrounding the malignant epithelial cells than in benign MC in duct-like areas of pleomorphic adenoma, thus revealing that in the malignization process the benign MC become differentiated and produce important proteins related to the tumor suppressor function.

  10. Metastasising pleomorphic salivary gland adenoma presenting as synchronous pulmonary and hepatic metastases.

    PubMed

    Abou-Foul, Ahmad K; Madi, Mohammed; Bury, Danielle; Merritt, Anita

    2014-01-01

    Pleomorphic salivary adenomas (PAs) are the commonest benign tumours of glandular origin in the head and neck. Occasionally PAs undergo malignant transformation to carcinoma-ex-PA and can metastasise. More rarely they metastasise without malignant transformation of the primary tumour. We present a case of a benign pleomorphic salivary gland adenoma, presenting 7 years later with multiple liver metastases and a synchronous pulmonary metastasis. Histological analysis of the lung and liver lesions confirmed a diagnosis of metastasising pleomorphic adenoma (MPA). The lung lesion was fully excised, but the multifocal nature of the liver lesions rendered them inoperable. The patient is being managed conservatively and to date has no local recurrence of the primary salivary gland tumour or any further metastases. To the best of our knowledge this is the first case of MPA with simultaneous metastasis to both lungs and liver, and also the first to describe multiple liver metastases.

  11. Schwannomas: a pitfall in the diagnosis of pleomorphic adenomas on fine-needle aspiration cytology.

    PubMed

    Kapila, Kusum; Mathur, Sandeep; Verma, Kusum

    2002-07-01

    Fine-needle aspiration cytology (FNAC) is being employed with increasing frequency for the preoperative diagnostic workup of salivary gland lesions. Although most cases show morphologic features very characteristic of specific entities, few lesions, both benign and malignant, can cause problems in interpretation. We report four cases initially diagnosed on FNA as spindle-cell tumors, possibly benign nerve sheath tumors (BNST) in the salivary gland region. These cases were later diagnosed on histologic examination as schwannoma (two cases) and as pleomorphic adenoma (two cases). Review of the cytomorphology of these four cases enabled the correct diagnosis of pleomorphic adenoma in the two cases misinterpreted as BNST. Benign peripheral nerve sheath tumors should always be considered in the differential diagnosis of pleomorphic adenoma. A diligent search for epithelial elements is recommended prior to diagnosing BNST in the head and neck region.

  12. [Grading of soft tissue and bone sarcomas].

    PubMed

    Petersen, I; Wardelmann, E

    2016-07-01

    Malignancy grading is an essential element in the classification of sarcomas. It correlates with the prognosis of the disease and the risk of metastasis. This article presents the grading schemes for soft tissue, bone and pediatric sarcomas. It summarizes the histological criteria of the Federation Nationale des Centres de Lutte Contre le Cancer (FNCLCC) system and the Pediatric Oncology Group as well as the grading of bone tumors by the College of American Pathologists (CAP). Furthermore, the potential relevance of gene expression signatures, the complexity index in sarcoma (CINSARC) and single genetic alterations (p53, MDM2, p16, SWI/SNF, EWSR1 fusions and PAX3/PAX7-FOXO1 fusions) for the prognosis of sarcomas are discussed.

  13. [Grading of soft tissue and bone sarcomas].

    PubMed

    Petersen, I; Wardelmann, E

    2016-07-01

    Malignancy grading is an essential element in the classification of sarcomas. It correlates with the prognosis of the disease and the risk of metastasis. This article presents the grading schemes for soft tissue, bone and pediatric sarcomas. It summarizes the histological criteria of the Federation Nationale des Centres de Lutte Contre le Cancer (FNCLCC) system and the Pediatric Oncology Group as well as the grading of bone tumors by the College of American Pathologists (CAP). Furthermore, the potential relevance of gene expression signatures, the complexity index in sarcoma (CINSARC) and single genetic alterations (p53, MDM2, p16, SWI/SNF, EWSR1 fusions and PAX3/PAX7-FOXO1 fusions) for the prognosis of sarcomas are discussed. PMID:27384333

  14. Penile Sarcoma: Report of a Rare Malignancy

    PubMed Central

    Kumar, Vijay; Chaturvedi, Arun; Vishnoi, Jeevan Ram; Dontula, Prashant

    2016-01-01

    Penile cancer is an uncommon malignancy. Squamous cell carcinoma constitutes approximately 95% of all histology. Non-squamous malignancies are rare in penis. Sarcomas of penis are rarer among them. Spindle cell sarcoma is one of the extremely rare sarcoma of penis. To best of our knowledge, only two cases have been reported so far, one in English literature and other in Japanese. We are presenting this uncommon case of spindle cell sarcoma of penis, which was diagnosed with microscopy with its characteristic immunohistochemistry. The disease had an aggressive course with multiple recurrences in a short duration despite margin negative resection. Disease responded poorly with the chemotherapy and patient succumbed to the disease. PMID:27630937

  15. Pulmonary Artery Intimal Sarcoma: A Case Report

    PubMed Central

    Kriz, Joseph P.; Munfakh, Nabil A.; King, Gregory S.; Carden, Juan O.

    2016-01-01

    Pulmonary artery intimal sarcomas are rare and lethal malignant tumors that typically affect larger vessels: the aorta, inferior vena cava, and pulmonary arteries. Since symptoms and imaging of pulmonary arterial intimal sarcomas mimic pulmonary thromboembolism, the differential diagnosis of a patient presenting with chest pain, dyspnea, and filling defect within the pulmonary arteries should include intimal sarcoma. Often right ventricular failure is observed due to pulmonary hypertension caused by the obstructive effect of the tumor and concomitant chronic thromboembolism. We report the case of a 72-year-old African-American male with arterial intimal sarcoma of the left and right pulmonary artery with extension through the right artery into the bronchus and right lung. PMID:27239183

  16. Soft Tissue Sarcomas and Agent Orange

    MedlinePlus

    ... survivors' benefits . Research on soft tissue sarcoma and herbicides The Health and Medicine Division (formally known as ... report " Veterans and Agent Orange: Health Effects of Herbicides Used in Vietnam " and other updates that there ...

  17. Solitary pulmonary nodule: pleuropulmonary synovial sarcoma.

    PubMed

    Ward, Robert C; Birnbaum, Ariel E; Aswad, Bassam I; Healey, Terrance T

    2014-05-01

    Pleuropulmonary synovial sarcoma (PPSS) is an extremely rare primary malignancy of the lung. We present a case of a middle-aged female with PPSS that was initially discovered as an incidental indeterminate nodule on chest radiograph. Following evaluation with computed tomography (CT), the patient went on to positron-emission tomography (PET)/CT for work-up of the solitary pulmonary nodule, which demonstrated mild FDG-avidity and no other evidence of FDG-avid disease. The patient then underwent thoracotomy and right upper lobectomy for definitive treatment. Only after evaluation of the gross pathology, histology, immunohistochemistry and cytogenetics was the diagnosis of synovial sarcoma made. Importantly, the preceding PET/CT, in addition to physical exam of the upper and lower extremities, helped exclude the more common extra-thoracic soft-tissue variety of synovial sarcoma, which frequently metastasizes to lung, carrying a worse prognosis. Discussion of synovial sarcoma and PPSS follows. PMID:24791267

  18. Immunosuppressive Therapy-Related Kaposi Sarcoma

    MedlinePlus

    ... therapy are used to treat Kaposi sarcoma lesions . Photon radiation therapy treats lesions with high-energy light. ... complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information ...

  19. Penile Sarcoma: Report of a Rare Malignancy.

    PubMed

    Rajan, Shiv; Kumar, Vijay; Chaturvedi, Arun; Vishnoi, Jeevan Ram; Dontula, Prashant

    2016-07-01

    Penile cancer is an uncommon malignancy. Squamous cell carcinoma constitutes approximately 95% of all histology. Non-squamous malignancies are rare in penis. Sarcomas of penis are rarer among them. Spindle cell sarcoma is one of the extremely rare sarcoma of penis. To best of our knowledge, only two cases have been reported so far, one in English literature and other in Japanese. We are presenting this uncommon case of spindle cell sarcoma of penis, which was diagnosed with microscopy with its characteristic immunohistochemistry. The disease had an aggressive course with multiple recurrences in a short duration despite margin negative resection. Disease responded poorly with the chemotherapy and patient succumbed to the disease. PMID:27630937

  20. Depsipeptide (Romidepsin) in Treating Patients With Metastatic or Unresectable Soft Tissue Sarcoma

    ClinicalTrials.gov

    2016-09-12

    Adult Alveolar Soft-part Sarcoma; Adult Angiosarcoma; Adult Epithelioid Sarcoma; Adult Extraskeletal Chondrosarcoma; Adult Extraskeletal Osteosarcoma; Adult Fibrosarcoma; Adult Leiomyosarcoma; Adult Liposarcoma; Adult Malignant Fibrous Histiocytoma; Adult Malignant Hemangiopericytoma; Adult Malignant Mesenchymoma; Adult Neurofibrosarcoma; Adult Rhabdomyosarcoma; Adult Synovial Sarcoma; Gastrointestinal Stromal Tumor; Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Adult Soft Tissue Sarcoma; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Stage III Adult Soft Tissue Sarcoma; Stage IV Adult Soft Tissue Sarcoma

  1. WT1 expression in salivary gland pleomorphic adenomas: a reliable marker of the neoplastic myoepithelium.

    PubMed

    Langman, Gerald; Andrews, Claire L; Weissferdt, Annikka

    2011-02-01

    Pleomorphic adenoma is a benign salivary gland neoplasm with a diverse morphology. This is considered to be a function of the neoplastic myoepithelium, which shows histological and immunophenotypical variability. Wilms' tumor 1 gene (WT1) protein, involved in bidirectional mesenchymal-epithelial transition, has been detected by reverse transcription PCR in salivary gland tumors showing myoepithelial-epithelial differentiation. The aim of this study was to investigate the immunoreactivity of WT1 in pleomorphic adenomas and to compare the pattern of staining with p63 and calponin, two reliable markers of myoepithelial cells. A total of 31 cases of pleomorphic adenoma were selected. The myoepithelium was classified as myoepithelial-like (juxtatubular and spindled), modified myoepithelium (myxoid, chondroid and plasmacytoid) and transformed myoepithelium (solid epithelioid, squamous and basaloid cribriform). Immunohistochemistry for WT1, p63 and calponin was assessed in each myoepithelial component, as well as in nonneoplastic myoepithelial cells and inner tubular epithelial cells. There was no immunostaining of tubular epithelial cells by any of the markers. In contrast to p63 and calponin, WT1 did not react with normal myoepithelial cells. Cytoplasmic WT1 staining was present in all pleomorphic adenomas, and in 29 cases (94%), >50% of neoplastic myoepithelial cells were highlighted. p63 and calponin stained the myoepithelium in 30 tumors. In comparison, 50% of cells were positive in 21 (68%) and 9 (29%) cases of p63 and calponin, respectively. Staining with WT1 showed less variability across the spectrum of myoepithelial differentiation with the difference most marked in the transformed myoepithelium. WT1 is a sensitive marker of the neoplastic myoepithelial cell in pleomorphic adenomas. The role of this protein in influencing the mesenchymal-epithelial state of cells suggests that WT1 and the myoepithelial cell have an important role in the histogenesis of

  2. Myeloid Sarcoma: Current Approach and Therapeutic Options

    PubMed Central

    Avni, Batia; Koren-Michowitz, Maya

    2011-01-01

    Myeloid sarcoma is a rare disease that can present as an isolated extramedullary leukemic tumor, concurrently with or at relapse of acute myeloid leukemia. Owing to the rarity of this disorder, most of the literature comprises small retrospective studies and case reports. The aim of this review is to summarize the current published data regarding the clinical presentation, morphological, cytogenetic and molecular features, prognosis and treatment of myeloid sarcoma. PMID:23556098

  3. Imaging spectrum in soft tissue sarcomas.

    PubMed

    Aga, Pallavi; Singh, Ragini; Parihar, Anit; Parashari, Umesh

    2011-12-01

    Imaging plays an important role in detection, diagnosis as well as pre and post operative management of patients with soft tissue sarcomas. Soft tissue sarcomas are generally a diagnostic dilemma needing the complimentary use of both radiology and pathology for their accurate diagnosis. In this review article, we have tried to highlight the important facts about the various imaging modalities available as well as the recent advances in the field of radiology. PMID:23204782

  4. Surgical management of soft tissue sarcomas

    SciTech Connect

    Arlen, M.; Marcove, R.C.

    1987-01-01

    This volume reflects the latest thinking in surgical and adjuvant forms of therapy that can be offered to the sarcoma patient. Based on their analysis of sarcoma patients, the authors stress management based on site of origin, and discuss tumors on and about the shoulder girdle, hip joint, extremity, retroperitoneum, etc. Coverage includes methods for limb preservation; techniques for regional node resection; indications and methods for arterial perfusion, cryosurgery and isotope implantation; pre- and post-operative immunotherapy chemotherapy and radiation therapy.

  5. Imaging in Soft Tissue Sarcomas: Current Updates.

    PubMed

    Jagannathan, Jyothi P; Tirumani, Sree Harsha; Ramaiya, Nikhil H

    2016-10-01

    Soft tissue sarcomas (STS) are heterogeneous malignant tumors that have nonspecific imaging features. A combination of clinical, demographic, and imaging characteristics can aid in the diagnosis. Imaging provides important information regarding the tumor extent, pretreatment planning, and surveillance of patients with STS. In this article, we illustrate the pertinent imaging characteristics of the commonly occurring STS and some uncommon sarcomas with unique imaging characteristics. PMID:27591491

  6. Kaposi's Sarcoma Herpesvirus Genome Persistence.

    PubMed

    Juillard, Franceline; Tan, Min; Li, Shijun; Kaye, Kenneth M

    2016-01-01

    Kaposi's sarcoma-associated herpesvirus (KSHV) has an etiologic role in Kaposi's sarcoma, primary effusion lymphoma, and multicentric Castleman's disease. These diseases are most common in immunocompromised individuals, especially those with AIDS. Similar to all herpesviruses, KSHV infection is lifelong. KSHV infection in tumor cells is primarily latent, with only a small subset of cells undergoing lytic infection. During latency, the KSHV genome persists as a multiple copy, extrachromosomal episome in the nucleus. In order to persist in proliferating tumor cells, the viral genome replicates once per cell cycle and then segregates to daughter cell nuclei. KSHV only expresses several genes during latent infection. Prominent among these genes, is the latency-associated nuclear antigen (LANA). LANA is responsible for KSHV genome persistence and also exerts transcriptional regulatory effects. LANA mediates KSHV DNA replication and in addition, is responsible for segregation of replicated genomes to daughter nuclei. LANA serves as a molecular tether, bridging the viral genome to mitotic chromosomes to ensure that KSHV DNA reaches progeny nuclei. N-terminal LANA attaches to mitotic chromosomes by binding histones H2A/H2B at the surface of the nucleosome. C-terminal LANA binds specific KSHV DNA sequence and also has a role in chromosome attachment. In addition to the essential roles of N- and C-terminal LANA in genome persistence, internal LANA sequence is also critical for efficient episome maintenance. LANA's role as an essential mediator of virus persistence makes it an attractive target for inhibition in order to prevent or treat KSHV infection and disease. PMID:27570517

  7. [Problems in diagnosis and therapy of carcinoma in stroma-abundant pleomorphic adenoma of the parotid gland].

    PubMed

    Stojadinovic, S; Reinert, S; Philippou, S

    1998-09-01

    The case is reported of a 27-year-old patient who developed multiple recurrences of a carcinoma arising from richly stromal pleomorphic adenoma of the parotid gland over 13 years. Originally the tumor was classified as a benign pleomorphic adenoma because there were no cytological characteristics of malignancy. Later on the diagnosis had to be modified to that of a carcinoma arising from a pleomorphic adenoma because of the infiltrating growth pattern of the tumor. A multicentric local recurrence and metastases on the left side of the neck supported the correct diagnosis. This case demonstrates that, beside the classic cytological criteria of malignancy invasion and penetration of the capsule can be decisive for the diagnosis of malign transformed pleomorphic adenoma. Treatment and prognosis of salivary gland tumors assume a clear determination of their diguity. The unusual course in this case of a pleomorphic adenoma originally diagnosed as benign demonstrates the importance of regular follow-up.

  8. Pleomorphic adenoma of breast-a case report and review of literature.

    PubMed

    Leekha, Nitin; Muralee, Madhu; Mathews, Anitha; Preethi, T R; Ahamed, M Iqbal

    2014-06-01

    Pleomorphic adenoma (PA) is a rare benign tumour of the breast. However inadequate surgery of this tumour, which is notorious for its pseudopod like extension into adjacent tissue, results in recurrence. We report a case of pleomorphic adenoma of the breast that has been excised at a local hospital and then referred to a tertiary care hospital for definite management. The diagnostic dilemmas and optimal management, of such cases where dependable pathology report and details of primary surgery are often not available, are discussed. PMID:25114471

  9. [Enucleation of a pleomorphic adenoma of the palate: a conservative choice].

