Cases reported "Brain Neoplasms"

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1/98. Cerebral B-cell lymphoma following treatment for tolosa-hunt syndrome.

    We herein report a unique case of cerebral lymphoma which occurred after lymphocytic neuritis of cranial nerves causing tolosa-hunt syndrome and demonstrate the histological difference between these two diseases. A 70-year-old woman developed a sensory disturbance in the first and third divisions of the left trigeminal nerve and a left ocular movement disturbance five years before death. Although she was clinically diagnosed to have a schwannoma in the left cavernous sinus, a histologic examination verified a diffuse infiltration of T lymphocytes in the left trigeminal ganglion. Corticosteroid therapy was effective. Thereafter she demonstrated a disturbance of consciousness and dysphasia four years after surgery. A T1-weighted magnetic resonance image (MRI) disclosed high intensity lesions in both the basal ganglia and corpus callosum. She also showed progressive spastic paralysis. At autopsy a diagnosis of primary intracranial B-cell lymphoma was made. Although there was no invasion of the lymphoma cells into the left trigeminal nerves, a mild inflammatory infiltration of T cells still remained.
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2/98. Intracranial germ cell tumors in children with and without down syndrome.

    PURPOSE: Two Chinese children with down syndrome affected by intracranial germ cell tumors are described. Because they represent two of eight affected patients in the current series from 1990 to 1996, it is postulated that such occurrence may be more than a coincidental event. patients AND methods: Two children with down syndrome developed germ cell tumors in atypical intracranial sites that affected basal ganglion and cerebellum. The pathology showed germinoma and yolk sac tumor, respectively. These were treated by radical surgical resection and chemotherapy with cisplatin, etoposide, and bleomycin, but without radiotherapy. RESULTS: One patient survived 3 years without radiologic evidence of tumor. The other died from infective complications caused by severe myelosuppression after chemotherapy. CONCLUSIONS: Subtle neurologic manifestations in developmentally handicapped patients with intracranial space-occupying lesions could result in delayed diagnosis. Children with down syndrome suffering from brain tumors may have a higher chance for germ cell tumors. Assay for alpha-fetoprotein and beta-human chorionic gonadotrophin could hasten diagnosis in some cases. This observation and review of literature suggest an increased risk of developing intracranial germ cell tumors in subjects with down syndrome.
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3/98. Primary cerebral neuroblastoma. A clinicopathological study of 35 cases.

    A series of 35 primary cerebral neuroblastoma is reported. These rare tumours occur most often in children in the first half of the first decade. Grossly the tumors are often massive, discrete, lobular, firm and cystic. Histologically three variants, largely determined by the extent and distribution of the fibrous connective tissue stroma, are recognized: (1) a classical variant, which most resembles the peripheral neuroblastoma and is characterized by a high frequency of Homer Wright rosettes and a relatively high frequency of ganglionic differentiation; (2) a desmoplastic variant, which is characterized by an intense connective tissue stroma; and (3) a transitional variant, in which both the classical and the desmoplastic features may be present within the same case, either concurrently or consecutively. Both the desmoplastic and the transitional forms are less likely to exhibit differentiation to mature ganglion cells, but the importance of identifying the primitive cell elements as neuroblasts is emphasized. With rare exceptions, this can be established only by specific silver impregnations on frozen material. Occasionally the direction of growth may be largely leptomeningeal. Seven illustrative clinical histories with pathological correlations are described. The over-all clinical behaviour of these tumours is that of malignant neuroepithelial neoplasms, characterized by a high recurrence rate. recurrence may, however, be a late development, in some cases occurring five or seven years after apparently successful surgical removal. The tumour shows shows a high incidence of metastatic spread, almost 40 per cent of the cases examined at autopsy having disseminated in the cerebrospinal pathways. Exceptionally, extraneural metastases may also develop. However, long post-operative survival occasionally occurs, and the subsequent clinical course is not always predictable in the individual case. The differential diagnosis is briefly discussed. The cellular nature of the tumour and its biological behaviour recall those of the cerebellar medulloblastoma. Post-operative radiation to the entire neuraxis should be considered for these neoplasms.
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4/98. Extraventricular neurocytoma with ganglionic differentiation associated with complex partial seizures.

    We report an unusual case of extraventricular ("cerebral") neurocytoma with ganglion cells located in the right temporal lobe in a 9-year-old girl with complex partial seizures and precocious puberty. CT showed a calcified mass with central cystic zones. MR imaging showed a markedly hyperintense predominately solid tumor on both T1- and T2-weighted images, without appreciable contrast enhancement. Cerebral neurocytomas are histologically benign and radical surgery is curative; they should be included in the differential diagnosis of temporal lobe tumors in children.
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5/98. Malignant supratentorial ganglioglioma (ganglion cell-giant cell glioblastoma): a case report and review of the literature.

