Cases reported "Ganglioglioma"

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1/5. Anaplastic ganglioglioma of the cerebellopontine angle. Case report.

    A 64-year-old woman presented with a rare anaplastic ganglioglioma in the right cerebellopontine angle manifesting as dizziness persisting for 2 weeks. Preoperative magnetic resonance (MR) imaging revealed a partially enhanced cystic lesion of the right cerebellopontine angle. The tumor was subtotally removed through a right lateral suboccipital craniectomy. The tumor was thought to originate from the brain stem with exophytic growth into the right cerebellopontine angle. Histological examination showed neoplastic ganglional and glial cells with anaplastic features such as mitosis, pleomorphism, and endothelial proliferation. The MIB-1 labeling index of the glial components was 40% to 60%. The diagnosis was anaplastic ganglioglioma (world health organization grade IV). She received postoperative radiotherapy but died of respiratory failure with tumor recurrence 11 months after the operation. Gangliogliomas usually have a good prognosis. Histological features of anaplasia and a high MIB-1 labeling index may be predictive of a poor clinical outcome.
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2/5. Desmoplastic infantile ganglioglioma with high proliferation index: report of a case.

    Desmoplastic infantile ganglioglioma (DIG) is an uncommon neuroepithelial tumor associated with epilepsy, mostly occurring in the first 2 years of life. Most DIGs carry good prognosis after complete resection, even when a primitive cellular element is present. However a few examples of DIG with histologic anaplasia have recently been reported, and one demonstrated an unusual aggressive behavior. The authors describe herein a DIG with high Ki-67 proliferation index (30%) in a 10-month-old male infant with epilepsy, but with an excellent prognosis after total tumor resection.
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3/5. Malignant transformation in a ganglioglioma with anaplastic neuronal and astrocytic components. Report of a case with flow cytometric and cytogenetic analysis.

    BACKGROUND. Malignant transformation of a ganglioglioma is rare and is generally restricted to the glial component. The authors described a unique case in which neuronal and glial elements exhibited anaplasia in a ganglioglioma. A subtotal resection of a large left temporal tumor extending into the diencephalon and brain stem in a 10-year-old boy revealed a ganglioglioma with no atypical features. The histologic findings were unchanged at further resections 4 and 12 months later. radiotherapy was instituted with 5500 cGy in 30 fractions 21 months after initial resection. The patient returned 3 years later with a massive midline tumor recurrence. methods. The tumor was studied by conventional histologic methods, immunohistochemistry, flow cytometric methods, transmission electron microscopy, immune electron microscopy, and cytogenetic analysis. RESULTS. Although the first three resections revealed a typical ganglioglioma, the fourth resection revealed a cellular pleomorphic tumor with many multinucleated cells and mitoses. The tumor cells expressed glial fibrillary acid protein (GFAP) and synaptophysin on double labeling. By electron microscopy, intermediate filaments, microtubules and abundant rough endoplasmic reticulum, and neurosecretory granules were seen. Immune electron microscopy showed GFAP and synaptophysin within tumor cells. Flow cytometric studies revealed G0G1, 78%; S-phase, 9%; and G2M, 13%. Tumor cytogenetics on short term cultures revealed a complex abnormal karyotype with three sublines containing several structural chromosomal abnormalities. CONCLUSIONS. A unique anaplastic transformation of a ganglioglioma is reported with the anaplastic cells exhibiting neuronal and astrocytic features.
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4/5. Composite pleomorphic xanthoastrocytoma and ganglioglioma: report of four cases and review of the literature.

    Composite pleomorphic xanthoastrocytoma (PXA)-ganglioglioma (GG) is a rare recently described entity. Only three examples have been documented, one of which showed evidence of malignant transformation. We report an additional four cases and update the literature. With the exception of an 82-year-old man, all patients have been under 30 years of age. The temporal lobe was involved in three cases and cerebellum in another three. Radiologic features were those common to PXA and GG. Histologically, all were "collision tumors" composed of abutting, although spatially distinct, PXA and GG components. In two cases, the second element was only recognized at reexcision or recurrence. Histologic anaplasia, always in the PXA component, was evident as brisk mitotic activity and/or necrosis in five cases. Of the seven patients, one died of disease 17 years after the onset of seizures and after multiple recurrences, the last of which largely resembled glioblastoma. We conclude that the composite PXA-GG is a rare neoplasm that shares many features of its individual components. In addition to its temporal lobe predilection, the cerebellum is frequently affected. As when it occurs in isolation, the PXA component of composite PXA-GG possesses the potential for malignant transformation.
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5/5. Intracranial and spinal metastases from a ganglioglioma with unusual cytogenetic abnormalities in a patient with complex partial seizures.

    We describe an unusual clinical presentation of a ganglioglioma in a patient with complex partial seizures. The patient underwent a right temporal lobectomy with subtotal tumor resection at age 15 years, followed by a complete resection 1 year later. Follow-up MRI scan a year later documented recurrence and leptomeningeal dissemination. Another biopsy was performed. Pathological examination revealed similar histology in all three resections, with a ganglioglioma showing no evidence of anaplasia. The tumor exhibited a number of karyotypic abnormalities, notably, a paracentric inversion of chromosome 7. In summary, despite lacking anaplastic features by conventional histological criteria, this ganglioglioma showed an unsusual karyotype and demonstrated radiological evidence of widespread dissemination.
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