    PubMed

    Tucci, E; Santilli, F; Galli, M

    2004-03-01

    The benign mixed tumor (pleomorphic adenoma) is the most common intraoral salivary gland tumor: more than 50% occurs on the hard and soft palate. The epidemiological data, histopathological and clinical features, preoperative diagnostic methodologies, treatment options, recurrence rates and prediction published in the literature for this neoplasm are discussed. A case of minor salivary gland tumor of the palate in a 26 year old man is reported. The therapy chosen was a surgical conservative enucleation of the lesion. Histological examination showed pleomorphic adenoma; there is no recurrence after 2 years.

  10. Pleomorphic adenoma--unusual presentation of a salivary gland tumor in the neck of a child.

    PubMed

    Arunkumar, K V; Kumar, Sanjeev; Bansal, Vishal; Saxena, Susmita; Elhence, Poonam

    2011-01-01

    Ectopic salivary gland tumors are rare in children. When salivary gland tumors do develop, they preferentially affect major salivary glands and then minor salivary glands. Pleomorphic adenoma, also referred to as a benign mixed tumor, is the most common tumor of the salivary glands. Approximately 90% of these tumors occur in the parotid gland, while the remaining 10% affect the minor salivary glands. However, it is uncommon to find them elsewhere in the head and neck region. We report a rare case of pleomorphic adenoma in the upper neck, an unusual site in an 8-year-old boy.

  11. [Malignant pleomorphic adenoma of the salivary gland: a clinicopathological and immunohistochemical study].

    PubMed

    Ji, X L

    1993-11-01

    Eighty three cases of malignant pleomorphic adenomas (MPA) of salivary glands were studied. Twenty tumors among of them were labeled with 4 kinds of antibodies including anti-CEA, anti-S 100 P, anti-CK12 and CK27 by immunohistochemical staining. Based on this study, it was concluded that the majority cases of MPAs derived from malignant transformation of benign pleomorphous adenomas. The author suggested that the MPAs might be classified as carcinomatous and carcino-sarcomatous subtypes according to the histologic appearances of malignant component in the tumor. The CEA positive reaction in the cytoplasm of tumor cells was a reliable marker of malignancy.

  12. Pleomorphic adenoma of the external auditory canal: a case report and review of the literature.

    PubMed

    Ayers, Lisa Skultety; Depasquale, Kalpana; Marlowe, Frank I; Ghaderi, Mahmoud

    2010-03-01

    Pleomorphic adenomas of the external auditory canal (EAC) are benign tumors of the ceruminal glands; they are a rare entity. Arising from the lateral cartilaginous portion of the EAC, these lesions can be challenging to diagnose in view of their rare clinical presentation, indolent symptoms, and a lack of familiarity on the part of histopathologists. We report the case of a pleomorphic adenoma in a 32-year-old woman, and we review the literature on glandular neoplasms of the EAC, with particular emphasis on terminology and factors that can hinder the diagnosis.

  13. Carcinoma ex pleomorphic adenoma of the sublingual gland: a case report.

    PubMed

    Ariyoshi, Yasunori; Shimahara, Masashi; Konda, Toshiyuki; Tsuji, Motomu

    2012-03-01

    We report a case of carcinoma ex pleomorphic adenoma of a sublingual gland in a 70-year-old man. Under a clinical diagnosis of benign salivary gland tumor, excision of the mass with the sublingual salivary gland in an en bloc fashion via an intraoral approach was performed. Histopathologically, there was a rupture of the fibrous capsule and diffuse cell-rich sheets composed of myoepithelial cells with round nuclei were also seen. Immunohistochemically, the cells that composed of cell rich sheets were positive to smooth muscle actin. Final diagnosis of myoepithelial carcinoma ex pleomorphic adenoma was made.

  14. Benign metastasizing pleomorphic adenoma presenting as a solitary kidney mass: imaging features.

    PubMed

    Vivian, Mark A; Sahni, V Anik; Lowe, Alarice C; Silverman, Stuart G

    2012-08-01

    Benign metastasizing pleomorphic adenoma is a rare condition that occurs in patients with a prior history of pleomorphic adenoma of the salivary glands. Metastases to the kidney are extremely rare, and, to the best of our knowledge, their imaging appearance on multiple cross-sectional imaging modalities has not been described. We present a solitary metastasis to the kidney in a 40-year-old woman. Computed tomography and magnetic resonance imaging demonstrated a 2.4 cm, well-marginated, enhancing mass that protruded into the renal sinus fat. Findings were indistinguishable from a primary renal malignancy. Prior history is crucial in suggesting the correct diagnosis.

  15. [Mixed tumor (pleomorphic adenoma) of head and neck. Typical and atypical patterns].

    PubMed

    García Berrocal, J R; Ramírez Camacho, R; Trinidad, A; Salas, C

    2000-01-01

    Pleomorphic adenoma is a benign growth frequently encountered in major salivary glands, although minor salivary glands sometimes can be affected. In the latter non-specific clinical signs allows only the postoperative diagnosis. Nevertheless the existence of lesser salivary glands outside the oral cavity, and therefore pleomorphic adenomas, justify the specialist's need of its knowledge because of the difficulties arising in differential diagnosis. The AA. present a review of 22 cases in various sites treated in their Department in a 9 year-term.

  16. Pleomorphic adenoma of an accessory submandibular salivary gland: a rare entity.

    PubMed

    Desai, Rajiv S; Meshram, Deepashree; Jangam, Sagar S; Singh, Jatinder S

    2015-10-01

    An accessory submandibular salivary gland is a rare anatomical variant, and a tumour within one is even rarer. We describe a 54-year-old man who presented with a slowly-enlarging mass in the right submandibular region which on magnetic resonance imaging (MRI) seemed to be close to, but not arising from, the right submandibular salivary gland. This was found to be a benign pleomorphic adenoma arising from an accessory submandibular salivary gland. To our knowledge it is only the second report of a pleomorphic adenoma that developed within an accessory submandibular salivary gland.

  17. Benign neurilemmoma (schwannoma) masquerading as a pleomorphic adenoma of the submandibular salivary gland.

    PubMed

    Mair, S; Leiman, G

    1989-01-01

    A case of solitary benign neurilemmoma (schwannoma) arising in the submandibular region is presented. The tumor was mistaken clinically for an enlarged submandibular salivary gland. Fine needle aspiration cytology made an erroneous diagnosis of a pleomorphic adenoma, predominantly stromal in composition. Histology of the resection specimen resulted in the correct diagnosis of a benign schwannoma. Review of the needle aspirate demonstrated cytologic features that should enable both the correct diagnosis of this neoplasm and its distinction from pleomorphic adenoma, which it mimicked in this location.

  18. Thymidine phosphorylase expression in Kaposi sarcoma.

    PubMed Central

    Dada, M A; Boshoff, C H; Comley, M A; Turley, H; Schneider, J W; Chetty, R; Gatter, K C

    1996-01-01

    AIMS: To examine the immunohistochemical distribution of thymidine phosphorylase (TP) in all clinicopathological subtypes of Kaposi sarcoma. METHODS: Thirty two biopsy specimens of Kaposi sarcoma (29 patients) were studied. Six of these patients represented classic, six endemic, eight HIV associated, seven post-immunosuppression/transplant related, and two unclassified variants of Kaposi sarcoma. The average age was 49 years (range 22-83 years) and the male: female ratio 24:5. Four samples of angiosarcoma and one of spindle cell haemangio-endothelioma were stained in parallel. All specimens were fixed in formalin, embedded in paraffin wax and processed routinely. Immunohistochemistry was carried out using an antibody directed against CD31 (JC70) and the monoclonal antibody P-GF.44C against TP. RESULTS: All biopsy specimens showed immunoexpression for TP. The spindle cell component stained more strongly than newly formed endothelium lined vessels and normal, resident vessels at a distance from the lesions. CONCLUSIONS: The strong immunoexpression of TP suggests up-regulation of TP and a role for TP in angiogensis in Kaposi sarcoma. The mechanism for the up-regulation of TP remains unknown, but viral infections may trigger it. The differential staining of the various cell components of Kaposi sarcoma also suggest that TP either plays a role in the differentiation and maturation of Kaposi sarcoma or is a reflection of such changes. Images PMID:8707955

  19. 5α-reductase Inhibitors and Risk of High-grade or Lethal Prostate Cancer

    PubMed Central

    Preston, Mark A.; Wilson, Kathryn; Markt, Sarah C.; Ge, Rongbin; Morash, Christopher; Stampfer, Meir J.; Loda, Massimo F.; Giovannucci, Edward; Mucci, Lorelei A.; Olumi, Aria F.

    2014-01-01

    Importance 5α-reductase inhibitors (5ARIs) are widely used for benign prostatic hyperplasia despite controversy regarding potential risk of high-grade prostate cancer with use. Furthermore, the effect of 5ARIs on progression and prostate cancer death remains unclear. Objective To determine the association between 5ARI use and development of high-grade or lethal prostate cancer. Design, Setting, and Participants Prospective observational study of 38,058 men followed for prostate cancer diagnosis and outcomes between 1996–2010 in the Health Professionals Follow-up Study. Exposure Use of 5ARIs between 1996–2010. Main Outcome Measures Cox proportional hazards models were used to estimate risk of prostate cancer diagnosis or development of lethal disease with 5ARI use, adjusting for possible confounders including prostate specific antigen testing. Results During 448,803 person-years of follow-up, we ascertained 3681 incident prostate cancer cases. Of these, 289 were lethal (metastatic or fatal), 456 were high-grade (Gleason 8–10), 1238 were Gleason grade 7, and 1600 were low-grade (Gleason 2–6). A total of 2878 (7.6%) men reported use of 5ARIs between 1996 and 2010. After adjusting for confounders, men who reported ever using 5ARIs over the study period had a reduced risk of overall prostate cancer (HR 0.77; 95% CI, 0.65–0.91). 5ARI users had a reduced risk of Gleason 7 (HR 0.67; 95% CI, 0.49–0.91) and low-grade (Gleason 2–6) prostate cancer (HR 0.74; 95% CI, 0.57–0.95). 5ARI use was not associated with risk of high-grade (Gleason 8–10, HR 0.97; 95% CI, 0.64–1.46) or lethal disease (HR 0.99; 95% CI, 0.58–1.69). Increased duration of use was associated with significantly lower risk of overall prostate cancer (HR for 1 year of additional use 0.95; 95% CI, 0.92–0.99), localized (HR 0.95; 95% CI, 0.90–1.00), and low-grade disease (HR 0.92; 95% CI, 0.85–0.99). There was no association for lethal, high-grade, or grade 7 disease. Conclusions and

  20. Studying Genes in Tissue Samples From Younger and Adolescent Patients With Soft Tissue Sarcomas

    ClinicalTrials.gov

    2016-05-13

    Childhood Alveolar Soft-part Sarcoma; Childhood Angiosarcoma; Childhood Desmoplastic Small Round Cell Tumor; Childhood Epithelioid Sarcoma; Childhood Fibrosarcoma; Childhood Leiomyosarcoma; Childhood Liposarcoma; Childhood Malignant Mesenchymoma; Childhood Neurofibrosarcoma; Childhood Synovial Sarcoma; Chordoma; Desmoid Tumor; Metastatic Childhood Soft Tissue Sarcoma; Nonmetastatic Childhood Soft Tissue Sarcoma; Recurrent Childhood Soft Tissue Sarcoma

  1. Isolated Limb Perfusion of Melphalan With or Without Tumor Necrosis Factor in Treating Patients With Soft Tissue Sarcoma of the Arm or Leg

    ClinicalTrials.gov

    2012-03-14

    Stage IVB Adult Soft Tissue Sarcoma; Stage IIB Adult Soft Tissue Sarcoma; Stage IIC Adult Soft Tissue Sarcoma; Recurrent Adult Soft Tissue Sarcoma; Stage IVA Adult Soft Tissue Sarcoma; Stage III Adult Soft Tissue Sarcoma

  2. Identification of HHV8 in early Kaposi's sarcoma: implications for Kaposi's sarcoma pathogenesis.

    PubMed Central

    Kennedy, M M; Cooper, K; Howells, D D; Picton, S; Biddolph, S; Lucas, S B; McGee, J O; O'Leary, J J

    1998-01-01

    AIMS: Kaposi's sarcoma is a vascular tumour of uncertain pathogenesis possibly caused by an infectious agent, identified in high risk groups. Accumulating solution phase polymerase chain reaction (PCR) and seroepidemiological data suggest that a previously undescribed herpes DNA virus (human herpesvirus 8 (HHV8)) is the causative agent. Using a unique cohort of early Kaposi's sarcoma, the precise cell type infected with HHV8 in such lesions was identified to elucidate further the role of HHV8 in the pathobiology of Kaposi's sarcoma. METHODS: Sixteen cases of early Kaposi's sarcoma (derived from skin and lymph node) were assessed for the presence of HHV8 using both standard solution phase PCR and TaqMan PCR to the KS330 Bam region of HHV8. In situ amplification was also performed on a selected group in an attempt to identify the candidate infected cells. RESULTS: Using both conventional solution phase and TaqMan PCR, 87% of cases were positive. In addition, HHV8 amplicons were localised in situ to endothelial and spindle cell proliferations in early Kaposi's sarcoma. The HHV8 viral load varied from lesion to lesion. CONCLUSIONS: The presence of HHV8 in early lesions supports a role for HHV8 in the pathogenesis of Kaposi's sarcoma. Coupled with recent seroepidemiological studies, these results suggest that HHV8 is the aetiological agent of Kaposi's sarcoma. Its precise interaction with other factors known to be involved in the development of Kaposi's sarcoma, including cytokines and anti-apoptosis genes, requires elucidation. PMID:9624414

  3. The eastern limit of Acadian high grade metamorphism in northern New England: Implications for the location of the Acadian Suture''

    SciTech Connect

    West, D.P. Jr. . Dept. of Geological Sciences)

    1993-03-01

    Identifying the eastern limit of Acadian high grade metamorphism in New England is complicated by the presence of pre-Devonian high grade relics, locally intense Late Paleozoic thermal overprints, and post-metamorphic faults. New [sup 40]Ar/[sup 39]Ar mineral ages from along the eastern margin of high grade metamorphism in Maine and New Hampshire help delineate the eastern limit of Devonian amphibolite facies metamorphism thereby placing constraints on the location of the Acadian suture. In New Hampshire, Acadian high grade metamorphism extends southeast at least as far as the Campbell Hill fault and perhaps as far as the Flint Hill fault. New [sup 40]Ar/[sup 39]Ar hornblende ages and previously published U-Pb monazite ages from the Massabesic Gneiss Complex are Permian indicating high grade Alleghanian metamorphism. New [sup 40]Ar/[sup 39]Ar hornblende ages from the Rye Formation, although complicated by excess argon, are considerably older, indicating an earlier Pre-Silurian amphibolite facies metamorphism affected these rocks. North of Portland, [sup 40]Ar/[sup 39]Ar hornblende ages east of the Norumbega Fault Zone from high grade rocks of the Casco Bay Group have ages that range from Middle Devonian to Early Carboniferous, consistent with diachronous cooling following Acadian metamorphism. Further northeast, in upper amphibolite facies rocks of the Passagassawakeag Gneiss, new [sup 40]Ar/[sup 39]Ar hornblende ages range from 385--395 Ma suggesting that these rocks were also affected by high grade Acadian metamorphism.

  4. FDG-PET/CT Imaging Predicts Histopathologic Treatment Responses after Neoadjuvant Therapy in Adult Primary Bone Sarcomas

    DOE PAGES

    Benz, Matthias R.; Czernin, Johannes; Tap, William D.; Eckardt, Jeffrey J.; Seeger, Leanne L.; Allen-Auerbach, Martin S.; Dry, Sarah M.; Phelps, Michael E.; Weber, Wolfgang A.; Eilber, Fritz C.

