1/72. Invasive giant cell astrocytoma of the retina in a patient with tuberous sclerosis.OBJECTIVE: To report an unusual case of giant cell astrocytoma of the retina. DESIGN: Case report. INTERVENTION: A 10-month-old girl with tuberous sclerosis was found to have bilateral astrocytic hamartomas, the right eye being prominently involved by elevated and pedunculated lesions. At 7 years of age, she had posterior subcapsular cataract, retinal detachment, and subretinal exudation develop in the right eye. At 12 years of age, her blind, painful right eye had to be enucleated because of neovascular glaucoma and a spontaneous scleral perforation. RESULTS: Histopathologic examination showed that the entire vitreous cavity was filled with a mixture of tumor, granulation tissue, and necrotic debris. Part of the tumor was composed of spindle-shaped glial cells. The remainder was composed of large gemistocytic cells that contained large atypical nuclei and copious amounts of cytoplasm, which was intensely eosinophilic in some areas. The tumor contained foci of necrosis and rare mitotic figures. It had infiltrated the parenchyma of the retrolaminar nerve and extended to the surgical margin. Areas of unequivocal choroidal invasion were also identified. The tumor cells were intensely immunoreactive for neuron-specific enolase and S-100 protein. In contrast, glial fibrillary acidic protein was only minimally positive. CONCLUSIONS: The histologic and immunohistochemical features of this retinal tumor resemble those of subependymal giant cell astrocytoma, a characteristic lesion in tuberous sclerosis. Although this unusual giant cell astrocytoma of the retina had atypical histopathologic features and local aggressive behavior, the systemic prognosis was excellent.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
2/72. Squash cytology of pleomorphic xanthoastrocytoma mimicking glioblastoma. A case report.BACKGROUND: Pleomorphic xanthoastrocytoma (PXA) is an uncommon, superficially located and well-circumscribed brain tumor that originates in astrocytic cells. Despite the fact that the tumor cells are pleomorphic, with bizarre nuclei, the clinical course is favorable. Cytologic and histologic differentiation from other high grade gliomas is necessary to determine adequate therapy during surgery. Cytomorphologic features of this tumor have been described only rarely. CASE: A 22-year-old male had complained of visual disturbance for about a year. Radiologic imaging revealed a well-circumscribed mass with cyst formation in the left temporal area. Squash specimens from fresh tissues were highly cellular. Tumor cells were markedly pleomorphic, with long and coarse cytoplasmic processes showing a fibrillary astrocytic appearance. Pleomorphic cells varied in shape from round to elongated and had large, multilobed, hyperchromatic nuclei but few nuclear mitoses. Sometimes eosinophilic granular bodies were also observed. blood vessels were found frequently in tumor cell clusters, but their endothelium was not swollen. In the background, considerable leukocytic infiltration, but no cellular debris, was observed. With immunohistochemical studies, most of the tumor cells were positive for glial fibrillary acidic protein and S-100 protein. Some of the mononuclear giant cells were positive for synaptophysin. CONCLUSION: Squash preparations showed the peculiar cytologic features of PXA. Together with the peculiar radiologic findings, the cytologic results make it possible to render a diagnosis of PXA.- - - - - - - - - - ranking = 0.14285714285714keywords = giant (Clic here for more details about this article) |
3/72. Congenital subependymal giant-cell astrocytoma: case report with prenatal ultrasonogram.BACKGROUND: A prenatal sonogram at 27 weeks of gestation revealed a brain mass along the frontal horn and body of the lateral ventricle near the foramen of Monro in the fetus. MATERIALS AND methods: A huge subependymal giant-cell astrocytoma was nearly totally resected at 11 days of age. RESULTS: There was no syndromic family history, but features substantiating the diagnosis of tuberous sclerosis were recognized at 4 years of age. CONCLUSION: The sonographic finding of a tumor in the region of the foramen of Monro should raise the suspicion of a subependymal giant-cell astrocytoma, a tumor characteristically associated with tuberous sclerosis.- - - - - - - - - - ranking = 0.85714285714286keywords = giant (Clic here for more details about this article) |
