Cases reported "Ependymoma"

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1/23. Supratentorial desmoplastic ependymoma with giant ependymal rosettes.

    We report a case of a 22-year-old female who presented with a solid tumor in the frontal lobe having no continuity with the wall of the lateral ventricle. The tumor was excised. The patient has been free from clinical symptoms and tumor recurrence for over nine years. Microscopically, the tumor was composed of extremely large ependymal true rosettes, resembling medulloepithelioma, and thick fibrous septa, which were surrounded by thick reactive gliosis. There were no histological signs of malignancy. In our case, the tumor is assumed to be a variant of gliofibroma for which we propose the term "desmoplastic ependymoma".
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2/23. Supratentorial giant cell ependymoma.

    Ependymomas account for 3 to 9% of all neuroepithelial tumors and, although occurring most often within the ventricular system, they may arise from the extraventricular parenchyma as well. Several histologic patterns of these neoplasm are well known, but little attention has been devoted to a variant composed of giant elements. We describe the case of a 13-year-old girl experiencing a 3-month history of partial seizures in whom cranial magnetic resonance imaging showed an extraventricular, right parietotemporal neoplasm, adherent to the overlying dura mater. Histologic, immunohistochemical and ultrastructural findings were consistent with those of a high-grade ependymoma. The tumor was characterized by the presence of a major component of pleomorphic giant cells, as also seen in pleomorphic xantoastrocytoma, subependymal giant cell astrocytoma and glioblastoma multiforme. Similar elements have been described in two filum terminale and one supratentorial, intraventricular ependymoma, respectively. Histologic and evolutional data of those and of our own case suggest that isolated giant cells are not necessarily linked to a bad prognosis in ependymomas.
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3/23. Intraosseous sacral myxopapillary ependymoma and the differential diagnosis of sacral tumors.

    Although involvement of other regions of the spinal cord and brain stem is seen, myxopapillary ependymoma is most commonly found at the filum terminale or cauda equina. Less commonly, myxopapillary ependymoma may occur outside the central nervous system from direct metastatic extension of an intrathecal tumor, and rarely it may present as a primary tumor outside the thecal sac. The authors present a case of primary sacral myxopapillary ependymoma, which was first diagnosed as a chordoma. They then discuss the magnetic resonance imaging findings of this and other sacral tumors. Myxopapillary ependymoma should be considered in the differential diagnosis for a primary expansile sacral mass along with other lesions such as chordoma, aneurysmal bone cyst, and giant cell tumor.
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4/23. Bifocal myxopapillary ependymoma of the terminal filum: the end of a spectrum? Case report.

    Myxopapillary ependymomas represent the most frequent type of ependymomas found at the conus medullaris-cauda equina-terminal filum level. They are neuroectodermal tumors mainly observed during the fourth decade of life. Pediatric cases have been rarely described at an age range of 10 to 13 years. Myxopapillary ependymomas are typically solitary tumors involving the terminal filum. Simultaneous discovery of two tumors located both on the terminal filum has been reported once. The pathogenesis of this focal ependymoma located at the same embryological level, on the terminal filum, is uncertain; it may represent one end of a spectrum, the other end being the giant ependymoma of the terminal filum.
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5/23. Highly anaplastic extraventricular ependymoma arising in an adult, mimicking metastatic adenocarcinoma with heavy stromal inflammation and emperiporesis.

    We report a case of extraventricular ependymoma arising in a 50-year-old woman that took an aggressive clinical course with recurrence three times. The initial tumor was a well-circumscribed nodule in the right temporal white matter measuring 2 cm in diameter. It showed variegated histological findings mimicking metastatic adenocarcinoma: an epithelioid arrangement of highly pleomorphic cells with pseudopapillary structures and perivascular pseudorosettes, and bizarre multinucleated giant cells with occasional emperiporesis surrounded by abundant mononuclear inflammatory cells, as well as a focal small area of conventional ependymoma. Emperiporesis and abundant mononuclear cell infiltration were not previously described in an ependymoma. The recurrent tumors predominantly showed an epithelioid pattern with frequent formation of astroblastoma-like pseudopapillary structures. Neoplastic cells were markedly atypical and had characteristic intracytoplasmic eosinophilic inclusion bodies. Much of the cells in both the initial and recurrent tumors showed a positive immunostaining for glial fibrillary acidic protein (GFAP) with accentuation to the cytoplasmic processes of the pseudorosettes and pseudopapillary structures. Epithelial membrane antigen (EMA) highlighted the epithelial differentiation of the tumor cells, while cytokeratin was completely negative. Although this tumor might be classified to at least WHO grade III from the histology and aggressive behavior, the exact grading is still controversial because of the rarity of such cases.
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6/23. Epithelioid ependymoma: a new variant of ependymoma: report of three cases.