    2010-01-01

    Purpose . Tmore » he aim of this study was to prospectively evaluate whether FDG-PET allows an accurate assessment of histopathologic response to neoadjuvant treatment in adult patients with primary bone sarcomas. Methods . Twelve consecutive patients with resectable, primary high grade bone sarcomas were enrolled prospectively. FDG-PET/CT imaging was performed prior to the initiation and after completion of neoadjuvant treatment. Imaging findings were correlated with histopathologic response. Results . Histopathologic responders showed significantly more pronounced decreases in tumor FDG-SUVmax from baseline to late follow up than non-responders ( 64 ± 19 % versus 29 ± 30 %, resp.; P = .03 ). Using a 60% decrease in tumor FDG-uptake as a threshold for metabolic response correctly classified 3 of 4 histopathologic responders and 7 of 8 histopathologic non-responders as metabolic responders and non-responders, respectively (sensitivity, 75%; specificity, 88%). Conclusion . These results suggest that changes in FDG-SUVmax at the end of neoadjuvant treatment can identify histopathologic responders and non-responders in adult primary bone sarcoma patients.« less

  5. Explant culture of sarcoma patients' tissue.

    PubMed

    Muff, Roman; Botter, Sander M; Husmann, Knut; Tchinda, Joelle; Selvam, Philomina; Seeli-Maduz, Franziska; Fuchs, Bruno

    2016-07-01

    Human sarcomas comprise a heterogeneous group of rare tumors that affect soft tissues and bone. Due to the scarcity and heterogeneity of these diseases, patient-derived cells that can be used for preclinical research are limited. In this study, we investigated whether the tissue explant technique can be used to obtain sarcoma cell lines from fresh as well as viable frozen tissue obtained from 8 out of 12 soft tissue and 9 out of 13 bone tumor entities as defined by the World Health Organization. The success rate, defined as the percent of samples that yielded sufficient numbers of outgrowing cells to be frozen, and the time to freeze were determined for a total of 734 sarcoma tissue specimens. In 552 cases (75%) enough cells were obtained to be frozen at early passage. Success rates were higher in bone tumors (82%) compared with soft tissue tumors (68%), and the mean time to freezing was lower in bone tumors (65 days) compared with soft tissue tumors (84 days). Overall, from 40% of the tissues cells could be frozen at early passage within <2 month after tissue removal. Comparable results as with fresh tissue were obtained after explant of viable frozen patient-derived material. In a selected number of bone and soft tissue sarcoma entities, conventional karyotyping and/or FISH (fluorescence in situ hybridization) analysis revealed a high amount (>60%) of abnormal cells in 41% of analyzed samples, especially in bone sarcomas (osteosarcoma and Ewing sarcoma). In conclusion, the explant technique is well suited to establish patient-derived cell lines for a large majority of bone and soft tissue sarcoma entities with adequate speed. This procedure thus opens the possibility for molecular analysis and drug testing for therapeutic decision making even during patient treatment. PMID:27111283

  6. Intratumoral oxygen gradients mediate sarcoma cell invasion.

    PubMed

    Lewis, Daniel M; Park, Kyung Min; Tang, Vitor; Xu, Yu; Pak, Koreana; Eisinger-Mathason, T S Karin; Simon, M Celeste; Gerecht, Sharon

    2016-08-16

    Hypoxia is a critical factor in the progression and metastasis of many cancers, including soft tissue sarcomas. Frequently, oxygen (O2) gradients develop in tumors as they grow beyond their vascular supply, leading to heterogeneous areas of O2 depletion. Here, we report the impact of hypoxic O2 gradients on sarcoma cell invasion and migration. O2 gradient measurements showed that large sarcoma mouse tumors (>300 mm(3)) contain a severely hypoxic core [≤0.1% partial pressure of O2 (pO2)] whereas smaller tumors possessed hypoxic gradients throughout the tumor mass (0.1-6% pO2). To analyze tumor invasion, we used O2-controllable hydrogels to recreate the physiopathological O2 levels in vitro. Small tumor grafts encapsulated in the hydrogels revealed increased invasion that was both faster and extended over a longer distance in the hypoxic hydrogels compared with nonhypoxic hydrogels. To model the effect of the O2 gradient accurately, we examined individual sarcoma cells embedded in the O2-controllable hydrogel. We observed that hypoxic gradients guide sarcoma cell motility and matrix remodeling through hypoxia-inducible factor-1α (HIF-1α) activation. We further found that in the hypoxic gradient, individual cells migrate more quickly, across longer distances, and in the direction of increasing O2 tension. Treatment with minoxidil, an inhibitor of hypoxia-induced sarcoma metastasis, abrogated cell migration and matrix remodeling in the hypoxic gradient. Overall, we show that O2 acts as a 3D physicotactic agent during sarcoma tumor invasion and propose the O2-controllable hydrogels as a predictive system to study early stages of the metastatic process and therapeutic targets. PMID:27486245

  7. RTOG Sarcoma Radiation Oncologists Reach Consensus on Gross Tumor Volume (GTV) and Clinical Target Volume (CTV) on Computed Tomographic Images for Preoperative Radiotherapy of Primary Soft Tissue Sarcoma of Extremity in RTOG Studies

    PubMed Central

    Wang, Dian; Bosch, Walter; Roberge, David; Finkelstein, Steven E.; Petersen, Ivy; Haddock, Michael; Chen, Yen-Lin E.; Saito, Naoyuki G.; Kirsch, David G.; Hitchcock, Ying J.; Wolfson, Aaron H.; DeLaney, Thomas F.

    2011-01-01

    Objective To develop an Radiation Therapy Oncology Group (RTOG) atlas delineating gross tumor volume (GTV), and clinical target volume (CTV) to be used for preoperative radiotherapy of primary extremity soft tissue sarcoma (STS). Methods A consensus meeting was held during the RTOG meeting in January 2010 to reach agreement about GTV and CTV delineation on CT images for preoperative radiotherapy of high-grade large extremity STS. Data were presented to address the local extension of STS. Extensive discussion ensued to develop optimal criteria for GTV and CTV delineation on CT images. Results A consensus was reached on appropriate CT-based GTV and CTV. GTV is gross tumor defined by T1 contrast-enhanced MRI images. Fusion of MRI and CT is recommended to delineate the GTV. CTV for high-grade large STS typically includes GTV plus 3 cm margins in the longitudinal directions. If this causes the field to extend beyond the compartment, the field can be shortened to include the end of a compartment. The radial margin from the lesion should be 1.5 cm including any portion of the tumor not confined by an intact fascial barrier, bone or skin surface. Conclusion The consensus on GTV and CTV for preoperative radiotherapy of high-grade large extremity STS is available as web-based images as well as descriptive format through the RTOG. This is expected to improve target volume consistency and allow for rigorous evaluation of the benefits and risks of such treatment. PMID:21676552

  8. Role of Combined Circumareolar Skin Excision and Liposuction in Management of High grade Gynaecomastia

    PubMed Central

    Sarkar, Arindam; Bain, Jayanta; Bhattacharya, Debtanu; Sawarappa, Raghavendra; Munian, Kinkar; Dutta, Gouranga; Naiyer, Ghulam Jeelani; Ahmad, Shamshad

    2014-01-01

    Introduction: High-grade gynaecomastia (Simon IIb and III) has tissue excess (skin excess, enlarged areola, and displaced nipple), which is best managed surgically; however, results of conventional breast reduction surgeries and liposuction is not very good. Aim of our study was to describe a combined technique to manage these problems to produce a good result. Material and Method: This was a 2-year study among 12 patients of high grade gynaecomastia. Clinical and laboratory findings were normal. Pre-operatively in standing position, diameter of breast and areola, position of nipple, and amount of skin excess were marked. Under general anaesthesia, tumescent infiltration, circumareolar de-epithelisation of skin excess, and liposuction was completed. Redundant portion of the breast was sharply dissected and pulled out. Areola was fixed over pectoralis fascia at mid humerus level, just medial to the mid-clavicular line. Outer borders of the de-epithelised area were apposed by the purse-string effect of a subdermal suture, and further apposed by few half buried horizontal mattress sutures. Drains for 24 hour and compressive dressings for 6 weeks were used. Result: Mean age of presentation was 25.8 year; emotional discomfort was the chief complaint. Among 12 patients, 10 patients had bilateral gynaecomastia and 8 patients had enlarged and displaced nipple-areola complex. Average hospital stay was 2.41 days and recoveries were usually uneventful. Conclusion: The problem of tissue excess and tissue displacement in high grade gynaecomastia can be well managed by this combined circumareolar skin reduction and liposuction technique to achieve a scar-less flat male chest. PMID:25136214

  9. An International Collaborative Family-Based Whole-Genome Linkage Scan for High-Grade Myopia

    PubMed Central

    Li, Yi-Ju; Guggenheim, Jeremy A.; Bulusu, Anuradha; Metlapally, Ravikanth; Abbott, Diana; Malecaze, Francois; Calvas, Patrick; Rosenberg, Thomas; Paget, Sandrine; Creer, Rosalind C.; Kirov, George; Owen, Michael J.; Zhao, Bei; White, Tristan; Mackey, David A.; Young, Terri L.

    2013-01-01

    Purpose Several nonsyndromic high-grade myopia loci have been mapped primarily by microsatellite markers and a limited number of pedigrees. In this study, whole-genome linkage scans were performed for high-grade myopia, using single nucleotide polymorphisms (SNPs) in 254 families from five independent sites. Methods Genomic DNA samples from 1411 subjects were genotyped (Linkage Panel IVb; Illumina, San Diego, CA). Linkage analyses were performed on 1201 samples from 10 Asian, 12 African-American, and 221 Caucasian families, screening for 5744 SNPs after quality-control exclusions. Two disease states defined by sphere (SPH) and spherical equivalence (SE; sphere+cylinder/2) were analyzed. Parametric and nonparametric two-point and multipoint linkage analyses were performed using the FASTLINK, HOMOG, and MERLIN programs. Multiple stratified datasets were examined, including overall, center-specific, and race-specific. Linkage regions were declared suggestive if they had a peak LOD score ≥ 1.5. Results The MYP1, MYP3, MYP6, MYP11, MYP12, and MYP14 loci were replicated. The novel region q34.11 on chromosome 9 (max NPL= 2.07 at rs913275) was identified. Chromosome 12, region q21.2-24.12 (36.59 cM, MYP3 locus) showed significant linkage (peak HLOD = 3.48) at rs337663 in the overall dataset by SPH and was detected by the Duke, Asian, and Caucasian subsets as well. Potential shared interval was race dependent—a 9.4-cM region (rs163016–rs1520724) driven by the Asian subset and a 1343-cM region (rs163016–rs1520724) driven by the Caucasian subset. Conclusions The present study is the largest linkage scan to date for familial high-grade myopia. The outcomes will facilitate the identification of genes implicated in myopic refractive error development and ocular growth. PMID:19324860

  10. Proliferative activity (ki-67 expression) and outcome in high grade osteosarcoma: a study of 27 cases.

    PubMed

    Jong, R; Davis, A M; Mendes, M G; Wunder, J S; Bell, R S; Kandel, R

    2000-01-01

    Purpose. Although pre-operative chemotherapy has improved the prognosis for individuals with osteosarcoma, approximately 40% of patients will die of their disease.The aim of this study was to quantitate proliferative activity in high grade osteosarcomas and to determine whether proliferation is a prognostic factor.Patients. The study consisted of 27 patients with high grade non-metastatic osteosarcoma at various sites for whom pre-operative biopsies and resection specimens were available for review. All patients were treated similarly and had at least 24 months' follow-up from the date of diagnosis.Methods. Proliferative activity (Ki-67 expression) was examined in the diagnostic biopsies immunohistochemically using the MIB-1 antibody. Proliferation was quantitated in two ways; (1) the number of immunopositive cells was counted manually using an ocular grid; or (2) the percentage of immunopositive nuclear area was assessed using morphometric image analysis. Proliferative index was evaluated in relation to patient outcome.Results. Proliferative activity was seen in all biopsies.The median proliferative index as determined by counting cells was 24% (mean of 27%, range of 7-61%) and by image analysis was 2% (mean 3%, range 0.32-8.4).The correlation between MIB-1 proliferation indices determined either by image analysis methodology or manual cell counting was high (Spearman's rho=0.79). Proliferative index did not appear to predict either disease-free or overall survival.Discussion. Tumor proliferation does not appear to be prognostic for high grade osteosarcomas.Whether assessment of this feature in conjunction with other tumor characteristics might be prognostic requires further study. PMID:18521434

  11. What's New in Kaposi Sarcoma Research and Treatment?

    MedlinePlus

    ... Next Topic Additional resources for Kaposi sarcoma What’s new in Kaposi sarcoma research and treatment? A great ... once it has developed. Treatment Researchers are studying new and different ways to treat KS. Imiquimod (Aldara) ...

  12. What's New in Uterine Sarcoma Research and Treatment?

    MedlinePlus

    ... Next Topic Additional resources for uterine sarcoma What`s new in uterine sarcoma research and treatment? Molecular pathology ... the chromosomes leads to the formation of a new gene, called JAZF1/JJAZ. This gene may help ...

  13. Unique genetic and epigenetic mechanisms driving signatures of paediatric diffuse high-grade glioma

    PubMed Central

    Jones, Chris; Baker, Suzanne J

    2016-01-01

    Diffuse high-grade gliomas (HGGs) of childhood are a devastating spectrum of disease with no effective cures. The two-year survival for paediatric HGG ranges from 30%, for tumours arising in the cerebral cortex, to less than 10% for diffuse intrinsic pontine gliomas (DIPGs), which arise in the brainstem. Recent genome-wide studies provided abundant evidence that unique selective pressures drive HGG in children compared to adults, identifying novel oncogenic mutations connecting tumourigenesis and chromatin regulation as well as developmental signaling pathways. These new genetic findings provide insights into disease pathogenesis, and the challenges and opportunities for improving patient survival in these largely incurable childhood brain tumours. PMID:25230881

  14. Unique genetic and epigenetic mechanisms driving paediatric diffuse high-grade glioma.

    PubMed

    Jones, Chris; Baker, Suzanne J

    2014-10-01

    Diffuse high-grade gliomas (HGGs) of childhood are a devastating spectrum of disease with no effective cures. The two-year survival for paediatric HGG ranges from 30%, for tumours arising in the cerebral cortex, to less than 10% for diffuse intrinsic pontine gliomas (DIPGs), which arise in the brainstem. Recent genome-wide studies provided abundant evidence that unique selective pressures drive HGG in children compared to adults, identifying novel oncogenic mutations connecting tumorigenesis and chromatin regulation, as well as developmental signalling pathways. These new genetic findings give insights into disease pathogenesis and the challenges and opportunities for improving patient survival in these mostly incurable childhood brain tumours.

  15. Genome Sequence of Rough and Smooth Variants of Pleomorphic Strain Lactobacillus farciminis CNCM-I-3699

    PubMed Central

    Tareb, R.; Bernardeau, M.

    2015-01-01

    The probiotic Lactobacillus farciminis CNCM-I-3699 is a pleomorphic strain exhibiting smooth and rough variants. We report their complete genomes consisting of a chromosome of 2, 4 Mb and a plasmid of 6,417 bp. The smooth variant differs by the presence of an additional plasmid of 35,418 bp. PMID:26383668

  16. Pleomorphic adenoma with lymph-node metastases report of two cases.

    PubMed

    Collina, G; Eusebi, V; Carasoli, P T

    1989-02-01

    Two cases of pleomorphic adenoma showing morphologically benign metastases to lymph nodes are reported. In the first case, a 26-year old woman, the lymph node metastases appeared nine years after the primary tumor. In the second case, an 8-year old boy, six years elapsed between the primary and metastatic lesion. A review of the literature of similar cases has been performed.

  17. Benign mixed tumor (pleomorphic adenoma) of the breast: ultrastructural study and review of the literature.

    PubMed

    Cuadros, C L; Ryan, S S; Miller, R E

    1987-09-01

    A patient is presented with a benign mixed tumor (pleomorphic adenoma) of the breast. There are 11 well-documented cases of this rare breast neoplasm. It is histologically and ultrastructurally identical to that seen in the salivary gland and follows a similar benign course. A central role of the ductal myoepithelial cell is proposed for the histogenesis of this tumor.

  18. Pleomorphic adenoma of the nasopharyngeal mucosal space with locally aggressive appearance (2010: 11b).

    PubMed

    Downer, Jonathan; Fryer, Eve; Capper, John; Woo, Eric K

    2011-02-01

    Pleomorphic adenoma arising from submucosal minor salivary gland tissue in the head and neck is well described in the published literature. Where associated bone changes are present, benign-appearing bone remodelling is the rule, reflecting the slow growth of this benign tumour. Aggressive-appearing bone destruction was observed in this case. This atypical appearance has not been widely described.

  19. Pleomorphic adenoma (benign "mixed" tumor) of the human female breast. Case report.

    PubMed

    Fiks, T

    1999-01-01

    A case of solitary pleomorphic adenoma, ("mixed" tumor of salivary gland type) of the left breast associated with the right breast fibroadenoma in 43-year-old woman is reported. The paper describes clinical, cytological, immunohistological and pathological findings in this case and indicates the importance of separating this benign entity from malignances with stromal metaplasia.

  20. Carcinoma ex pleomorphic adenoma of the palate--a case report.

    PubMed

    Kim, K M; Lee, A; Yoon, S H; Kang, J H; Shim, S I

    1997-02-01

    A case of squamous cell carcinoma ex pleomorphic adenoma in a palate is presented and comments on diagnostic criterias are described. The patient was 36-year-old male presenting with an ovoid elevated palate mass for 6 months. The tumor located in the junctional area of soft and hard palate. The mucosa was diffusely ulcerated and the mass focally tightly adherent to adjacent tissue. The initial cytologic and pathological diagnosis by fine needle aspiration biopsy and open biopsy was benign pleomorphic adenoma. After total removal, histologic examination revealed that tumor was composed partly of benign pleomorphic adenoma and partly of an squamous cell carcinoma component with areas of necrosis and capsular invasion. Immunohistochemical staining in the carcinoma area revealed positive reaction for low and high molecular weight cytokeratin, and epithelial membrane antigen, but negative for desmin, actin, GFAP and S-100 protein. In situ hybridization using biotinylated Epstein-Barr virus probe was done and the neoplastic cells were negative. Our case in an unusual partially encapsulated carcinoma ex pleomorphic adenoma in the palate and is not related in EBV infection.

  1. Carcinoma ex pleomorphic adenoma in the oral cavity: a huge oral cavity mass with neck metastasis.

    PubMed

    Hong, Hyun Jun; Byeon, Hyung Kwon; Bae, Seong Hoon; Park, Ah Young; Choi, Eun Chang; Choi, Hong-Shik

    2013-11-01

    Carcinoma ex pleomorphic adenoma (CEPA) is a rare, aggressive, poorly understood malignancy. In CEPA, an epithelial malignancy develops in association with a primary or recurrent benign pleomorphic adenoma. Carcinoma ex pleomorphic adenoma is very difficult to identify before surgery because the clinical presentation of many cases is similar to that of pleomorphic adenomas. The risk for malignancy increases with the duration of a mixed tumor. Treatment of CEPA must be individualized on the basis of the tumor location, involvement of adjacent structures, histologic subtype, and grade. The authors recently experienced a case of CEPA arising in the oral cavity with neck metastasis. The patient was a 70-year-old man presenting a huge mass that was present for 20 years and that slowly grew on the left side of the neck. We treated it with a total excision with wide margins and neck dissection. There was no recurrence during the follow-up period of 5 years up until now. We present a case of an unusually huge CEPA in the oral cavity.

