Cases reported "Supratentorial Neoplasms"

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1/8. 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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2/8. Supracerebellar transtentorial approach to posterior temporomedial structures.

    The supracerebellar transtentorial (SCTT) approach, a modification of the infratentorial supracerebellar approach, facilitates simple and minimally invasive access to posterior temporomedial structures without requiring retraction of the temporal or occipital lobe. The SCTT approach was used in 16 patients over a 3-year period. Eleven patients harbored tumors confined to, or located mainly within, the posterior hippocampal formation, three patients harbored aneurysms (one ruptured posterior cerebral artery [PCA] aneurysm at the P2-P3 junction, one ruptured giant PCA [P2] aneurysm, and one giant basilar artery-superior cerebellar artery aneurysm), one patient had juvenile-type moyamoya disease, and one patient suffered from medically intractable epilepsy. In these patients, the SCTT approach enabled tumor removal, aneurysm clipping, and vascular bypass procedures. The authors' experience suggests that this approach can be used routinely in treating lesions in the posterior temporomedial region.
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3/8. 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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4/8. Desmoplastic neuroepithelial tumor of infancy in the nevus sebaceus syndrome: report of a unique constellation and review of the literature.

    The nevus sebaceus syndrome (NSS) is a neurocutaneous disorder characterized by unilateral hyperplasia of skin appendages and skeletal hemihypertrophy, hemimegalencephaly, or hemiatrophy along with disabling seizures. Despite the proneness of the dermal stigmata to eventually undergo neoplastic transformation, the malformative lesions of the central nervous system rarely evolve into frank tumors. We present the case of a 10-year-old girl with left-sided sebaceus nevi, ipsilateral enlargement of the skull, and a desmoplastic neuroepithelial tumor (DNET) in the right fronto-parietal area of the brain. The tumor was removed by surgery. Histologically, it corresponded to a mitotically active small-cell anaplastic astrocytoma with genuine desmoplasia. Investigative methods included immunohistochemical positivity for glial fibrillary acidic protein, lack of expression of neuronal markers, and ultrastructural documentation of sheaths of basal lamina and collagen around tumor cells. A survey of the literature of brain tumors associated with NSS revealed two cases of histologically verified pilocytic astrocytomas, and one each of a choroid plexus papilloma, a mixed glioma, and a meningioma, as well as a subependymal giant cell astrocytoma--the latter possibly in an overlap syndrome of NSS and tuberous sclerosis. We hypothesize that the tumor described herein, one involving both atypical differentiation and enhanced growth potential, is paradigmatic of neuropathological events to be expected in the NSS.
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5/8. Giant supratentorial enterogenous cyst: report of a case, literature review, and discussion of pathogenesis.

    OBJECTIVE AND IMPORTANCE: To describe a histologically well-documented adult case of a giant supratentorial enterogenous cyst (EC). Fewer than 15 cases of supratentorial ECs are on record: 8 associated with the brain hemispheres or the overlying meninges, 4 with the sellar region, and 2 with the optic nerve. CLINICAL PRESENTATION: A 31-year-old woman complained of long-standing mild left brachial and crural motor deficit precipitated by headache and signs of intracranial hypertension. magnetic resonance imaging revealed a huge cyst overlying the frontoparietal brain. INTERVENTION: Symptoms were relieved by evacuation of the cyst content by means of a Rickam's reservoir, and the lesion was subsequently removed in toto. Histological and immunohistochemical examination of the cyst wall clearly established the enterogenous nature of its epithelium. Follow-up for up to 2 years after intervention showed no sign of recurrence, and symptoms, including treatment-resistant seizures in the postoperative period, have entirely subsided. CONCLUSION: Supratentorial ECs, distinctly rare in adult patients, may in some cases present as giant lesions. Total removal seems to be curative once careful examination has eliminated the possibility of a metastasis from an unknown primary. A correct histological diagnosis is important because, in contrast to other benign cysts of similar location and size, ECs may be prone to intraoperative dissemination.
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6/8. 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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7/8. Anaplastic pleomorphic xanthoastrocytomas. review of the literature with reference to malignancy potential.

    Malignancy potential of pleomorphic xanthoastrocytomas (PXAs) has rather been an underestimated reality. We report the case of a 13-year-old boy who presented with signs of increased intracranial pressure. The child had been epileptic since the age of 2. Computed tomography and magnetic resonance scans revealed a huge left frontal mass. At surgery, a subtotal excision was accomplished. Histopathological diagnosis was anaplastic PXA (grade III; WHO, 2000). The tumor showed an increased mitotic index and minimal endothelial proliferation. The patient died 3.5 months later due to a fatal intracranial hemorrhage. A review of the entire PXA literature revealed 15 well-documented cases of PXA with subsequent malignant transformation and 11 cases of primary anaplastic PXA. The prognosis was grim for both subsets of patients. Anaplastic PXAs clearly represent the transition between the original PXA concept and lipidized giant-cell glioblastoma.
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8/8. Supratentorial giant cell ependymoma: a case report.

    Ependymomas are neoplasms of the central nervous system that are capable of demonstrating remarkably heterogeneous histologic features. These tumors originate from ependymal cells lining the ventricles, the choroid plexus, the central canal of the spinal cord, and the filum terminale, so they are therefore seen throughout the neuraxis. We describe the case of a 26-year-old man who experienced a 3-week history of right-sided numbness and a 1-week history of worsening bifrontal headache. Computed tomographic scanning and magnetic resonance imaging of his head demonstrated an irregularly enhancing mass involving the left medial frontal lobe, with extension across the corpus callosum and expansion into the body and atrium of the left lateral ventricle. Histologic, immunohistochemical, and electron microscopic findings were consistent with an anaplastic ependymoma. Unique to this neoplasm was the presence of multiple tumor giant cells. The presence of pleomorphic tumor giant cells is a characteristic feature of the subependymal giant cell astrocytoma, and it is also commonly seen in pleomorphic xanthoastrocytoma and glioblastoma multiforme. Bizarre giant cells were recently described in two filum terminale ependymomas. This report presents the first case of a supratentorial giant cell ependymoma with anaplastic features.
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