Cases reported "Infratentorial Neoplasms"

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1/8. ependymoma with extensive lipidization mimicking adipose tissue: a report of five cases.

    Lipomatous ependymoma is a recently described entity and only 3 cases of this variant have been reported in the literature. We report 5 cases of this rare variant of ependymoma. patients age ranged from 4 years to 45 years and, interestingly, all of them were males. Two tumors were supratentorial in location, 2 in the fourth ventricle and 1 was intramedullary. Microscopically all of them showed the classical histology of ependymoma along with lipomatous differentiation. The lipomatous component was composed of cells with a large clear vacuole pushing the nucleus to the periphery and giving a signet ring cell appearance. This component demonstrated positivity for GFAP and S-100 protein thereby confirming its glial lineage. Three of the 5 tumors were high grade (WHO-grade III), had a high MIB-1 labelling index (MIB-1 LI) and showed recurrence on follow-up. However, 2 were low grade (WHO grade II) and patients are free of disease till the last follow up.
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2/8. Posterior fossa ependymoma with intense but transient disseminated enhancement but not metastasis.

    We report a pediatric patient with a posterior fossa ependymoma in whom the second of two magnetic resonance imaging (MRI) scans obtained preoperatively within a 10-day span showed intense but transient disseminated enhancement. However, following posterior fossa craniotomy for near total tumor resection, neither three MRI exams nor two craniospinal fluid samplings revealed evidence of disseminated disease. Concerned that the one MRI with disseminated enhancement indicated an elevated risk of leptomeningeal metastasis, we initiated craniospinal irradiation. However, we terminated this treatment after realizing that the bulk of the clinical evidence refuted the diagnosis of metastatic disease.
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3/8. MRI findings in a case of a superficial siderosis associated with an ependymoma.

    We present the imaging findings of superficial siderosis of the central nervous system associated with an ependymoma of the posterior fossa in a patient who presented with progressive bilateral sensorineural hearing loss and cerebellar ataxia. The ependymoma was a homogeneous well-defined mass of the fourth ventricle without hydrocephalus. Secondary siderosis due to chronic bleeding from the ependymoma appeared as linear hypointensity delineating the surface of the cortex, thin and subtle on spin-echo T2-weighted images, thick and obvious on gradient-echo T2-weighted images.
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4/8. 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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5/8. MR Imaging of pial melanosis secondary to a posterior fossa melanotic ependymoma.

    A 36-year-old man presented with trouble speaking and bilateral progressive hearing loss. MR imaging and histopathologic results revealed a posterior fossa melanotic ependymoma. Pial surfaces appeared hyperintense on T1-weighted images and hypointense on T2-weighted images. Histopathologic examination revealed that tumor cells and interstitial spaces had abundant melanin accumulation. There was no evidence of hemosiderin in tumor cells and in interstitial spaces. Pial melanin accumulation secondary to a posterior fossa melanotic ependymoma explained our MR findings.
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6/8. Extra-axial ependymoma of posterior fossa extending to the Meckel's cave.

    An extra-axial ependymoma extending from the left cerebellopontine corner to the Meckel's cave is reported. This lesion's clinical, radiological, and histological characteristics are presented. This tumor's infrequent extra-axial location, extension to the Meckel's cave and possible origin are discussed.
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keywords = ependymoma
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7/8. torticollis secondary to posterior fossa and cervical spinal cord tumors: report of five cases and literature review.

    torticollis is either congenital or acquired in childhood. Acquired torticollis is not a diagnosis but rather a sign of an underlying disorder. The causes of acquired torticollis include ligamentous, muscular, osseous, ocular, psychiatric, and neurologic disorders and may be a symptom of significant abnormalities of the spinal cord and brain, such as spinal syrinx or central nervous system neoplasia. torticollis is rarely considered to be an initial clinical presentation of posterior fossa and cervical spinal cord tumors. We report five cases of pediatric tumors with torticollis at the onset: an astrocytoma originating from the medulla oblongata, another presumptive astrocytoma of the spinal cord located between C1 and C6 cervical vertebrae (not operated), an ependymoma located throughout the whole cervical spinal cord extending into the bulbomedullary junction, an astrocytoma originating from the bulbus and extending into the posterior fossa, and another case of a eosinophilic granuloma located extradurally through the anterior and posterior portions of the vertebral bodies from C3 to C7 producing the collapse of the sixth cervical vertebra. All five cases were seen in children, aged between 3 and 12 years. All these cases reflect the misinterpretation of this neurological sign and the lack of association with the possibility of spinal or posterior fossa tumor. This delay in the diagnosis of these diseases led to progressive neurological deterioration and to the increase in the tumor size, which made surgical intervention difficult and the prognosis unfavorable. Although torticollis secondary to tumors is rarely seen, it is necessary to be kept in mind in the differential diagnosis.
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keywords = ependymoma
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8/8. Catastrophic hemorrhage as the presenting symptom of an infratentorial ependymoma.

    Children with posterior fossa ependymomas most often have slowly evolving signs and/or symptoms of intracranial hypertension or cerebellar dysfunction. Rarely, however, the tumor can cause a rapidly evolving, disastrous illness.
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