  2. Rare benign pleomorphic adenoma of the nose: short study and literature review.

    PubMed

    Wierzchowska, Małgorzata; Bodnar, Magdalena; Burduk, Paweł K; Kaźmierczak, Wojciech; Marszałek, Andrzej

    2015-07-01

    Pleomorphic adenoma is one of the most common benign tumors of the major salivary glands. It can also occur in the minor salivary glands, which exist in the nasal cavity. Intranasal pleomorphic adenoma usually originates from glands of the nasal septum mucosa. We present the results of endoscopic endonasal surgery of this pathology. The aim of the study was to present the endoscopic technique for nasal septum pleomorphic adenoma surgery. The retrospective examination of 3 patients was performed. There were 2 women and 1 man. Age ranged from 15 to 46 years. All the patients presented with nasal obstruction and occasional epistaxis for at least for 6 months. We performed endoscopic surgery to remove the tumors. In all cases we dissected the septal perichondrium to achieved free margins of the tumor. The microscopic examination revealed an epithelial and myoepithelial component with tubular structures composed of two cell layers, ducts, and a solid area found in a loose myxochondroid area. Additionally, immunohistochemical staining was performed using antibodies against cytokeratin, Ki-67, and vimentin. Patients' postoperative course was uneventful, and no complications were encountered. No recurrence was present during patients' postoperative visits. Nasal benign pleomorphic adenoma is a rare tumor which should be taken into consideration in the nasal cavity during surgery. The correct histological diagnosis can be confirmed by additional histological studies. Endoscopic endonasal surgery is reserved for small tumors.

  3. Pleomorphic adenoma (benign mixed tumor) of the breast: a case report and review of the literature.

    PubMed

    Sato, Katsuaki; Ueda, Yoshimichi; Shimasaki, Miyako; Ozaki, Mamoru; Nitta, Naoki; Chada, Kiran; Ishikawa, Yoshimaro; Katsuda, Shogo

    2005-01-01

    We report a case of pleomorphic adenoma (benign mixed tumor) of the breast, which is an extremely rare location for this tumor. Examination of a 55-year-old woman unexpectedly revealed a mass measuring 0.8 cm in diameter in the subareolar region of the right breast. Excisional biopsy was performed, and the tumor histologically showed pleomorphic adenoma composed of duct epithelial cells, myoepithelial cells, and a myxochondroid matrix. Immunohistochemically, duct epithelial cells were positive for the estrogen receptor, but negative for the progesterone receptor. The nuclei of the spindle and myoepithelial cells were immunoreactive for HMGI-C and HMGI(Y) proteins, indicating a histogenesis similar to pleomorphic adenoma of the salivary glands. Interphase fluorescence in situ hybridization performed on paraffin-embedded tissue sections with 12q15 probes and a 6p21 probe demonstrated no chromosomal rearrangement. Sixty-nine cases of this type of tumor arising in the breast have been described previously. Using imaging procedures, the tumor has occasionally been misdiagnosed as malignant clinically and even pathologically in frozen section diagnosis. Careful diagnosis based on paraffin sections is required to avoid unnecessary aggressive surgery, and pathologists should include pleomorphic adenoma in the differential diagnosis of a demarcated, juxtaareolar, small hard mass.

  4. c-myc, ras p21 and p53 expression in pleomorphic adenoma and its malignant form of the human salivary glands.

    PubMed

    Deguchi, H; Hamano, H; Hayashi, Y

    1993-01-01

    Using an immunohistochemical study and an immunoblot analysis, the expression of cellular oncogenes of the human salivary glands such as c-myc, ras p21, and p53 tumor-suppressor gene in pleomorphic adenomas and its malignant form, carcinoma in pleomorphic adenomas was examined to evaluate a differential biological significance, in comparison with that in normal salivary gland tissues. Immunohistochemically, the c-myc product was detected in 42% of the pleomorphic adenomas and in 56% of the carcinomas in pleomorphic adenoma. The ras p21 expression was observed in 24% of pleomorphic adenomas, and in 50% of carcinomas in pleomorphic adenoma. The p53 protein was detected in 18% of the pleomorphic adenomas and in 67% of the carcinomas in pleomorphic adenoma. Although there was no significant difference between the benign and malignant forms for the expression of c-myc, a statistical significance in ras p21 and p53 expression was found between the pleomorphic adenoma and its malignant form (P < 0.05) and P < 0.001, respectively). An immunoblotting assay clearly demonstrated the expression of c-myc and p53 gene products in both the benign and malignant forms of the pleomorphic adenoma, and that of ras p21 in the malignant form. These results indicate that activation of c-myc and ras p21 proto-oncogenes and the involvement of p53 mutation may play important roles in the malignant transformation of salivary gland pleomorphic adenoma.

  5. Phase II Study of Neoadjuvant Bevacizumab and Radiotherapy for Resectable Soft Tissue Sarcomas

    SciTech Connect

    Yoon, Sam S.; Duda, Dan G.; Karl, Daniel L.; Kim, Tae-Min; Kambadakone, Avinash R.; Chen, Yen-Lin; Rothrock, Courtney; Rosenberg, Andrew E.; Nielsen, G. Petur; Kirsch, David G.; Choy, Edwin; Harmon, David C.; Hornicek, Francis J.; Dreyfuss, Jonathan; Ancukiewicz, Marek; and others

    2011-11-15

    Purpose: Numerous preclinical studies have demonstrated that angiogenesis inhibitors can increase the efficacy of radiotherapy (RT). We sought to examine the safety and efficacy of bevacizumab (BV) and RT in soft tissue sarcomas and explore biomarkers to help determine the treatment response. Methods and Materials: Patients with {>=}5 cm, intermediate- or high-grade soft tissue sarcomas at significant risk of local recurrence received neoadjuvant BV alone followed by BV plus RT before surgical resection. Correlative science studies included analysis of the serial blood and tumor samples and serial perfusion computed tomography scans. Results: The 20 patients had a median tumor size of 8.25 cm, with 13 extremity, 1 trunk, and 6 retroperitoneal/pelvis tumors. The neoadjuvant treatment was well tolerated, with only 4 patients having Grade 3 toxicities (hypertension, liver function test elevation). BV plus RT resulted in {>=}80% pathologic necrosis in 9 (45%) of 20 tumors, more than double the historical rate seen with RT alone. Three patients had a complete pathologic response. The median microvessel density decreased 53% after BV alone (p <.05). After combination therapy, the median tumor cell proliferation decreased by 73%, apoptosis increased 10.4-fold, and the blood flow, blood volume, and permeability surface area decreased by 62-72% (p <.05). Analysis of gene expression microarrays of untreated tumors identified a 24-gene signature for treatment response. The microvessel density and circulating progenitor cells at baseline and the reduction in microvessel density and plasma soluble c-KIT with BV therapy also correlated with a good pathologic response (p <.05). After a median follow-up of 20 months, only 1 patient had developed local recurrence. Conclusions: The results from the present exploratory study indicated that BV increases the efficacy of RT against soft tissue sarcomas and might reduce the incidence of local recurrence. Thus, this regimen warrants

  6. Targeted Therapy of Ewing's Sarcoma

    PubMed Central

    Subbiah, Vivek; Anderson, Pete

    2011-01-01

    Refractory and/or recurrent Ewing's sarcoma (EWS) remains a clinical challenge because the disease's resistance to therapy makes it difficult to achieve durable results with standard treatments that include chemotherapy, radiation, and surgery. Recently, insulin-like-growth-factor-1-receptor (IGF1R) antibodies have been shown to have a modest single-agent activity in EWS. Patient selection using biomarkers and understanding response and resistance mechanisms in relation to IGF1R and mammalian target of rapamycin pathways are areas of active research. Since EWS has a unique tumor-specific EWS-FLI1 t(11;22) translocation and oncogenic fusion protein, inhibition of EWS-FLI1 transcription, translation, and/or protein function may be key to eradicating EWS at the stem-cell level. Recently, a small molecule that blocks the protein-protein interaction of EWS-FLI1 with RNA helicase A has been shown in preclinical models to inhibit EWS growth. The successful application of this first-in-class protein-protein inhibitor in the clinic could become a model system for translocation-associated cancers such as EWS. PMID:21052545

  7. Spinal intradural extraosseous Ewing's sarcoma.

    PubMed

    Mateen, Farrah J; Nassar, Aziza; Bardia, Aditya; Jatoi, Aminah; Haddock, Michael G; Buckner, Jan C; Lachance, Daniel H

    2011-03-30

    Extraosseous Ewing's sarcoma (EES) involving the central nervous system is rare, but can be diagnosed and distinguished from other primitive neuroectodermal tumors (PNET) by identification of the chromosomal translocation (11;22)(q24;q12). We report EES arising from the spinal intradural extramedullary space, based on imaging, histopathological, and molecular data in two men, ages 50 and 60 years old and a review of the literature using PubMed (1970-2009). Reverse transcriptase polymerase chain reaction (RT-PCR) identified the fusion product FL1-EWS. Multimodal therapy, including radiation and alternating chemotherapy including vincristine, cyclophosphamide, doxorubicin and ifosfamide and etoposide led to local tumor control and an initial, favorable therapeutic response. No systemic involvement was seen from the time of diagnosis to the time of last follow-up (26 months) or death (4 years). This report confirms that EES is not confined to the earliest decades of life, and like its rare occurrence as an extra-axial meningeal based mass intracranially, can occasionally present as an intradural mass in the spinal canal without evidence of systemic tumor. Gross total resection followed by multimodal therapy may provide for extended progression free and overall survival.

  8. Antisense oligonucleotides as innovative therapeutic strategy in the treatment of high-grade gliomas.

    PubMed

    Caruso, Gerardo; Caffo, Mariella; Raudino, Giuseppe; Alafaci, Concetta; Salpietro, Francesco M; Tomasello, Francesco

    2010-01-01

    Despite the intensive recent research in cancer therapy, the prognosis in patients affected by high-grade gliomas is still very unfavorable. The efficacy of classical anti-cancer strategies is seriously limited by lack of specific therapies against malignant cells. The extracellular matrix plays a pivotal role in processes such as differentiation, apoptosis, and migration in both the normal and the pathologic nervous system. Glial tumors seem to be able to create a favorable environment for the invasion of glioma cells in cerebral parenchyma when they combine with the extracellular matrix via cell surface receptors. Glioma cells synthesize matrix proteins, such as tenascin, laminin, fibronectin that facilitate the tumor cell's motility. New treatments have shown to hit the acting molecules in the tumor growth and to increase the efficacy and minimize the toxicity. Antisense oligonucleotides are synthetic stretches of DNA which hybridize with specific mRNA strands. The specificity of hybridization makes antisense method an interesting strategy to selectively modulate the expression of genes involved in tumorigenesis. In this review we will focus on the mechanisms of action of antisense oligonucleotides and report clinical and experimental studies on the treatment of high-grade gliomas. We will also report the patents of preclinical and/or clinical studies that adopt the antisense oligonucleotide therapy list in cerebral gliomas.

  9. A chemotherapy response classifier based on support vector machines for high-grade serous ovarian carcinoma

    PubMed Central

    Zhou, Bo; Guo, En-Song; Yang, Zong-Yuan; Liao, Jing; Ding, Dong; Xu, Qin; Lu, Hao; Meng, Li; Wang, Shi-Xuan; Zhou, Jian-Feng; Xing, Hui; Weng, Dan-Hui; Ma, Ding; Chen, Gang

    2016-01-01

    Long-term outcome of high-grade serous epithelial ovarian carcinoma (HGSOC) remains poor as a result of recurrence and the emergence of drug resistance. Almost all the patients were given the same platinum-based chemotherapy after debulking surgery even though some of them are naturally resistant to the first-line chemotherapy. No method could verify this part of patients right after the surgery currently. In this study, we used 156 paraffin-embedded high-grade HGSOC specimens for immunohistochemical analysis with 37 immunology markers, and association between the expression levels of these markers and the chemoresponse were evaluated. A support vector machine (SVM)-based HGSOC prognostic classifier was then established, and was validated by a 95-patient independent cohort. The classifier was strongly predictive of chemotherapy resistance, and divided patients into low- and high-risk groups with significant differences progression-free survival (PFS) and overall survival (OS). This classifier may provide a potential way to predict the chemotherapy resistance of HGSOC right after the surgery, and then allow clinicians to make optimal clinical decision for those potentially chemoresistant patients. The potential clinical application of this classifier will benefit those patients with primary drug resistance. PMID:26675546

  10. Prognostic Value of MRS Metabolites in Postoperative Irradiated High Grade Gliomas

    PubMed Central

    Tolia, Maria; Verganelakis, Dimitrios; Tsoukalas, Nikolaos; Kyrgias, George; Papathanasiou, Matilda; Mosa, Eftichia; Kokakis, Ioannis; Kouvaris, John R.; Pissakas, George; Pistevou-Gombaki, Kyriaki; Kelekis, Nikolaos; Kouloulias, Vasileios

    2015-01-01

    Purpose. We studied the prognostic significance of Magnetic Resonance Spectroscopy (MRS) in operated high grade gliomas. Materials and Methods. Twelve patients were treated with radiotherapy and Temozolomide. The MRS data were taken four weeks after operation (before radiotherapy) and every six months after the completion of RT. The N-acetyl aspartate, choline, creatine, and myo-inositol parameters were quantified, analyzed, and correlated to recurrence-free survival (RFS). Results. The median RFS was 26.06 months. RFS was significantly worse in elderly patients (P = 0.001) along with the higher choline/creatine ratios at either baseline (P = 0.003) or six months post Radiotherapy (P = 0.042). Median RFS was 23 months in high choline/creatine levels ≥2 at 6 months after radiotherapy and 11 months for those with <2 choline/creatine levels. There was a significant correlation of maximum difference of choline/creatine ratio with RFS (rho = 0.64, P = 0.045). Conclusion. Age and choline/creatine ratio are strong independent prognostic factors in high grade gliomas. PMID:26339606

  11. All reovirus subtypes show oncolytic potential in primary cells of human high-grade glioma.

    PubMed

    Alloussi, S H; Alkassar, M; Urbschat, S; Graf, N; Gärtner, B

    2011-09-01

    Reoviridae are non-human pathogenic viruses. The family of reoviridae consists of 4 different subtypes. Many studies have proven that the Dearing subtype 3 has oncolytic potential. This potential is related to the RAS protein expression in tumour cells. The aim of this study, was to investigate whether all reovirus subtypes have oncolytic potential and whether there are differences in their efficacy, in particular for high-grade glioma. To evaluate the oncolytic potential, we performed an in vitro head-to-head study for all reovirus subtypes in 5 primary cell cultures of high-grade gliomas. The oncolytic activity was determined using end-point titration with observation of the cytopathogenic effect. For measurement of RAS activity, we performed an immunofluorescent detection stain on all cell cultures. For quantification of the virus, an RT-PCR measurement for all subtypes was performed. All reovirus subtypes showed oncolytic activity in the observed glioma biopsies. These observations correlated with RAS overexpression in the observed cells. All glioma biopsies overexpressed the RAS protein. The quantitative oncolytic potential differed in relation to the single observed cell culture and in relation to the chosen reovirus subtype. To our knowledge, this is the first study showing oncolytic activity for all reovirus subtypes. We show the relationship and correlation between RAS protein overexpression and vulnerability of cells to reovirus. Efficacy of the different subtypes is interindividually different and cannot be forecast.

  12. Structural patterns in high grade terrain in parts of Tamil Nadu and Karnataka

    NASA Technical Reports Server (NTRS)

    Sugavanam, E. B.; Vidyadharan, K. T.

    1988-01-01

    Detailed geological mapping in parts of Tamil Nadu and Karnataka has brought out vast areas occupied by highly deformed charnockite and high grade gneisses. These areas, similar to high grade shield terrains in other parts of the world have the impress of extensive tectonic reworking multideformation and polymetamorphism and are closely associated with layered ultramafics, shelf type sediments and different igneous events. In North Arcot and Charmapuri districts of Tamil Nadu and Kollegal taluk in Mysore district in Karnataka, charnockite is intensely cofolded with a supracrustal succession of layered ultramafics, pyroxene granulite, pink granolites, magnetite quartzite and khondalites. These areas have undergone five phases of deformation, five generations of basic dyke activities, four phases of migmatisation and two periods of metallogeny. Geochronological data ranges from 2900 m.y. to 750 m.y. In working out the tectanostratigraphy of the above areas the basic dykes of different generations have served as major time markers. In addition, the persistent strike continuity of linear bands of pyroxene granulite, pink granolite and magnetite quartzite has been of great utility in using them as structural markers for bringing out the complex structural history in these areas.

  13. ADVANCES IN IMAGING TECHNOLOGIES IN THE EVALUATION OF HIGH-GRADE BLADDER CANCER

    PubMed Central

    Zlatev, Dimitar V.; Altobelli, Emanuela; Liao, Joseph C.