    OBJECTIVE: To describe the pathological features of three very similar and unusual primary central nervous system tumors that are not readily recognized as conventional ependymomas but which, by ultrastructural examination, have an ependymomatous character. methods: Three distinctive tumors were found in a review of our files for cases of ependymoma. In each case, hematoxylin and eosin-stained sections were reviewed, and immunostains for epithelial membrane antigen, cytokeratin, vimentin, and glial fibrillary acidic protein were performed on formalin-fixed, paraffin-embedded sections. Electron microscopy was performed in each case. RESULTS: The tumors had a diffuse myxoid background, often containing tightly clustered cells that mimicked multinucleated giant cells, but lacking perivascular pseudorosettes or central lumen rosettes. glial fibrillary acidic protein and vimentin immunostains did not reveal perivascular processes. Epithelial membrane antigen immunostains showed a dot-like cytoplasmic immunoreactivity in some cell clusters in two of the three cases. Cytokeratin was negative in all three cases. However, ultrastructurally, the cells of each tumor had extensive surface microvilli; the giant cell-like clusters had cells with extensive close appositions, some junctions, and, in two cases, lumina with microvilli. Two of the patients were adults (both with temporal lobe tumors), and one patient was 13 years old and had a cervical spinal cord intramedullary tumor. Each tumor was sharply circumscribed from adjacent central nervous system tissue but was not encapsulated. One of the cases in an adult was mitotically highly active; this tumor recurred locally 4 years after initial gross total excision. CONCLUSION: These tumors are unusual variants of ependymoma. This pattern of ependymoma is sufficiently distinctive to be recognized in hematoxylin and eosin stains once the architecture of the epithelioid clusters is appreciated.
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7/23. Giant cell ependymoma of the spinal cord. Case report and review of the literature.

    Several rare histological variants of ependymoma have been described. The authors report on a patient in whom cervical spinal cord astrocytoma was originally diagnosed after evaluation of a limited biopsy specimen. More abundant tissue obtained during gross-total resection included areas of well-differentiated ependymoma. The histological features of the tumor were extremely unusual, with a major component of pleomorphic giant cells. Its histological, immunohistochemical, and electron microscopic features, however, were consistent with ependymoma. Only two cases of terminal filum and two of supratentorial giant cell variant of ependymoma have been reported. To the authors' knowledge, this represents the first case of giant cell ependymoma of the spinal cord. The clinical significance is the potential for misdiagnosis with anaplastic (gemistocytic) astrocytoma, especially in cases in whom limited biopsy samples have been obtained.
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8/23. The use of neoadjuvant chemotherapy to achieve complete surgical resection in recurring supratentorial anaplastic ependymoma.

    INTRODUCTION: ependymoma is a central nervous system neoplasm that is often managed with surgery and radiotherapy. The benefits of chemotherapy in treating this tumor remain poorly defined. CASE REPORT: The use of a platinum-based chemotherapy as a neoadjuvant treatment to permit complete surgical resection of a supratentorial anaplastic ependymoma recurring for the third time is described. DISCUSSION: This paper also discusses the possible use of chemotherapy as a strategy that may allow tumor shrinkage and ease tumor resection in giant recurring ependymomas.
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9/23. Infratentorial giant cell ependymoma: a rare variant of ependymoma.

    We describe a giant cell ependymoma occurring in a 50-year-old man. The mass was located in the posterior aspect of the foramen magnum, extending from the cerebellar tonsil to the upper cervical spine. The tumor was a highly cellular neoplasm showing biphasic histology. Diffuse sheets of non-cohesive atypical giant cells, having eccentrically located single or multiple nuclei and plump eosinophilic cytoplasm, partly infiltrated the desmoplastic inflammatory stroma. Parts of perivascular pseudorosette-forming or pseudopapillary areas were composed of atypically elongated cells, which looked like conventional anaplastic ependymoma. There was a transitional area between two patterns. Numerous mitoses and focal necrosis were observed. Immunohistochemically, the tumor cells were immunoreactive for glial fibrillary acidic protein, vimentin, S-100 protein, and CD99. None of the tumor cells showed immunoreactivity for epithelial membrane antigen except for the intracytoplasmic lumen of a few vacuolated cells. Ultrastructurally, tumor cells were ependymal in nature; we noted cytoplasmic intermediate filaments and intercellular microrosettes with microvilli, cilia, and long zonula adherens. The features of this tumor, e.g. its superficial location, mixed giant cells, perivascular pseudorosettes or papillaries, complicated its differentiation from rhabdoid/papillary meningioma. However, immunohistochemistry and electron microscopy confirmed the diagnosis of ependymoma. The giant cell variant should be included in the subclassification of the ependymoma.
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10/23. thalidomide neuropathy in childhood.

    thalidomide was withdrawn from world markets in 1961 following recognition of its teratogenic effects. More recently, however, thalidomide treatment has been reintroduced to adult and paediatric practice for a variety of dermatologic, immunologic, rheumatologic and neoplastic disorders. Neuropathy is a significant side effect of thalidomide therapy, which may limit its clinical use. We report four cases of sensorimotor axonal neuropathy in children aged 10-15 years, treated with thalidomide for myxopapillary ependymoma, Crohn's disease and recurrent giant aphthous ulceration. thalidomide neuropathy is often associated with proximal weakness and may progress even after discontinuation of treatment, in the phenomenon of 'coasting'. Children treated with thalidomide should undergo regular neurophysiologic studies in order to detect presymptomatic or progressive peripheral neuropathy.
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