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1/29. Central neurocytoma: 2 case reports and review of the literature.

    Central neurocytoma is a rare benign tumor of the central nervous system occuring in young adults and typically located in the ventricles. The tumor is composed of small round cells with neuronal differentiation and has a favourable prognosis. We report two cases of giant central neurocytomas with a triventricular extension in two young women. The first case concerned a 26 years old righthanded woman in whom an intraventricular mass was discovered, after a car accident with head trauma. skull radiography showed an enlargment of the sella turcica. A CT scan performed in order to examine the pituitary gland revealed a voluminous and heterogenous intraventricular tumor with calcification. The second case concerned a 26 years old righthanded woman, presenting with a 4 glasgow coma scale Score preceded by an acute onset of headache with projectile vomiting. A CT scan performed in emergency revealed a voluminous intraventricular mass with significant hydrocephalus. We review the different pathological and topographical patterns of previously published neurocytomas and discuss surgical management, effectiveness of radiation therapy and biological behavior.
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2/29. Subependymal giant cell astrocytoma: clinical and neuroimaging features of four cases.

    The clinical history, neuroimaging features, treatments, and outcome of 4 patients with histologically verified subependymal giant cell astrocytomas (SEGA) were retrospectively reviewed. The average age at the time of surgery was 13.3 years. headache related to raised intracranial pressure was the first and only sign in 2 patients, with the remaining 2 being admitted because of sequential neuroimaging studies over several years revealing the growth of 'subependymal nodules' into intraventricular tumours. In each case the tumour was in the region of Monro's foramen and was associated with ventricular dilatation. On computed tomography (CT), multiple subependymal nodules were found in 3 patients, and these well circumscribed isodense SEGAs were markedly enhanced by contrast medium. On magnetic resonance imaging (MRI), which was obtained in 3 patients, 2 SEGAs were isointense with the cerebral cortex and one with the white matter on T1-weighted images, and on T2-weighted images, 2 were isointense with the cortex and one with the white matter. At surgery the tumours appeared to originate from the inferolateral wall of the lateral ventricle in the region of the head of the caudate nuclei. Total macroscopic removal was achieved in 3 patients, and subtotal removal in one patient. Follow up ranged from 4.6 to 13.2 years, and all patients have exhibited similar physical and mental conditions to preoperative. So far there has been no evidence of any recurrences. The diagnosis and the surgical indications for SEGA are discussed, with periodic monitoring with neuroimaging studies being recommended even for asymptomatic patients with 'subependymal nodules'.
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3/29. Malignant fibrous histiocytoma of scalp with intracranial invasion 20 years after postoperative irradiation.

    We report a case of malignant fibrous histiocytoma with intracranial invasion occurring 20 years after radiation therapy for astrocytoma. magnetic resonance imaging suggested that a dumbbell-shaped tumor arising from soft tissue was pressing the brain. Histopathological findings revealed a storiform pattern with spindle cells such as fibroblasts, histiocytes, epithelioid cells with irregularly shaped nuclei, and bizarre giant cells. Three operations involving craniectomy were necessary because of recurrence.
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4/29. diffusion MR imaging of giant cell tumors in tuberous sclerosis.

    giant cell tumors were studied by diffusion MR imaging in three patients with tuberous sclerosis. diffusion MR imaging was performed on a 1.5-T unit with a gradient strength of 30 mT/m. b = 1000 seconds/mm2 images, and apparent diffusion coefficient (ADC) maps were studied. ADC values were recorded from ADC maps with particular care to obtain the values from the noncalcified regions of the giant cell tumors. ADC values of the normal brain parenchyma and hamartomas were also recorded by multiple evaluations. The ADC values in all three giant cell tumors (0.78, 0.92, and 0.92 x 10-3mm2/s) were within the ranges of the normal brain parenchyma (0.62-1.04 x 10-3mm2/s), and they were prominently less than those of parenchymal hamartomas (1.18-1.86 x 10-3mm2/s). The finding of ADC values of the noncalcified portions of the giant cell tumors being identical to those of normal brain parenchyma is consistent with the presence of normal molecular motion of water, hence relatively normal tissue integrity within these tumors. This was likely a reflection of benignity of these slowly growing tumors.
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5/29. Anterior lateral ventricular subependymal giant cell astrocytomas. Microsurgical aspects of two cases.

    The surgical management of two cases of lateral ventricular subependymal giant cell astrocytoma, arising in the head of the caudate nucleus, and causing hydrocephalus due to obstruction of the foramen of Monro is described. One lesion, in a patient with tuberous sclerosis, was resected using a transcallosal approach and the other, in a patient with no stigmata of tuberous sclerosis, using a frontal, trans-cortical transcystoventricular approach. The microsurgical aspects of excision and pathological anatomy of both tumours were very similar. Following tumour excision and pellucidotomy both patients had partial resolution of their hydrocephalus with complete resolution of their preoperative symptoms. The merits of transcallosal and transcortical approaches to these lesions are discussed.
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6/29. Subependymal giant cell astrocytoma: a clinicopathological study of 23 cases with special emphasis on proliferative markers and expression of p53 and retinoblastoma gene proteins.