    2015-01-01

    Bladder cancer is a heterogeneous disease that ranges from low-grade variant with an indolent course, to high-grade subtype with a recurrent, progressive, and potentially lethal outcome. Accurate assessment for individualized treatment depends critically on the diagnostic accuracy of white light cystoscopy. Despite its central role, white light cystoscopy has several well-documented shortcomings including difficult flat lesion detection, imprecise tumor delineation that limits complete resection, differentiation between inflammation and malignancy, and grade and stage determination. As the limitations of white light cystoscopy contribute to the risk of cancer persistence, recurrence, and progression, there is a need for improved visualization of flat, multifocal, high-grade, and muscle-invasive lesions. Optical imaging technologies have emerged as an adjunct to white light cystoscopy with the goal to guide more effective treatment by improving cancer detection and patient stratification on the basis of grade and stage. Photodynamic diagnosis and narrow band imaging are macroscopic imaging modalities similar to white light cystoscopy, but provide additional contrast enhancement of bladder tumors and have been shown to improve detection rates. Confocal laser endomicroscopy and optical coherence tomography are microscopic imaging technologies that enable real-time high resolution, subsurface tissue characterization with spatial resolutions similar to histology. Molecular imaging offers the potential for the combination of optical imaging technologies with cancer-specific molecular agents to improve the specificity of disease detection. PMID:25882557

  14. Nonlinear ghost waves accelerate the progression of high-grade brain tumors

    NASA Astrophysics Data System (ADS)

    Pardo, Rosa; Martínez-González, Alicia; Pérez-García, Víctor M.

    2016-10-01

    We study a reduced continuous model describing the evolution of high grade gliomas in response to hypoxic events through the interplay of different cellular phenotypes. We show that hypoxic events, even when sporadic and/or limited in space, may have a crucial role on the acceleration of high grade gliomas growth. Our modeling approach is based on two cellular phenotypes. One of them is more migratory and a second one is more proliferative. Transitions between both phenotypes are driven by the local oxygen values, assumed in this simple model to be uniform. Surprisingly, even very localized in time hypoxia events leading to transient migratory populations have the potential to accelerate the tumor's invasion speed up to speeds close to those of the migratory phenotype. The high invasion speed persists for times much longer than the lifetime of the hypoxic event. Moreover, the phenomenon is observed both when the migratory cells form a persistent wave of cells located on the invasion front and when they form a evanescent "ghost" wave disappearing after a short time by decay to the more proliferative phenotype. Our findings are obtained through numerical simulations of the model equations both in 1D and higher dimensional scenarios. We also provide a deeper mathematical analysis of some aspects of the problem such as the conditions for the existence of persistent waves of cells with a more migratory phenotype.

  15. Phase 1 trial of vocimagene amiretrorepvec and 5-fluorocytosine for recurrent high-grade glioma.

    PubMed

    Cloughesy, Timothy F; Landolfi, Joseph; Hogan, Daniel J; Bloomfield, Stephen; Carter, Bob; Chen, Clark C; Elder, J Bradley; Kalkanis, Steven N; Kesari, Santosh; Lai, Albert; Lee, Ian Y; Liau, Linda M; Mikkelsen, Tom; Nghiemphu, Phioanh Leia; Piccioni, David; Walbert, Tobias; Chu, Alice; Das, Asha; Diago, Oscar R; Gammon, Dawn; Gruber, Harry E; Hanna, Michelle; Jolly, Douglas J; Kasahara, Noriyuki; McCarthy, David; Mitchell, Leah; Ostertag, Derek; Robbins, Joan M; Rodriguez-Aguirre, Maria; Vogelbaum, Michael A

    2016-06-01

    Toca 511 (vocimagene amiretrorepvec) is an investigational nonlytic, retroviral replicating vector (RRV) that delivers a yeast cytosine deaminase, which converts subsequently administered courses of the investigational prodrug Toca FC (extended-release 5-fluorocytosine) into the antimetabolite 5-fluorouracil. Forty-five subjects with recurrent or progressive high-grade glioma were treated. The end points of this phase 1, open-label, ascending dose, multicenter trial included safety, efficacy, and molecular profiling; survival was compared to a matching subgroup from an external control. Overall survival for recurrent high-grade glioma was 13.6 months (95% confidence interval, 10.8 to 20.0) and was statistically improved relative to an external control (hazard ratio, 0.45; P = 0.003). Tumor samples from subjects surviving more than 52 weeks after Toca 511 delivery disproportionately displayed a survival-related mRNA expression signature, identifying a potential molecular signature that may correlate with treatment-related survival rather than being prognostic. Toca 511 and Toca FC show excellent tolerability, with RRV persisting in the tumor and RRV control systemically. The favorable assessment of Toca 511 and Toca FC supports confirmation in a randomized phase 2/3 trial (NCT02414165). PMID:27252174

  16. Telomerase activity is a biomarker for high grade malignant peripheral nerve sheath tumors in neurofibromatosis type 1 individuals.

    PubMed

    Mantripragada, Kiran K; Caley, Matthew; Stephens, Phil; Jones, Christopher J; Kluwe, Lan; Guha, Abhijit; Mautner, Victor; Upadhyaya, Meena

    2008-03-01

    Telomerase activity (TA) and the expression of its enzymatic subunits, which have been demonstrated in many tumors, remain poorly investigated in tumors associated with neurofibromatosis type 1 (NF1). In this study, we analysed the association of TA and the expression of telomerase RNA (TR) and telomerase reverse transcriptase (TERT) in 23 malignant peripheral nerve sheath tumors (MPNST) (17 high grade and 6 low grade tumors), 11 plexiform neurofibromas (PNF) and 6 dermal neurofibromas (DNF). TA was studied using telomerase repeat amplification protocol (TRAP) assay and expression of TR and TERT was investigated using reverse transcription PCR (RT-PCR) and real-time PCR. TA was detected in 14 out of 17 (82%) high grade MPNST, whereas all 6 low grade MPNST and 17 benign tumors were telomerase negative. The TERT transcripts were detected in all high grade MPNST, 50% of the low grade MPNST, and 4 benign tumors. However, the expression level of the TERT strikingly correlated with TA and high grade MPNST. Thus, while TERT expression was similar in both low grade MPNST and PNF (P = 0.115), it was significantly higher in high grade MPNST when compared to either low grade MPNST (P = 0.042), PNF (P = 0.001) or DNF tumors (P = 0.010). These findings indicate that TA and expression level of TERT are potential markers for high grade malignancy in NF1 patients.

  17. Primary pulmonary synovial sarcoma: a rare neoplasm

    PubMed Central

    Ramos, Montserrat Blanco; Fontán, Eva María García; Carretero, Miguel Ángel Cańizares; Pińeiro, Ana González

    2016-01-01

    Primary pulmonary synovial sarcoma is an extremely rare tumor with an unknown cause. The diagnosis is established after other primary lung malignancies or metastatic extrathoracic sarcoma have been excluded. We report the case of a 69-year-old man who presented with a well-defined mass in the right upper lobe on a chest X-ray. A video-assisted thoracoscopic surgery (VATS) right upper lobectomy was performed. Immunohistochemically, neoplastic cells were positive for vimentin, CD56 and Bcl-2, and focally positive for CD99, epithelial membrane antigen and cytokeratin 7 and 19. The cytogenetic study revealed a SYT genetic reassortment. So, the final pathological diagnosis was primary pulmonary synovial sarcoma. PMID:27516790

  18. Primary pulmonary synovial sarcoma: a rare neoplasm.

    PubMed

    García, José Soro; Ramos, Montserrat Blanco; Fontán, Eva María García; Carretero, Miguel Ángel Cańizares; Pińeiro, Ana González

    2016-06-01

    Primary pulmonary synovial sarcoma is an extremely rare tumor with an unknown cause. The diagnosis is established after other primary lung malignancies or metastatic extrathoracic sarcoma have been excluded. We report the case of a 69-year-old man who presented with a well-defined mass in the right upper lobe on a chest X-ray. A video-assisted thoracoscopic surgery (VATS) right upper lobectomy was performed. Immunohistochemically, neoplastic cells were positive for vimentin, CD56 and Bcl-2, and focally positive for CD99, epithelial membrane antigen and cytokeratin 7 and 19. The cytogenetic study revealed a SYT genetic reassortment. So, the final pathological diagnosis was primary pulmonary synovial sarcoma. PMID:27516790

  19. Pazopanib, a multikinase angiogenesis inhibitor, in patients with relapsed or refractory advanced soft tissue sarcoma: a phase II study from the European organisation for research and treatment of cancer-soft tissue and bone sarcoma group (EORTC study 62043).

    PubMed

    Sleijfer, Stefan; Ray-Coquard, Isabelle; Papai, Zsuzsa; Le Cesne, Axel; Scurr, Michelle; Schöffski, Patrick; Collin, Françoise; Pandite, Lini; Marreaud, Sandrine; De Brauwer, Annick; van Glabbeke, Martine; Verweij, Jaap; Blay, Jean-Yves

    2009-07-01

    PURPOSE Given the importance of angiogenesis in soft tissue sarcoma (STS), pazopanib, an oral angiogenesis inhibitor that targets vascular endothelial growth factor receptor and platelet-derived growth factor receptor, was explored in patients with advanced STS. PATIENTS AND METHODS Patients with intermediate- or high-grade advanced STS who were ineligible for chemotherapy or who had received no more than two prior cytotoxic agents for advanced disease, who had documented progression, who had adequate performance status, and who had good organ function were eligible. Pazopanib 800 mg was given daily. The primary end point was progression-free rate at 12 weeks (PFR(12 weeks)). Secondary end points were response, safety, and overall survival. Four different strata were studied: adipocytic STS, leiomyosarcomas, synovial sarcomas, and other STS types. A Simon two-stage design was applied (P1 = 40%; P0 = 20%; alpha = beta = .1) for each stratum. Results One hundred forty-two patients were enrolled. The adipocytic STS stratum was closed after the first stage, given insufficient activity (PFR(12 weeks), five [26%] of19). PFR(12 weeks) was 18 (44%) of 41 patients in the leiomyosarcoma cohort, 18 (49%) of 37 in the synovial sarcomas, and 16 (39%) of 41 in the other STS types. Compared with historical controls who were treated with second-line chemotherapy, progression-free and overall survivals were prolonged in the three cohorts in which the primary end point was reached. The most frequent drug-related toxicities were hypertension, fatigue, hypopigmentation, and nausea. Other toxicities included liver enzyme elevations, myelosuppression, and proteinuria, all of which were mostly grades 1 to 2. The most frequent grades 3 to 4 toxicities were hyperbilirubinemia (6.3%), hypertension (7.7%), and fatigue (7.7%). CONCLUSION Pazopanib is well tolerated in patients with relapsed, advanced STS and demonstrates interesting activity that warrants additional study in patients with

  20. Use of negative pressure wound therapy as an adjunct to the treatment of extremity soft-tissue sarcoma with ulceration or impending ulceration

    PubMed Central

    CHEN, YU; XU, SONG-FENG; XU, MING; YU, XIU-CHUN

    2016-01-01

    Major wound complications of the extremities, following wide tumor resection and reconstruction for soft-tissue sarcomas (STSs), remain a challenge for limb-sparing surgery. Furthermore, STSs with ulceration or impending ulceration predispose patients to an increased risk of post-operative infection. The present study was conducted to assess the efficacy of negative pressure wound therapy (NPWT) in preventing wound complications associated with surgical treatment of STSs with ulceration or impending ulceration, in patients treated between February 2012 and January 2013. A total of 5 patients, with a mean age of 48 years (range, 24–68 years), were enrolled in the present study. The diagnoses consisted of undifferentiated pleomorphic sarcoma (n=2), leiomyosarcoma (n=1), synovial sarcoma (n=1) and epithelioid sarcoma (n=1). According to American Joint Committee on Cancer criteria, 3 cases were stage III tumors, and the remaining 2 cases were of stages IIA and IIB, respectively. A total of 3 patients exhibited ulceration at diagnosis, and the remaining patients demonstrated impending ulceration. The mean wound area following wide resection of the tumor was 73 cm2 (range, 45–110 cm2). A continuous suction mode, with pressures measuring −200 to −300 mmHg, was used for 7–10 days on the soft-tissue defects as preparation for wound closure. Soft-tissue reconstruction included muscle flaps (n=2) and skin grafts (n=5). No major wound complications occurred. Post-operative functional and cosmetic outcomes were acceptable. A single patient demonstrated local recurrence 12 months after surgery and re-excision of the tumor was performed. All patients remained alive at the conclusion of follow-up, with a mean follow-up time of 26 months (range, 12–36 months). The present study demonstrated that NPWT is effective and safe when used as an adjunct to wound closure following resection of extremity STS with ulceration/impending ulceration. PMID:27347212

  1. Osteosarcoma With Apparent Ewing Sarcoma Gene Rearrangement.

    PubMed

    Mathias, Melissa D; Chou, Alexander J; Meyers, Paul; Shukla, Neerav; Hameed, Meera; Agaram, Narasimhan; Wang, Lu; Berger, Michael F; Walsh, Michael; Kentsis, Alex

    2016-07-01

    Poorly differentiated round cell sarcomas present diagnostic challenges because of their variable morphology and lack of specific immunophenotypic markers. We present a case of a 15-year-old female with a tibial tumor that exhibited features of Ewing-like sarcoma, including apparent rearrangement of the EWSR1 gene. Hybridization capture-based next-generation DNA sequencing showed evidence of complex genomic rearrangements, absence of known pathogenic Ewing-like chromosome translocations, and deletions RB1, PTCH1, and ATRX, supporting the diagnosis of osteosarcoma. This illustrates the potential of clinical genomic profiling to improve diagnosis and enable specifically targeted therapies for cancers with complex pathologies. PMID:27352193

  2. [Sarcomas, example of a pathologist network organization].

    PubMed

    Neuville, Agnes; Coindre, Jean-Michel

    2013-12-01

    Sarcomas are rare and heterogeneous with many subtypes explaining the high level of diagnostic difficulty with frequent important therapeutic consequences. In 2009, a national network of pathologists has been set up with the main objective to perform a systematic histological review of every new sarcoma, gastro-intestinal stromal tumor (GIST) and desmoid tumor. We describe the network organization and report the results of the first two years of activity. These results clearly show the interest of this organization for the patients as well as for all pathologists. Moreover, data and material collect allows a better knowledge of these tumors and an improvement of the rules for their diagnostic management.

  3. Retroperitoneal Sarcoma: Fact, Opinion, and Controversy.

    PubMed

    Gladdy, Rebecca A; Gupta, Abha; Catton, Charles N

    2016-10-01

    After diagnosis of retroperitoneal sarcoma (RPS), detailed imaging and multidisciplinary discussion should guide treatment including surgical resection and in select cases, neoadjuvant therapy. Local recurrence is common in RPS and is associated with grade, histologic subtype, completeness of resection, and size. As guidelines to standardize RPS patient management emerge, expert pathologic assessment and management in centers of excellence are benchmarks of quality of care. The efficacy of current chemotherapy is limited and there is a critical need to understand the molecular basis of sarcoma so that new drug therapies are developed. Multicenter clinical trials are needed to limit opinion and controversy in this complex and challenging disease. PMID:27591493

  4. Systemic Therapy for Advanced Soft Tissue Sarcoma.

    PubMed

    Sheng, Jennifer Y; Movva, Sujana

    2016-10-01

    Soft tissue sarcomas are rare tumors that present with distant metastasis in up to 10% of patients. Survival has improved significantly because of advancements in histologic classification and improved management approaches. Older agents such as doxorubicin, ifosfamide, gemcitabine, and paclitaxel continue to demonstrate objective response rates from 18% to 25%. Newer agents such as trabectedin, eribulin, aldoxorubicin, and olaratumab have demonstrated improvements in progression-free survival, overall survival, or toxicity profiles. Future studies on treatment of advanced soft tissue sarcoma will continue to concentrate on reducing toxicity, personalization of therapy, and targeting novel pathways. PMID:27542647

  5. Primary cutaneous Ewing sarcoma--case report.

    PubMed

    Oliveira Filho, Jayme de; Tebet, Ana Carolina Franco; Oliveira, Anna Rita Ferrante Mitidieri de; Nasser, Kassila

    2014-01-01

    Ewing sarcoma is a primitive neuroectodermal tumor rarely occurs in the skin and sobcutaneous tissues. Generally Ewing's sarcoma is a primary bone tumor, but when present in soft tissues it characterizes an extremely uncommon clinical picture. It usually involves the deep subcutaneous tissue or muscles, and more rarely occurs like a primary skin cancer. Most patients are white, women, and in the second decade of life. The clinical features are a superficial mass, in average measuring 2-3 cm, of soft consistency, freely mobile and sometimes painful. The more affected locations are upper and lower extremities, trunk, head, neck or multiple lesions. The presence of metastases is very rare. PMID:24937829

  6. State-of-the-art approach for bone sarcomas.

    PubMed

    Mavrogenis, Andreas F; Angelini, Andrea; Vottis, Christos; Palmerini, Emanuela; Rimondi, Eugenio; Rossi, Giuseppe; Papagelopoulos, Panayiotis J; Ruggieri, Pietro

    2015-01-01

    Bone sarcomas are a variety of non-epithelial, malignant neoplasms of bone. The most common bone sarcomas are osteosarcoma, Ewing's sarcoma, and chondrosarcoma. The approach to a patient with a suspected bone sarcoma from initial examination to the histological diagnosis and classification is staging. Staging is of critical importance, in order to classify different treatment options and point out which combination of them is more suitable depending on the severity of the tumor in every individual patient. Staging should include medical history, physical and imaging examination, and biopsy. This article presents the current approach for staging, principles of biopsy, tumor classification, treatment, and follow-up of patients with bone sarcomas.