    BACKGROUND: From both epidemiologic and pathologic viewpoints, gangliogliomas exhibiting components of giant cell glioblastomas are extraordinary neoplasms. We report herein the case of a 6-year-old girl who presented initially with a World health Organization grade IV anaplastic ganglioglioma (a mixed ganglion cell tumor-giant cell glioblastoma). Despite aggressive management, the patient died of disease in a relatively short period. methods: Formalin-fixed, paraffin-embedded tissue blocks were sectioned at 5 microm for histochemical and immunohistochemical analyses. hematoxylin-eosin-stained sections and immunohistochemically stained sections from the primary and secondary resections were reviewed. Reactivity for glial fibrillary acidic protein, neurofilament protein, synaptophysin, and Ki67 nuclear antigen was evaluated. RESULTS: Histologically, 2 distinct cell populations were noted on both the primary and secondary resections. The primary resection revealed a neoplasm having a predominant glial component consistent with a glioblastoma. Interspersed were dysmorphic ganglion cells supporting a diagnosis of ganglioglioma. The second resection (following therapy) demonstrated a much more prominent dysmorphic ganglion cell component and a subdued glial component. CONCLUSION: Although immunohistochemical analysis clearly distinguished the 2 tumor cell populations, the identification of Nissl substance in neurons proved to be equally helpful. Although other cases of grade III gangliogliomas and rare cases of grade IV gangliogliomas have been reported, the present case is exceptional in that, to our knowledge, it is the only report of a patient who presented initially with a composite grade IV ganglioglioma and who was clinically followed up to the time of death. This case allows direct comparison between the histologic findings in a giant cell glioblastoma and a ganglioglioma and documents the aggressive biologic behavior of this complex neoplasm.
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6/98. A distinctive glioneuronal tumor of the adult cerebrum with neuropil-like (including "rosetted") islands: report of 4 cases.

    Four examples of a novel glioneuronal neoplasm are presented. All tumors affected adults (including two males and two females aged 25-40 years) as supratentorial, cerebral hemispheric masses with associated seizure activity and, in one case, symptoms of raised intracranial pressure and progressive hemiparesis. CT scans in two cases revealed hypodense masses without calcification. MRI scans at presentation demonstrated, in all cases, solid T1-hypointense and T2-hyperintense tumors with mass effect in one instance but no edema or contrast enhancement. Only one was relatively circumscribed on neuroradiologic study. All were infiltrative in their histologic growth pattern and predominantly glial in appearance, being composed mainly of fibrillary, gemistocytic, or protoplasmic astroglial elements of WHO grade II to III. Their distinguishing feature was their content of sharply delimited, neuropil-like islands of intense synaptophysin reactivity inhabited and rimmed in rosetted fashion by cells demonstrating strong nuclear immunolabeling for the neuronal antigens NeuN and Hu. These cells included small, oligodendrocyte-like ("neurocytic") elements as well as larger, more pleomorphic forms. Two cases contained, in addition, well-differentiated neurons of medium to ganglion-cell size. Proliferative activity was observed principally within the glial compartment; two cases contained mitotic figures and exhibited relatively elevated MIB-1 indices (6.8% and 8.2%). One of the latter progressed and proved fatal at 30 months following subtotal resection and radiotherapy. The three other patients are alive at intervals of 14 to 83 months, two tumor-free and one with extensive disease associated with the appearance of enhancement on MRI. This glioneuronal tumor variant may pursue an unfavorable clinical course.
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7/98. Dysembryoplastic neuroepithelial tumor. A case report.

    Dysembryoplastic neuroepithelial tumor (DNT) is a rare, benign tumor encountered in the cortex. It is characterized by the presence of cells of different histogenesis. Due to its mixed nature (glial-neuronal), WHO histological classification of brain tumors included it into the group of neuronal and glial-neuronal mixed tumors. Case of tumor in a 19-year-old woman experiencing for three years seizure of temporal lobe epilepsy is presented. A cranial magnetic resonance imaging (MRI) showed "pseudocystic" tumor in temporal lobe. Histological and immunocytochemical examinations of the tumor fragment removed during surgery revealed large numbers of neuronalglial nodules occurring in the cerebral cortex. Columns of glial-neuronal structures crossing parallely to the cortex surface, surrounded by oligodendrocyte-like cells (OLC) were a characteristic feature of the tumor texture. In the tumor interstitium, "floating" maturated, dysplastic-free ganglionic cells were visible in numerous bright spaces. In addition, numerous lobuliform--structured areas consisted of oligodendrocyte-like cells. Oligodendrocyte-like cells were characterized by positive immunoreaction to the presence of S-100 protein and synaptophysin. Basing on clinical manifestation and histopathological findings dysembryoplastic neuroepithelial tumor was diagnosed.
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8/98. ganglioglioma in a patient with Turcot syndrome. Case report.

    A 33-year-old woman with Turcot syndrome harbored a brain tumor and colon cancer and had a familial history of this syndrome. On histological examination, the brain tumor was found to have large and diffusely scattered ganglion cells within a diffuse background of astrocytic cells in a fibrillary matrix. The tumor was diagnosed as a ganglioglioma. No germline mutation in the adenomatous polyposis coli gene was detected using a protein truncation assay. These findings indicate that this patient had brain tumor-polyposis syndrome Type 1 of Turcot syndrome. This is the first report of a ganglioglioma related to Turcot syndrome.
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9/98. Pineal ganglioneuroblastoma in an adult.

    A 57-year-old male with pineal ganglioneuroblastoma was reported. The tumor was composed of a predominant ganglioneuromatous component and a small neuroblastic component. Primary ganglioneuroblastoma of the pineal region is extremely rare. To our knowledge, only three cases have been documented. This case may have a good prognosis because there is a predominant ganglioneuromatous component defined by both neurofilament triplet proteins and synaptophysin. The clinical significance of the neuronal differentiation in the present case and pathological considerations are discussed.
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10/98. January 2000: 12 year old boy with recent onset seizures.

    A 12-year old boy presented with new onset of seizures and a CT scan showed a left frontal lobe tumor which was removed completely. Neuropathological examination showed a pleomorphic ganglion cell tumor with necrosi, and endothelial proliferation. The diagnosis was extraventricular atypical neurocytic neoplasm ("cystic ganglioneurocytoma").
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