    AIMS: To gain a better insight into the biological behaviour of subependymal giant cell astrocytoma (SEGA), tumour suppressor gene protein expression and various proliferative indices were studied in these tumours and correlated with histological features and clinical outcome. methods: We studied 23 cases of SEGA, 19 from our own Institute and four from the National Institute of mental health and Neurological Sciences (NIMHANS), Bangalore, india. Immunohistochemical staining for various glial and neuronal markers, proliferative markers (MIB-1, Topoisomerase II alpha PCNA) and tumour suppressor gene protein expression of p53 and retinoblastoma (Rb) were performed. RESULTS: Nineteen cases of SEGA were collected over a period of 23 years (January 1978-December 2001), which accounted for 0.16% of all intracranial tumours and 0.51% of all gliomas reported at this centre. Ages ranged from 4 to 37 years (mean 13.2 years) with a male preponderance. Nine of the 23 cases were associated with tuberous sclerosis (TS), six at the time of diagnosis, while three developed TS during the follow-up period. Treatment consisted of surgical resection (total in nine cases and subtotal in 14 cases) followed by radiotherapy in seven cases. Except for two patients who died in the immediate post-operative period of surgical complications, the remaining patients were all alive in the follow-up period (mean 37.1 months). One patient experienced recurrence 22 years after the first surgery and a second patient after 2 years. necrosis and/or mitoses were observed in five cases. Immunohistochemically, tumours were positive for both glial and neuronal markers. Interspersed inflammatory cells were a mixture of mast cells and lymphocytes of T immunophenotype. The MIB-1 labelling index (LI) ranged from 0 to 8% (mean 3.0%), topoisomerase II alpha (topo II alpha) LI from 0 to 9.5% (mean 2.9%) and PCNA LI from 10 to 59% (mean 32.5%). The difference in the labelling indices of tumours with and without mitoses and/or necrosis was not statistically significant. None of the tumours revealed loss of Rb gene protein expression. p53 immunopositivity was seen in 14 cases (labelling indices ranged from 1 to 7.3% with a mean of 2.4%). The correlation between the MIB-1 LI and topo II alpha LI, and topo II alpha LI and PCNA LI was significant (P<0.05) but not so with other parameters like p53 protein expression, duration of survival and morphological features such as mitoses and necrosis. CONCLUSIONS: SEGAs are rare intraventricular tumours associated with TS and express both neuronal and glial markers. They have a low proliferative potential. Mitoses and necrosis are not associated with a worse prognosis. In view of the low proliferative indices and long survival of these patients without recurrence, the role of post-operative radiotherapy is questionable. These patients should be followed up closely as many of them develop stigmata of tuberous sclerosis at a later stage.
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7/29. Subependymal giant cell astrocytoma: a report of five cases.

    Five cases of intraventricular subependymal giant cell astrocytomas (SEGCA) were retrospectively reviewed. Records and detailed work-up of all five cases were well maintained in view of rare occurrence of these tumors. The five patients were males aged 10, 12 (2 cases), 14 and 18 years. The 18-year-old aged male had no stigmata of tuberous sclerosis (TSC) on examination, while the rest presented with features of TSC, intraventricular tumors and hydrocephalus. None of the five cases had renal tumors and did not reveal cardiac tumors. skin manifestations like shagreen patches were present in two, facial angiofibromas in three, periungal fibromas in two, hypopigmented macules in two and ash-leaf spot in two patients. Fundoscopic examination revealed retinal astrocytomas in two cases only. Two of the five children did not have mental compromise. A child who started throwing seizures early (at 5 months of age) had severe mental retardation. Although SEGCA is a less vascular tumor, one tumor bled profusely intraoperatively; it was excised rapidly via the transcortical route to salvage the life of the child, though he was planned for the transcallosal route like the other cases. The tumor of another child was also very vascular. Four children survived surgery and required no shunt CSF diversion, while one died of severe ventriculitis and septicemia 3 weeks after surgery.
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8/29. Woman aged 24 years with fourth ventricular mass.

    April 2005. A woman aged 24 years presented with symptoms related to a tumor in the fourth ventricle. Cytologically, the tumor was biphasic with areas typical of a classic ependymoma, including rosettes, and other areas containing grossly atypical giant cells. Many tumor cells were GFAP-positive and ultrastructural examination revealed microvilli and cilia. The histopathologic abnormalities place this tumor among the ependymomas. Its focal giant cell phenotype is very rare, but has been reported in 4 intracranial or filum ependymomas.
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9/29. Subependymal giant cell astrocytoma associated with tuberous sclerosis: with special reference to cell kinetic studies--case report.

    The authors report a case of subependymal giant cell astrocytoma associated with tuberous sclerosis in a 15-year-old boy. Computed tomographic scans showed a large intraventricular mass with peritumoral calcification and a cyst in the left lateral ventricle. Left dominant unilateral hydrocephalus was also revealed. Magnetic resonance images clearly demonstrated the lesion. The tumor was subtotally removed and a ventriculoperitoneal shunt was performed because of the hydrocephalus. The proliferation potential was assessed by measuring the bromodeoxyuridine (BUdR) labeling index employing the in vitro labeling method, and determining the deoxyribonucleic acid (dna) content by flowcytometry. BUdR-positive cells were found to be rare, and the dna histogram demonstrated no evidence of high proliferative activity or aneuploidy.
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10/29. Malignant meningioma with cartilage and giant cells.

    An autopsy case of recurrent and malignant meningioma is reported. This case was originally typical benign transitional meningioma of the falx, however, the histology of the tumor changed to show malignant features during successive recurrences. At autopsy, the tumor revealed findings consistent with malignant meningioma. One of the most interesting features was the presence of cartilage and giant cells in some parts. immunohistochemistry showed positive immunoreactivity for S-100 protein in some cartilage and giant cells and for cytokeratin in some giant cells. Multidifferential potential of the meningioma cells was suggested in this case.
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