  7. Synovial sarcoma of kidney in a child: A rare presentation

    PubMed Central

    Radhakrishnan, Venkatraman; Dhanushkodi, Manikandan; Narayanswamy, Kathiresan; Raja, Anand; Sundersingh, Shirley; Sagar, Tenali

    2016-01-01

    There are no reported cases in the literature of primary renal synovial sarcoma in pediatric patients. The management of renal synovial sarcoma has been extrapolated from the management of soft tissue sarcomas at other sites. We present a 4-year-old female who was suspected to have Wilms’ tumor. The patient underwent guided biopsy as she did not respond to neoadjuvant chemotherapy for Wilms’ tumor. The biopsy was consistent with primary renal synovial sarcoma. The child was treated with change in her neoadjuvant chemotherapy regimen and surgery. The diagnosis of synovial sarcoma was confirmed by demonstrating the t (X, 18) translocation using polymerase chain reaction. PMID:27046979

  8. Observation, Radiation Therapy, Combination Chemotherapy, and/or Surgery in Treating Young Patients With Soft Tissue Sarcoma

    ClinicalTrials.gov

    2014-09-08

    Adult Alveolar Soft-part Sarcoma; Adult Angiosarcoma; Adult Epithelioid Sarcoma; Adult Extraskeletal Chondrosarcoma; Adult Extraskeletal Osteosarcoma; Adult Fibrosarcoma; Adult Leiomyosarcoma; Adult Liposarcoma; Adult Malignant Fibrous Histiocytoma; Adult Malignant Hemangiopericytoma; Adult Malignant Mesenchymoma; Adult Neurofibrosarcoma; Adult Synovial Sarcoma; Childhood Alveolar Soft-part Sarcoma; Childhood Angiosarcoma; Childhood Epithelioid Sarcoma; Childhood Fibrosarcoma; Childhood Leiomyosarcoma; Childhood Liposarcoma; Childhood Malignant Mesenchymoma; Childhood Neurofibrosarcoma; Childhood Synovial Sarcoma; Dermatofibrosarcoma Protuberans; Metastatic Childhood Soft Tissue Sarcoma; Nonmetastatic Childhood Soft Tissue Sarcoma; Stage I Adult Soft Tissue Sarcoma; Stage II Adult Soft Tissue Sarcoma; Stage III Adult Soft Tissue Sarcoma; Stage IV Adult Soft Tissue Sarcoma

  9. ARID1A loss correlates with mismatch repair deficiency and intact p53 expression in high-grade endometrial carcinomas

    PubMed Central

    Allo, Ghassan; Bernardini, Marcus Q; Wu, Ren-Chin; Shih, Ie-Ming; Kalloger, Steve; Pollett, Aaron; Gilks, C Blake; Clarke, Blaise A

    2015-01-01

    BAF250a (ARID1A) loss is a frequent event in high-grade endometrial cancers. It has been proposed that ARID1A is a driver gene, with ARID1A mutations occurring secondary to deregulated mismatch repair mechanism in gastric cancers, representing an alternative oncogenic pathway to p53 alteration. The prognostic significance of ARID1A loss is controversial. In this study, we investigated the frequency of BAF250a immunohistochemical loss in a cohort of high-grade endometrial cancers (n = 190) and correlated it with mismatch repair (hMLH1, hMSH2, hMSH6, and hPMS2) and p53 protein expression. The 190 cases consisted of 82 high-grade endometrioid, 88 serous, 10 clear cell, and 10 mixed (carcinosarcomas and mixed histology). There was BAF250a loss in 55/190 (29%) cancers, most commonly in high-grade endometrioid carcinomas (46 vs 9% in serous carcinomas, P<0.0001). Loss of any mismatch repair proteins was observed in 63/190 (33%) cancers, most commonly in high-grade endometrioid carcinomas (57 vs 10% in serous carcinomas, P<0.0001). Aberrant p53 expression was found in 86/190 (45%) cancers, more commonly in serous carcinomas (77 vs 18% in high-grade endometrioid carcinomas, P<0.0001). BAF250a loss was associated with mismatch repair loss (P<0.0001) and normal p53 expression (P<0.0001). These associations were maintained in the subset analysis within the high-grade endometrioid (P = 0.026 and P = 0.0083, respectively) and serous carcinoma cases (P = 0.0031 and P<0.0001, respectively). Survival analysis revealed a superior progression-free survival (P = 0.017) for patients with BAF250a loss within the entire cohort but not within the high-grade endometrioid and serous subtypes. Additionally, data from The Cancer Genome Atlas were extracted to correlate mutations in ARID1A, TP53, and MMR genes; we found that ARID1A mutations were negatively associated with TP53 mutations but were unrelated to mismatch repair gene mutations. In conclusion, BAF250a loss is more common in high-grade

  10. Prevalence of Human Papillomavirus Genotypes Among Women With High-Grade Cervical Lesions in Beijing, China.

    PubMed

    Xiao, Meizhu; Xu, Qiuxiang; Li, Hongyan; Gao, Huiqiao; Bie, Yachun; Zhang, Zhenyu

    2016-01-01

    The aim of the study is to investigate the prevalence of high-risk human papillomavirus (hr-HPV) genotypes among Han women with high-grade cervical lesions in Beijing, China.Cervical cell specimens from patients with histopathologically confirmed cervical lesions at 7 hospitals in Beijing were examined with a validated HPV kit for 13 hr-HPV genotypes during the study period. The patients were divided into a low-grade cervical lesions group (cervical intraepithelial neoplasia grade 1, CIN1) and a high-grade cervical lesions group (CIN2+, including cervical intraepithelial neoplasia grade 2, CIN2; cervical intraepithelial neoplasia grade 3, CIN3; squamous cervical cancer, SCC; and adenocarcinoma of the cervix, ACC) based on the histopathology results.A total of 2817 eligible patients were enrolled, including 610 cases identified as CIN1 and 2207 as CIN2+. The hr-HPV positive rates in the CIN1 and CIN2+ groups were 78.2% (477/610) and 93.3% (2060/2207), respectively. The most frequently detected genotypes were HPV16, 58, 52 and18 in the CIN1 group and HPV16, 58, 33, and 52 in the CIN2+ group, in descending order of prevalence. In addition, the prevalence of HPV18 among the patients with ACC was 28.6% (14/49), significantly >7.2% (54/752) prevalence among the SCC patients (P < 0.001). Additionally, significantly more women in the CIN2+ group had multiple infections compared with those in the CIN1 group (38.1% and 24.9%, respectively; P < 0.001). However, as the cervical lesion grade increased, the prevalence of multiple hr-HPV infections gradually deceased to 44.2% in the CIN2 patients, 36.7% in the CIN3 patients, and 35.3% in the cervical cancer (CC) patients, which included SCC and ACC patients. In cases of multiple hr-HPV infections in the CIN2+ group, double infections accounted for ∼76.6%, and HPV16+58, HPV16+52, and HPV16+18 were the most common combinations, in descending order. The most frequent combination for triple infections was HPV16+58+31, with

  11. Retinoblastoma pathway deregulatory mechanisms determine clinical outcome in high-grade serous ovarian carcinoma.

    PubMed

    Milea, Anca; George, Sophia H L; Matevski, Donco; Jiang, Haiyan; Madunic, Mary; Berman, Hal K; Gauthier, Mona L; Gallie, Brenda; Shaw, Patricia A

    2014-07-01

    Alterations in the retinoblastoma pathway are frequent in ovarian/tubal high-grade serous cancers, but the mechanism of deregulation and the impact on patient outcome are poorly understood. A cohort of 334 high-grade serous carcinomas was studied by immunohistochemical analysis of RB1, p16, cyclin D1, cyclin E1, and Ki67. Additional detailed analyses including RB1 allelic deletion (n=42), mutation (n=75), methylation (n=31), and SNP array analyses (n=75) were performed on cases with clinical parameters, including age, debulking status, treatment, and clinical outcome. p16/RB1 expression results yielded three distinct clinically relevant subgroups upon multivariable analysis controlling for stage, debulking status, and treatment types: p16 homogeneous/RB1+ with the shortest progression-free survival (median 15 months (95% CI: 13-18); P=0.016) compared with the p16 heterogeneous/RB1+ subgroup (median 22 months (95% CI: 16-32)) and the p16 homogeneous/RB1- subgroup (median 20 months (95% CI: 15-24)). Patients in the p16 homo/RB1- subgroup showed a significant increase in overall survival (>60 months; P=0.013), which suggests an increase in sensitivity to cytotoxic agents. Analyses of Rb pathway mechanistic differences among these groups revealed frequent RB1 genomic alterations such as RB1 allelic loss and/or large spanning deletions (83%) in the p16 homo/RB1- subgroups, also indicating that RB1 deletions are frequent in high-grade serous carcinoma. CCNE1 gene gains/amplifications were frequent in the p16 homogeneous/RB1+ subgroup (68%) and cyclin D1 protein overexpression was predominantly characteristic of the p16 heterogeneous/RB1+ subgroup. These subcategories occur early in tumor progression and are seen with similar frequency in the cancer precursor lesion, serous tubal intra-epithelial carcinoma. Overall, this study uniquely identifies multiple non-synonymous mechanisms of retinoblastoma pathway deregulation that correlate with significantly different clinical

  12. Prevalence of Human Papillomavirus Genotypes Among Women With High-Grade Cervical Lesions in Beijing, China

    PubMed Central

    Xiao, Meizhu; Xu, Qiuxiang; Li, Hongyan; Gao, Huiqiao; Bie, Yachun; Zhang, Zhenyu

    2016-01-01

    Abstract The aim of the study is to investigate the prevalence of high-risk human papillomavirus (hr-HPV) genotypes among Han women with high-grade cervical lesions in Beijing, China. Cervical cell specimens from patients with histopathologically confirmed cervical lesions at 7 hospitals in Beijing were examined with a validated HPV kit for 13 hr-HPV genotypes during the study period. The patients were divided into a low-grade cervical lesions group (cervical intraepithelial neoplasia grade 1, CIN1) and a high-grade cervical lesions group (CIN2+, including cervical intraepithelial neoplasia grade 2, CIN2; cervical intraepithelial neoplasia grade 3, CIN3; squamous cervical cancer, SCC; and adenocarcinoma of the cervix, ACC) based on the histopathology results. A total of 2817 eligible patients were enrolled, including 610 cases identified as CIN1 and 2207 as CIN2+. The hr-HPV positive rates in the CIN1 and CIN2+ groups were 78.2% (477/610) and 93.3% (2060/2207), respectively. The most frequently detected genotypes were HPV16, 58, 52 and18 in the CIN1 group and HPV16, 58, 33, and 52 in the CIN2+ group, in descending order of prevalence. In addition, the prevalence of HPV18 among the patients with ACC was 28.6% (14/49), significantly >7.2% (54/752) prevalence among the SCC patients (P < 0.001). Additionally, significantly more women in the CIN2+ group had multiple infections compared with those in the CIN1 group (38.1% and 24.9%, respectively; P < 0.001). However, as the cervical lesion grade increased, the prevalence of multiple hr-HPV infections gradually deceased to 44.2% in the CIN2 patients, 36.7% in the CIN3 patients, and 35.3% in the cervical cancer (CC) patients, which included SCC and ACC patients. In cases of multiple hr-HPV infections in the CIN2+ group, double infections accounted for ∼76.6%, and HPV16+58, HPV16+52, and HPV16+18 were the most common combinations, in descending order. The most frequent combination for triple infections was HPV16

  13. The genetic signatures of pediatric high-grade glioma: no longer a one-act play

    PubMed Central

    Diaz, Alexander K.; Baker, Suzanne J.

    2014-01-01

    Advances in understanding pediatric high-grade glioma (pHGG) genetics have revealed key differences between pediatric and adult high-grade gliomas (aHGGs), and have uncovered unique molecular drivers among subgroups within pHGG. The three core aHGG pathways, the receptor tyrosine kinase(RTK)/Ras/Phosphatidylinositide 3-kinase (PI3K), p53, and retinoblastoma (RB) networks, are also disrupted in pHGG, but they exhibit a different spectrum of effectors targeted by mutation. There are also similarities and differences in the genomic landscape of diffuse intrinsic pontine glioma (DIPG) and pediatric non-brainstem high-grade glioma (pNBS-HGG). In 2012, histone H3 mutations were identified in nearly 80% of DIPGs and ∼35% of pNBS-HGG. These were the first reports of histone mutations in human cancer, implicating novel biology in pediatric gliomagenesis. Additionally, DIPG and midline pNBS-HGG vary in the frequency and specific histone H3 amino acid substitution compared to pNBS-HGGs arising in the cerebral hemispheres, demonstrating a molecular difference among pHGG subgroups. The gene expression signatures as well as DNA methylation signatures of these tumors also carry distinctive signatures, reflecting a combination of the driving mutations and the developmental context from which they arise. These data collectively highlight unique selective pressures within the developing brainstem and solidify DIPG as a specific molecular and biological entity among pHGGs. Emerging studies continue to identify novel mutations that distinguish subgroups of pHGG. The molecular heterogeneity among pHGGs will undoubtedly have clinical implications moving forward. The discovery of unique oncogenic drivers is a critical first step in providing patients with appropriate, targeted therapies. Despite these insights, our vantage point has been largely limited to an in-depth analysis of protein coding sequences. Given the clear importance of histone mutations in pHGG, it will be interesting

  14. High-grade endometrial carcinoma: serous and grade 3 endometrioid carcinomas have different immunophenotypes and outcomes.

    PubMed

    Alkushi, Abdulmohsen; Köbel, Martin; Kalloger, Steve E; Gilks, C Blake

    2010-07-01

    High-grade endometrial carcinomas are a heterogeneous group of tumors and include grade 3 endometrioid (EC-3), serous (SC), and clear cell carcinomas (CCC). There are conflicting data about the prognosis of these subtypes of high-grade endometrial carcinoma; this may be a result of lack of reproducibility in classifying tumor cell type. The purpose of this study was to examine differences in immunophenotype and outcome in a series of high-grade endometrial carcinomas, focusing on the comparison of EC-3 versus SC. We selected 180 endometrial carcinomas of SC, EC, or CCC type. No mixed carcinomas were included in the study. We chose the following immunohistochemical markers, estrogen receptor (ER), insulin-like growth factor 2 mRNA-binding protein 3 (IMP3), p16, p53, progesterone receptor (PR), and phosphatase and tensin homolog (PTEN) as being significantly differentially expressed in endometrial carcinoma subtypes. The tumors were stratified into 4 groups on the basis of their cell type and grade: EC grade 1 or 2, EC-3, SC, and CCC. Univariate survival analysis revealed significant differences in outcome between the 4 groups (P<0.0001), with significantly longer disease-specific survival for grade 1 or 2 EC versus EC-3 (P=0.0001), and EC-3 versus SC (P=0.0003). p16, PTEN, and IMP3 expression was observed more frequently in SC compared with EC-3 (P<0.0001, P=0.021, and P=0.031, respectively). These 3 markers showed the highest sensitivity and specificity in distinguishing between EC-3 and SC, with receiver operating characteristics area under the curve of 0.85, 0.69, and 0.71, respectively. ER and p53 approached but did not reach significance for differential expression in EC-3 versus SC (P=0.055 and P=0.068, respectively). A combination of p16 and PTEN predicts EC-3 versus SC with a sensitivity of 90.0% and specificity of 96.8%. p16 and PTEN can aid in distinguishing between EC-3 and SC of the endometrium, and are superior to ER, PR, and p53 for this purpose. EC-3

  15. Prevalence of Human Papillomavirus Genotypes Among Women With High-Grade Cervical Lesions in Beijing, China.

    PubMed

    Xiao, Meizhu; Xu, Qiuxiang; Li, Hongyan; Gao, Huiqiao; Bie, Yachun; Zhang, Zhenyu

    2016-01-01

    The aim of the study is to investigate the prevalence of high-risk human papillomavirus (hr-HPV) genotypes among Han women with high-grade cervical lesions in Beijing, China.Cervical cell specimens from patients with histopathologically confirmed cervical lesions at 7 hospitals in Beijing were examined with a validated HPV kit for 13 hr-HPV genotypes during the study period. The patients were divided into a low-grade cervical lesions group (cervical intraepithelial neoplasia grade 1, CIN1) and a high-grade cervical lesions group (CIN2+, including cervical intraepithelial neoplasia grade 2, CIN2; cervical intraepithelial neoplasia grade 3, CIN3; squamous cervical cancer, SCC; and adenocarcinoma of the cervix, ACC) based on the histopathology results.A total of 2817 eligible patients were enrolled, including 610 cases identified as CIN1 and 2207 as CIN2+. The hr-HPV positive rates in the CIN1 and CIN2+ groups were 78.2% (477/610) and 93.3% (2060/2207), respectively. The most frequently detected genotypes were HPV16, 58, 52 and18 in the CIN1 group and HPV16, 58, 33, and 52 in the CIN2+ group, in descending order of prevalence. In addition, the prevalence of HPV18 among the patients with ACC was 28.6% (14/49), significantly >7.2% (54/752) prevalence among the SCC patients (P < 0.001). Additionally, significantly more women in the CIN2+ group had multiple infections compared with those in the CIN1 group (38.1% and 24.9%, respectively; P < 0.001). However, as the cervical lesion grade increased, the prevalence of multiple hr-HPV infections gradually deceased to 44.2% in the CIN2 patients, 36.7% in the CIN3 patients, and 35.3% in the cervical cancer (CC) patients, which included SCC and ACC patients. In cases of multiple hr-HPV infections in the CIN2+ group, double infections accounted for ∼76.6%, and HPV16+58, HPV16+52, and HPV16+18 were the most common combinations, in descending order. The most frequent combination for triple infections was HPV16+58+31, with

  16. Lipomatous pleomorphic adenoma in the hard palate: Report of a rare case with cyto-histo correlation and review.

    PubMed

    Musayev, Jamal; Onal, Binnur; Hasanov, Adalat; Farzaliyev, Ismayil

    2014-01-01

    Pleomorphic adenoma is the most common benign tumor of the salivary glands. They are usually composed of epithelial/myoepithelial cells and chondromyxoid stroma. Extensive lipomatous differentiation is very rare. We report a case of lipomatous pleomorphic adenoma (LPA) that presented with a mass in the hard palate of a 32-year-old woman. The fine-needle aspiration cytology material was reported as benign cytology consistent with adenoma with major adipocytic component. Histopathological examination of the excision material displayed that more than 90% of the tumor was adipocytic in texture, containing scant epithelial and myoepithelial cells and chondromyxoid stromal fragments. Preoperative cytodiagnosis of lipomatous pleomorphic adenoma on FNA is based on cytomorphology intimately associated pleomorphic adenomatous and lipomatous tissue elements. LPA should be on the mental list of the (cyto)pathologist in differential diagnosis of lipomatous tumors or non-tumorous lipomatosis or carcinoma invasion in the adipose tissue of the minor salivary gland of the hard palate.

  17. Secretion of interleukin-6 and vascular endothelial growth factor by spindle cell sarcoma complicating Castleman's disease (so-called 'vascular neoplasia').

    PubMed

    Kakiuchi, Chihiro; Ishida, Tsuyoshi; Sato, Hitoshi; Katano, Harutaka; Ishiko, Tositaka; Mukai, Hiroyuki; Kogi, Mieko; Kasuga, Naoki; Takeuchi, Kengo; Yamane, Kenichi; Fukayama, Masashi; Mori, Shigeo

    2002-06-01

    So-called 'vascular neoplasia' (VN) is a rare tumour of unknown origin that complicates hyaline vascular type Castleman's disease (CD). This paper reports a case of VN complicating CD of hyaline vascular type, in which neoplastic cells were shown to secrete interleukin-6 (IL-6) and vascular endothelial growth factor (VEGF). In this case, VN first occurred in the retroperitoneum of a 60-year-old male. The lesion showed typical morphology, with three distinct areas: (1) a lymph node-like area with regressively transformed lymph follicles showing hyaline vascular changes and with a hypervascular interfollicular region filled with slit-like vascular channels; (2) an area composed of spindle cell sarcoma; and (3) an area showing angiolipomatous hamartoma. A proportion of the cells in the spindle cell area showed severe pleomorphism. Subcutaneous recurrence after 8 months was composed purely of pleomorphic spindle cells. A karyotypic analysis of the recurrent tumour showed 47, XXY with some instability. Supernatant from primary culture contained high levels of IL-6 and VEGF, suggesting high secretion of these cytokines from neoplastic cells. Immunohistochemically, p53 overexpression was identified only in the pleomorphic spindle cells of the primary lesion and metastatic tumour. No features suggestive of vascular origin were shown on immunohistochemical or electron microscopic analysis of the neoplastic cells. Human herpesvirus type 8 was not detected by immunohistochemistry or PCR analysis. High levels of IL-6 and/or VEGF have been reported to play a role in CD. This is the first case report that clarifies the site of such cytokine production, showing the possibility of CD as a paraneoplastic phenomenon.

  18. High grade anorectal stricture complicating Crohn's disease: endoscopic treatment using insulated-tip knife

    PubMed Central

    Chon, Hyung Ku; Shin, Ik Sang; Kim, Sang Wook

    2016-01-01

    Endoscopic treatments have emerged as an alternative to surgery, in the treatment of benign colorectal stricture. Unlike endoscopic balloon dilatation, there is limited data on endoscopic electrocautery incision therapy for benign colorectal stricture, especially with regards to safety and long-term patency. We present a case of a 29-year-old female with Crohn's disease who had difficulty in defecation and passing thin stools. A pelvic magnetic resonance imaging scan, gastrograffin enema, and sigmoidoscopy showed a high-grade anorectal stricture. An endoscopic insulated-tip knife incision was successfully performed to resolve the problem. From our experience, we suggest that endoscopic insulated-tip knife treatment may be a feasible and effective modality for patients with short-segment, very rigid, fibrotic anorectal stricture. PMID:27433152

  19. High grade anorectal stricture complicating Crohn's disease: endoscopic treatment using insulated-tip knife.

    PubMed

    Chon, Hyung Ku; Shin, Ik Sang; Kim, Sang Wook; Lee, Soo Teik

    2016-07-01

    Endoscopic treatments have emerged as an alternative to surgery, in the treatment of benign colorectal stricture. Unlike endoscopic balloon dilatation, there is limited data on endoscopic electrocautery incision therapy for benign colorectal stricture, especially with regards to safety and long-term patency. We present a case of a 29-year-old female with Crohn's disease who had difficulty in defecation and passing thin stools. A pelvic magnetic resonance imaging scan, gastrograffin enema, and sigmoidoscopy showed a high-grade anorectal stricture. An endoscopic insulated-tip knife incision was successfully performed to resolve the problem. From our experience, we suggest that endoscopic insulated-tip knife treatment may be a feasible and effective modality for patients with short-segment, very rigid, fibrotic anorectal stricture.

  20. High grade glioma mimicking voltage gated potassium channel complex associated antibody limbic encephalitis.

    PubMed

    Athauda, Dilan; Delamont, R S; Pablo-Fernandez, E De

    2014-01-01

    Though raised titres of voltage gated potassium channel (VGKC) complex antibodies have been occasionally associated with extracranial tumours, mainly presenting as Morvan's Syndrome or neuromyotonia, they have not yet been reported to be associated with an intracranial malignancy. This is especially important as misdiagnosis of these conditions and delay of the appropriate treatment can have important prognostic implications. We describe a patient with a high grade glioma presenting with clinical, radiological, and serological features consistent with the diagnosis of VGKC antibody associated limbic encephalitis (LE). This is the first association between a primary brain tumour and high titre of VGKC complex antibodies. Clinicoradiological progression despite effective immunosuppressive treatment should prompt clinicians to look for alternative diagnoses. Further studies to elucidate a possible association between VGKC complex and other surface antigen antibodies with primary brain tumours should be carried out.

  1. Integrated Proteogenomic Characterization of Human High-Grade Serous Ovarian Cancer.

    PubMed

    Zhang, Hui; Liu, Tao; Zhang, Zhen; Payne, Samuel H; Zhang, Bai; McDermott, Jason E; Zhou, Jian-Ying; Petyuk, Vladislav A; Chen, Li; Ray, Debjit; Sun, Shisheng; Yang, Feng; Chen, Lijun; Wang, Jing; Shah, Punit; Cha, Seong Won; Aiyetan, Paul; Woo, Sunghee; Tian, Yuan; Gritsenko, Marina A; Clauss, Therese R; Choi, Caitlin; Monroe, Matthew E; Thomas, Stefani; Nie, Song; Wu, Chaochao; Moore, Ronald J; Yu, Kun-Hsing; Tabb, David L; Fenyö, David; Bafna, Vineet; Wang, Yue; Rodriguez, Henry; Boja, Emily S; Hiltke, Tara; Rivers, Robert C; Sokoll, Lori; Zhu, Heng; Shih, Ie-Ming; Cope, Leslie; Pandey, Akhilesh; Zhang, Bing; Snyder, Michael P; Levine, Douglas A; Smith, Richard D; Chan, Daniel W; Rodland, Karin D

    2016-07-28

    To provide a detailed analysis of the molecular components and underlying mechanisms associated with ovarian cancer, we performed a comprehensive mass-spectrometry-based proteomic characterization of 174 ovarian tumors previously analyzed by The Cancer Genome Atlas (TCGA), of which 169 were high-grade serous carcinomas (HGSCs). Integrating our proteomic measurements with the genomic data yielded a number of insights into disease, such as how different copy-number alternations influence the proteome, the proteins associated with chromosomal instability, the sets of signaling pathways that diverse genome rearrangements converge on, and the ones most associated with short overall survival. Specific protein acetylations associated with homologous recombination deficiency suggest a potential means for stratifying patients for therapy. In addition to providing a valuable resource, these findings provide a view of how the somatic genome drives the cancer proteome and associations between protein and post-translational modification levels and clinical outcomes in HGSC. VIDEO ABSTRACT. PMID:27372738

  2. Concurrent use of endourological and radiologic methods in the management of high-grade renal trauma

    PubMed Central

    Akbari, Ardalan; Heran, Manraj K.S.; Afshar, Kourosh

    2016-01-01

    This case report explores the efficacy of simultaneous use of endourological and radiological methods to assess and manage high-grade renal trauma. A male rugby player was diagnosed with Grade 4 blunt renal trauma. A segment of the patient’s kidney was isolated from the main renal pelvis with intact perfusion. This resulted in urinary extravasation. Ureteral stenting and angioembolization were used to treat the patient’s severe symptoms. Angioembolization ablated functional tissue that was causing a persistent urinary leak. Following the treatment, the patient was discharged with no significant bleeding or leakage from the kidney. This report illustrates an uncommon use of this combined approach. Followup 18 months post-trauma revealed normal blood pressure and approximately 30% loss of volume of the affected kidney. PMID:27695587

  3. Integrated Proteogenomic Characterization of Human High-Grade Serous Ovarian Cancer.

    PubMed

    Zhang, Hui; Liu, Tao; Zhang, Zhen; Payne, Samuel H; Zhang, Bai; McDermott, Jason E; Zhou, Jian-Ying; Petyuk, Vladislav A; Chen, Li; Ray, Debjit; Sun, Shisheng; Yang, Feng; Chen, Lijun; Wang, Jing; Shah, Punit; Cha, Seong Won; Aiyetan, Paul; Woo, Sunghee; Tian, Yuan; Gritsenko, Marina A; Clauss, Therese R; Choi, Caitlin; Monroe, Matthew E; Thomas, Stefani; Nie, Song; Wu, Chaochao; Moore, Ronald J; Yu, Kun-Hsing; Tabb, David L; Fenyö, David; Bafna, Vineet; Wang, Yue; Rodriguez, Henry; Boja, Emily S; Hiltke, Tara; Rivers, Robert C; Sokoll, Lori; Zhu, Heng; Shih, Ie-Ming; Cope, Leslie; Pandey, Akhilesh; Zhang, Bing; Snyder, Michael P; Levine, Douglas A; Smith, Richard D; Chan, Daniel W; Rodland, Karin D

    2016-07-28

    To provide a detailed analysis of the molecular components and underlying mechanisms associated with ovarian cancer, we performed a comprehensive mass-spectrometry-based proteomic characterization of 174 ovarian tumors previously analyzed by The Cancer Genome Atlas (TCGA), of which 169 were high-grade serous carcinomas (HGSCs). Integrating our proteomic measurements with the genomic data yielded a number of insights into disease, such as how different copy-number alternations influence the proteome, the proteins associated with chromosomal instability, the sets of signaling pathways that diverse genome rearrangements converge on, and the ones most associated with short overall survival. Specific protein acetylations associated with homologous recombination deficiency suggest a potential means for stratifying patients for therapy. In addition to providing a valuable resource, these findings provide a view of how the somatic genome drives the cancer proteome and associations between protein and post-translational modification levels and clinical outcomes in HGSC. VIDEO ABSTRACT.

  4. High grade anorectal stricture complicating Crohn's disease: endoscopic treatment using insulated-tip knife.

    PubMed

    Chon, Hyung Ku; Shin, Ik Sang; Kim, Sang Wook; Lee, Soo Teik

    2016-07-01

    Endoscopic treatments have emerged as an alternative to surgery, in the treatment of benign colorectal stricture. Unlike endoscopic balloon dilatation, there is limited data on endoscopic electrocautery incision therapy for benign colorectal stricture, especially with regards to safety and long-term patency. We present a case of a 29-year-old female with Crohn's disease who had difficulty in defecation and passing thin stools. A pelvic magnetic resonance imaging scan, gastrograffin enema, and sigmoidoscopy showed a high-grade anorectal stricture. An endoscopic insulated-tip knife incision was successfully performed to resolve the problem. From our experience, we suggest that endoscopic insulated-tip knife treatment may be a feasible and effective modality for patients with short-segment, very rigid, fibrotic anorectal stricture. PMID:27433152

  5. Induction of high grade astrocytoma (HGA) by protons: Molecular mechanisms and RBE considerations

    NASA Astrophysics Data System (ADS)

    Dalrymple, G. V.; Leichner, P. K.; Harrison, K. A.; Cox, A. B.; Hardy, K. A.; Salmon, Y. L.; Mitchell, J. C.

    1994-10-01

    Protons of a specific energy, 55 MeV, have been found to induce primary high grade astrocytomas (HGA) in the Rhesus monkey (Macaca mulatta). Brain tumors of this type were not induced by protons of other energies (32-2,300 MeV). Induction of HGA has been identified in human patients who have had radiation therapy to the head. We believe that the induction of HGA in the monkey is a consequence of dose distribution, not some unique ``toxic'' property of protons. Comparison of the human experience with the monkey data indicates the RBE for induction of brain tumors to be about one. It is unlikely that protons cause an unusual change in oncogenic expression, as compared to conventional electromagnetic radiation.

  6. Origin of deep crystal reflections: seismic profiling across high-grade metamorphic terranes in Canada

    USGS Publications Warehouse

    Green, A.; Milkereit, B.; Percival, J.; Davidson, A.; Parrish, R.; Cook, F.; Geis, W.; Cannon, W.; Hutchinson, D.; West, G.; Clowes, R.

    1990-01-01

    In an attempt to better understand the origin of deep crustal reflections LITHOPROBE has sponsored or co-sponsored Seismic reflection surveys across tracts of high-grade metamorphic rock in the Archean Superior craton, the Proterozoic Grenville orogen and the Phanerozoic Cordilleran orogen. Common to these three diverse terranes are near-surface zones of prominent Seismic reflectivity that are typically associated with velocity discontinuities at highly strained contacts between gneissic rocks of varying lithology. At some locations the reflective layering resulted from transposition and rearrangement of previously layered rocks (stratified assemblages, sills, etc.), whereas in other regions it was generated by extreme attenuation, stretching and ductile flow of weakly layered or irregularly organized rocks. It seems likely that compositionally layered gneissic rock is a common source of reflections in the deep crust, with reflections originating at lithological boundaries and zones of mylonite. ?? 1990.

  7. Concurrent use of endourological and radiologic methods in the management of high-grade renal trauma

    PubMed Central

    Akbari, Ardalan; Heran, Manraj K.S.; Afshar, Kourosh

    2016-01-01

    This case report explores the efficacy of simultaneous use of endourological and radiological methods to assess and manage high-grade renal trauma. A male rugby player was diagnosed with Grade 4 blunt renal trauma. A segment of the patient’s kidney was isolated from the main renal pelvis with intact perfusion. This resulted in urinary extravasation. Ureteral stenting and angioembolization were used to treat the patient’s severe symptoms. Angioembolization ablated functional tissue that was causing a persistent urinary leak. Following the treatment, the patient was discharged with no significant bleeding or leakage from the kidney. This report illustrates an uncommon use of this combined approach. Followup 18 months post-trauma revealed normal blood pressure and approximately 30% loss of volume of the affected kidney.

  8. High-grade glioma management and response assessment—recent advances and current challenges

    PubMed Central

    Khan, M.N.; Sharma, A.M.; Pitz, M.; Loewen, S.K.; Quon, H.; Poulin, A.; Essig, M.

    2016-01-01

    The management of high-grade gliomas (hggs) is complex and ever-evolving. The standard of care for the treatment of hggs consists of surgery, chemotherapy, and radiotherapy. However, treatment options are influenced by multiple factors such as patient age and performance status, extent of tumour resection, biomarker profile, and tumour histology and grade. Follow-up cranial magnetic resonance imaging (mri) to differentiate treatment response from treatment effect can be challenging and affects clinical decision-making. An assortment of advanced radiologic techniques—including perfusion imaging with dynamic susceptibility contrast mri, dynamic contrast-enhanced mri, diffusion-weighted imaging, proton spectroscopy, mri subtraction imaging, and amino acid radiotracer imaging—can now incorporate novel physiologic data, providing new methods to help characterize tumour progression, pseudoprogression, and pseudoresponse. In the present review, we provide an overview of current treatment options for hgg and summarize recent advances and challenges in imaging technology. PMID:27536188

  9. Pleomorphic rhabdomyosarcoma of the heart metastatic to bone. Report of a case with fine needle aspiration biopsy findings.

    PubMed

    Ali, S Z; Smilari, T F; Teichberg, S; Hajdu, S I

    1995-01-01

    Pleomorphic rhabdomyosarcoma is an extremely rare and highly malignant neoplasm. We report an unusual case of a 14-year-old boy with skeletal metastases from a primary cardiac tumor. Fine needle aspiration biopsy of a lytic lesion in the right iliac wing showed a pleomorphic population of clusters and single cells with hyperchromatic nuclei; granular, pink cytoplasm; and isolated, large cells with whiplike or globoid cytoplasmic processes and occasional striations. Histologic, ultrastructural and radiologic features are also presented.

  10. Solitary fibrous tumor with entrapment of minor salivary gland tissue: an unusual presentation that requires exclusion of pleomorphic adenoma.

    PubMed

    Tapia, Jose Luis; Goodloe, Samuel; Margarone, Joseph E; Markiewicz, Michael R; Aguirre, Alfredo

    2011-09-01

    We report two unusual cases of solitary fibrous tumor (SFT) of minor salivary glands that microscopically mimicked pleomorphic adenoma. One of these lesions presented in the retromolar region and the other in the buccal mucosa. The microscopic features of these two tumors and their intimate relationship with regional mucous minor salivary glands posed a diagnostic challenge. Awareness of the morphological diversity of SFT coupled to a judicious use of appropriate immunohistochemical probes should prove valuable to accurately segregate SFT from pleomorphic adenoma.

  11. Pleomorphic Rhabdomyosarcoma Arising from True Vocal Fold of Larynx: Report of a Rare Case and Literature Review

    PubMed Central

    Küçüktülü, Eda; Ersöz, Şafak; Çobanoğlu, Bengü

    2016-01-01

    We present an extremely rare case of a pleomorphic rhabdomyosarcoma of the true vocal fold. The histopathological diagnosis was confirmed by immunohistochemistry. The patient was treated with radical surgery including total laryngectomy and radical neck dissection followed by postoperative radiotherapy. The clinicopathologic features of this rare malignancy are discussed together with a review of the literature. This case report and literature review highlights the more favorable prognosis of pleomorphic rhabdomyosarcoma in the larynx than in other locations. PMID:27699075

  12. Pleomorphic Rhabdomyosarcoma Arising from True Vocal Fold of Larynx: Report of a Rare Case and Literature Review

    PubMed Central

    Küçüktülü, Eda; Ersöz, Şafak; Çobanoğlu, Bengü

    2016-01-01

    We present an extremely rare case of a pleomorphic rhabdomyosarcoma of the true vocal fold. The histopathological diagnosis was confirmed by immunohistochemistry. The patient was treated with radical surgery including total laryngectomy and radical neck dissection followed by postoperative radiotherapy. The clinicopathologic features of this rare malignancy are discussed together with a review of the literature. This case report and literature review highlights the more favorable prognosis of pleomorphic rhabdomyosarcoma in the larynx than in other locations.

  13. The epidemiology of classic, African, and immunosuppressed Kaposi's sarcoma.

    PubMed

    Wahman, A; Melnick, S L; Rhame, F S; Potter, J D

    1991-01-01

    The etiology of Kaposi's sarcoma remains somewhat obscure. While lesions of classic Kaposi's sarcoma, African Kaposi's sarcoma, and immunosuppressed Kaposi's sarcoma have been found to be indistinguishable from one another, the reasons for the variations in type and severity have not been established. The origin of the spindle cell is yet to be agreed on. Geographic variation does not seem as important as ethnic variation. The very young and the very old, perhaps two ages of weakened immunity, tend to have a higher incidence of Kaposi's sarcoma. Children and AIDS patients tend to develop more virulent disease. Males tend to get Kaposi's sarcoma at higher rates than do females. Jewish and Mediterranean males have the highest incidence of classic Kaposi's sarcoma, and African Bantu have the highest incidence of African Kaposi's sarcoma, classifications which do not apply to the Kaposi's sarcoma population in the United States. Male homosexuals have much higher incidence of Kaposi's sarcoma than do male heterosexuals, but since the early 1980s, its incidence as the presenting manifestation of AIDS has decreased dramatically. There is no unequivocal association with HLA haplotype (though DR5 carriers may be at especially high risk) or evidence of family clustering. There is an impressive but not always consistent association between Kaposi's sarcoma development and immunodeficiency. Environmental factors, such as nitrite use, immunosuppression, and repeated cytomegalovirus infection, are associated with Kaposi's sarcoma, but the exact mechanism is unclear and the associations remain inconsistent. Finally, it is still unclear if there is a causative infectious agent for Kaposi's sarcoma. While cytomegalovirus has been linked to Kaposi's sarcoma, there are weaknesses in its hypothetical role as an etiologic agent as is the case for HIV itself.(ABSTRACT TRUNCATED AT 400 WORDS)

  14. Preparation of High-Grade Powders from Tomato Paste Using a Vacuum Foam Drying Method.

    PubMed

    Sramek, Martin; Schweiggert, Ralf Martin; van Kampen, Andreas; Carle, Reinhold; Kohlus, Reinhard

    2015-08-01

    We present a rapid and gentle drying method for the production of high-grade tomato powders from double concentrated tomato paste, comparing results with powders obtained by foam mat air drying and freeze dried powders. The principle of this method consists of drying tomato paste in foamed state at low temperatures in vacuum. The formulations were dried at temperatures of 50, 60, and 70 °C and vacuum of 200 mbar. Foam stability was affected by low serum viscosity and the presence of solid particles in tomato paste. Consequently, serum viscosity was increased by maltodextrin addition, yielding optimum stability at tomato paste:maltodextrin ratio of 2.4:1 (w/w) in dry matter. Material foamability was improved by addition of 0.5% (w/w, fresh weight) egg white. Because of solid particles in tomato paste, foam air filling had to be limited to critical air volume fraction of Φ = 0.7. The paste was first pre-foamed to Φ = 0.2 and subsequently expanded in vacuo. After drying to a moisture content of 5.6% to 7.5% wet base (w.b.), the materials obtained were in glassy state. Qualities of the resulting powders were compared with those produced by freeze and air drying. Total color changes were the least after vacuum drying, whereas air drying resulted in noticeable color changes. Vacuum foam drying at 50 °C led to insignificant carotenoid losses, being equivalent to the time-consuming freeze drying method. In contrast, air drying caused lycopene and β-carotene losses of 18% to 33% and 14% to 19% respectively. Thus, vacuum foam drying enables production of high-grade tomato powders being qualitatively similar to powders obtained by freeze drying.

  15. CIP2A protein expression in high-grade, high-stage bladder cancer

    PubMed Central

    Huang, Lisa P; Savoly, Diana; Sidi, Abraham A; Adelson, Martin E; Mordechai, Eli; Trama, Jason P

    2012-01-01

    Bladder cancer is one of the most common cancers in the United States. Numerous markers have been evaluated for suitability of bladder cancer detection and surveillance. However, few of them are acceptable as a routine tool. Therefore, there exists a continuing need for an assay that detects the presence of bladder cancer in humans. It would be advantageous to develop an assay with a protein that is associated with the development of bladder cancer. We have identified the cancerous inhibitor of PP2A (CIP2A) protein as a novel bladder cancer biomarker. In this study, Western blot analysis was used to assess the expression level of CIP2A protein in bladder cancer cell lines and bladder cancer patient tissues (n = 43). Our studies indicated CIP2A protein was abundantly expressed in bladder cancer cell lines but not in nontumor epithelial cell lines. Furthermore, CIP2A was specifically expressed in transitional cell carcinoma (TCC) of the bladder tumor tissues but not in adjacent nontumor bladder tissue. Our data showed that CIP2A protein detection in high-grade TCC tissues had a sensitivity of 65%, which is 3.4-fold higher than that seen in low-grade TCC tissues (19%). The level of CIP2A protein expression increased with the stage of disease (12%, 27%, 67%, and 100% for pTa, pT1, pT2, and pT3 tumor, respectively). In conclusion, our studies suggest that CIP2A protein is specifically expressed in human bladder tumors. CIP2A is preferentially expressed in high-grade and high-stage TCC tumors, which are high-risk and invasive tumors. Our studies reported here support the role of CIP2A in bladder cancer progression and its usefulness for the surveillance of recurrence or progression of human bladder cancer. PMID:23342256

  16. Zircon ion microprobe dating of high-grade rocks in Sri Lanka

    SciTech Connect

    Kroener, A.; Williams, I.S.; Compston, W.; Baur, N.; Vitanage, P.W.; Perera, L.R.K.

    1987-11-01

    The high-grade gneisses of Sri Lanka display spectacular in-situ granulitization phenomena similar to those observed in southern India and of current interest for evolutionary models of the lower continental crust. The absolute ages of these rocks are poorly constrained and so, using the SHRIMP ion microprobe, the authors have analyzed small spots on zircons from upper amphibolite to granulite grade quartzitic and pelitic metasediments. Detrital grains from a metaquartzite of the Highland Group preserve premetamorphic U-Pb ages of between 3.17 and 2.4 Ga and indicate derivation of the sediment from an unidentified Archean source terrain. The Pb-loss patterns of these zircons and the other samples suggest severe disturbance at ca 1100 Ma ago, which the authors attribute to high-grade regional metamorphism. Two pelitic gneisses contain detrital zircons with ages up to 2.04 Ga and also record an approx. = 1100 Ma event that is also apparent from metamorphic rims around old cores and new zircon growth. A granite intrusive into the Highland Group granulites records an emplacement age of 1000-1100 Ma as well as metamorphic disturbance some 550 Ma ago but also contains older, crustally derived xenocrysts. Zircons from a metaquartzite xenolith within the granitoid Vijayan Complex are not older than approx. 1100 Ma; therefore the Vijayan is neither Archean in age nor acted as basement to the Highland Group, as previously proposed. The authors suggest that the Vijayan Complex formed significantly later than the Highland Group and that the two units were brought into contact through post-1.1 Ga thrusting. Although the granulitization phenomena in India and Sri Lanka are similar, the granulite event in Sri Lanka is not Archean in age but took place in the late Proterozoic.

  17. Preparation of High-Grade Powders from Tomato Paste Using a Vacuum Foam Drying Method.

    PubMed

    Sramek, Martin; Schweiggert, Ralf Martin; van Kampen, Andreas; Carle, Reinhold; Kohlus, Reinhard

    2015-08-01

    We present a rapid and gentle drying method for the production of high-grade tomato powders from double concentrated tomato paste, comparing results with powders obtained by foam mat air drying and freeze dried powders. The principle of this method consists of drying tomato paste in foamed state at low temperatures in vacuum. The formulations were dried at temperatures of 50, 60, and 70 °C and vacuum of 200 mbar. Foam stability was affected by low serum viscosity and the presence of solid particles in tomato paste. Consequently, serum viscosity was increased by maltodextrin addition, yielding optimum stability at tomato paste:maltodextrin ratio of 2.4:1 (w/w) in dry matter. Material foamability was improved by addition of 0.5% (w/w, fresh weight) egg white. Because of solid particles in tomato paste, foam air filling had to be limited to critical air volume fraction of Φ = 0.7. The paste was first pre-foamed to Φ = 0.2 and subsequently expanded in vacuo. After drying to a moisture content of 5.6% to 7.5% wet base (w.b.), the materials obtained were in glassy state. Qualities of the resulting powders were compared with those produced by freeze and air drying. Total color changes were the least after vacuum drying, whereas air drying resulted in noticeable color changes. Vacuum foam drying at 50 °C led to insignificant carotenoid losses, being equivalent to the time-consuming freeze drying method. In contrast, air drying caused lycopene and β-carotene losses of 18% to 33% and 14% to 19% respectively. Thus, vacuum foam drying enables production of high-grade tomato powders being qualitatively similar to powders obtained by freeze drying. PMID:26189747

  18. Mechanisms for folding of high-grade rocks in extensional tectonic settings

    NASA Astrophysics Data System (ADS)

    Harris, Lyal B.; Koyi, Hemin A.; Fossen, Haakon

    2002-11-01

    This review of structures developed in extensional high-grade terrains, combined with results of centrifuge analogue modelling, illustrates the range of fold styles and mechanisms for folding of amphibolite to granulite facies rocks during rifting or the collapse of a thrust-thickened orogen. Several extensional fold mechanisms (such as folding within detachment shear zones) are similar to those in contractional settings. The metamorphic P- T- t path, and not fold style or mode of formation, is therefore required to determine the tectonic setting in which some folds developed. Other mechanisms such as rollover above and folding between listric normal shear zones, and folding due to isostatic adjustments during crustal thinning, are unique to extensional tectonic settings. Several mechanisms for folding during crustal extension produce structures that could easily be misinterpreted as implying regional contraction and hence lead to errors in their tectonic interpretation. It is shown that isoclinal recumbent folds refolded by open, upright folds may develop during regional extension in the deep crust. Folds with a thrust sense of asymmetry can develop due to high shear strains within an extensional detachment, or from enhanced back-rotation of layers between normal shear zones. During back-rotation folding, layers rotated into the shortening field undergo further buckle folding, and all may rotate towards orthogonality to the maximum shortening direction. This mechanism explains the presence of many transposed folds, folds with axial planar pegmatites and folds with opposite vergence in extensional terrains. Examples of folds in high-grade rocks interpreted as forming during regional extension included in this paper are from the Grenville Province of Canada, Norwegian Caledonides, Albany Mobile Belt and Leeuwin Complex of Western Australia, Ruby Mountains in the Basin and Range Province of Nevada, the Atâ Sund area of Greenland, the Napier Complex of Enderby Land

  19. Wingspan Stent for High-Grade Symptomatic Vertebrobasilar Artery Atherosclerotic Stenosis

    SciTech Connect

    Li Jian Zhao Zhenwei Gao Guodong Deng Jianping; Yu Jia; Gao Li; Yuan Yang; Qv Youzhi

    2012-04-15

    Purpose: This study was designed to present the treatment outcomes with Wingspan stent angioplasty of high-grade intracranial vertebrobasilar artery (VBA) stenosis in symptomatic patients. Methods: Between 2007 and 2010, the records of 30 patients with 31 intracranial high-grade VBA stenoses (all{>=}70%) who underwent elective stenting due to the failure of medical therapy were retrospectively reviewed. Clinical evaluation was performed based on the modified Rankin scale and the National Institutes of Health Stroke Scale. Results: In all cases, the stent deployment was technically successful. The mean stenosis decreased significantly from 82.28 {+-} 8.02% (range, 72-99%) to 11.18 {+-} 7.28% (range, 0-25%) after stent-assisted angioplasty (P < 0.05). Periprocedure complications occurred in 3 (10%) of 30 patients; there were 2 cases of perforator strokes and 1 case of transient flow insufficiency with stent overlap. Clinical follow-up (mean, 17.81 {+-} 11.49 months; range, 5-40 months) was available for 27 patients, and angiographic follow-up (mean, 9.95 {+-} 5.74 months, range, 5-20 months) was available for 19 patients. Only one case demonstrated recurrent symptoms with restenosis ({>=}50%). There were no recurrent ischemic events and no cases of restenosis in the other patients. Conclusions: According to our data, the Wingspan stent for symptomatic intracranial VBA stenoses is a safe and efficacious treatment alternative in cases with recurrent symptoms despite medical therapy. However, the improvement of outcome requires the reduction in the rate of procedure-related complications and long-term outcomes still have to be demonstrated.

  20. ADC texture—An imaging biomarker for high-grade glioma?

    SciTech Connect

    Brynolfsson, Patrik; Hauksson, Jón; Karlsson, Mikael; Garpebring, Anders; Nyholm, Tufve; Nilsson, David; Trygg, Johan; Henriksson, Roger; Birgander, Richard; Asklund, Thomas

    2014-10-15

    Purpose: Survival for high-grade gliomas is poor, at least partly explained by intratumoral heterogeneity contributing to treatment resistance. Radiological evaluation of treatment response is in most cases limited to assessment of tumor size months after the initiation of therapy. Diffusion-weighted magnetic resonance imaging (MRI) and its estimate of the apparent diffusion coefficient (ADC) has been widely investigated, as it reflects tumor cellularity and proliferation. The aim of this study was to investigate texture analysis of ADC images in conjunction with multivariate image analysis as a means for identification of pretreatment imaging biomarkers. Methods: Twenty-three consecutive high-grade glioma patients were treated with radiotherapy (2 Gy/60 Gy) with concomitant and adjuvant temozolomide. ADC maps and T1-weighted anatomical images with and without contrast enhancement were collected prior to treatment, and (residual) tumor contrast enhancement was delineated. A gray-level co-occurrence matrix analysis was performed on the ADC maps in a cuboid encapsulating the tumor in coronal, sagittal, and transversal planes, giving a total of 60 textural descriptors for each tumor. In addition, similar examinations and analyses were performed at day 1, week 2, and week 6 into treatment. Principal component analysis (PCA) was applied to reduce dimensionality of the data, and the five largest components (scores) were used in subsequent analyses. MRI assessment three months after completion of radiochemotherapy was used for classifying tumor progression or regression. Results: The score scatter plots revealed that the first, third, and fifth components of the pretreatment examinations exhibited a pattern that strongly correlated to survival. Two groups could be identified: one with a median survival after diagnosis of 1099 days and one with 345 days, p = 0.0001. Conclusions: By combining PCA and texture analysis, ADC texture characteristics were identified, which seems