4/72. Malignant pilocytic astrocytoma in the medulla oblongata: case report.A 27-year-old woman visited our hospital with chief complaints of abducens nerve palsy and cerebellar symptoms. On computerized tomographic scanning and magnetic resonance imaging, a tumor with strong enhancement was found on the dorsal side of the medulla oblongata. A tumor was excised by suboccipital craniotomy and C1 laminectomy. Histologically, many Rosenthal fibers together with pilocytic tumor cells were found in some regions, but a very high Ki-67 labeling rate accompanied by cells with nuclei of irregular size and giant cells was observed in other regions. The tumor was diagnosed as malignant pilocytic astrocytoma originating from pilocytic astrocytoma by transformation. The biological behavior of pilocytic astrocytoma is obscure in several respects. We report our experience of a case of malignant pilocytic astrocytoma that developed in the brain stem and progressed extremely rapidly.- - - - - - - - - - ranking = 0.14285714285714keywords = giant (Clic here for more details about this article) |
5/72. Pleomorphic xanthoastrocytoma: report of a case diagnosed by intraoperative cytopathological examination.The authors present an unusual case of a 13-yr-old boy with a 3-mo history of seizures. A CT scan showed a contrast-enhancing mass located in the left temporal lobe. The patient underwent a stereotatic-guided craniotomy; intraoperative cytological diagnosis was performed by the smear technique, showing a pleomorphic xanthoastrocytoma. The tumor was totally resected. Definitive diagnosis was established by examination of paraffin-embedded material. Six months after the surgical intervention, the patient is doing well, with no radiological evidence of recurrence. The cytological differential diagnosis of giant cell lesions of the central nervous system is emphasized.- - - - - - - - - - ranking = 0.14285714285714keywords = giant (Clic here for more details about this article) |
6/72. Cranial nerve root entry zone primary cerebellopontine angle gliomas: a rare and poorly recognized subset of extraparenchymal tumors.With the exception of patients with neurofibromatosis type II, pediatric extraparenchymal cerebellopontine angle (CPA) tumors of any sort are extremely rare. Most gliomas encountered in the CPA in either children or adults involve the CPA as exophytic extensions of primary brain stem and/or cerebellar tumors. We encountered an unusual case of a giant CPA pilocytic astrocytoma arising from the proximal trigeminal nerve, completely separate from the brain stem. A nine-year-old girl with no evidence for any neurocutaneous syndrome, presented with headaches, mild obstructive hydrocephalus, trigeminal hypesthesia and a subtle peripheral facial paresis. Pre-operative neuroimaging suggested a petroclival meningioma. The tumor was completely resected via a right pre-sigmoid, retro-labyrinthine, subtemporal, transtentorial ('petrosal') approach, using intraoperative neurophysiological monitoring, with minimal morbidity. This appears to be the first reported case of a pediatric primary CPA glioma and the seventh reported case of primary CPA glioma, overall. It represents the second reported case of a primary CPA pilocytic astrocytoma. Given the findings in this case and the six other cases of primary CPA gliomas reported in the literature, as well as the results of histological studies of normal cranial nerves, we hypothesize that the point of origin of these rare and unusual tumors is the root entry zone of the involved cranial nerves. The differential diagnosis of primary CPA tumors should be expanded to include cranial nerve root entry zone primary CPA gliomas.- - - - - - - - - - ranking = 0.14285714285714keywords = giant (Clic here for more details about this article) |
7/72. Pleomorphic xanthoastrocytoma--a clinicopathological study.Six cases of Pleomorphic Xanthoastrocytoma of central nervous system are presented. One case presented with recurrent refractory seizures and others as intracranial space occupying lesions. They were located in temporal/parietal lobes, were partly cystic and partly solid on CT scan, enhancing with contrast. Histopathology was characterized by pleomorphic giant cells and cells with vacuolated cytoplasm. glial fibrillary acidic protein was strongly positive in all the cases.- - - - - - - - - - ranking = 0.14285714285714keywords = giant (Clic here for more details about this article) |
8/72. 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'.- - - - - - - - - - ranking = 0.71428571428571keywords = giant (Clic here for more details about this article) |
9/72. 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.- - - - - - - - - - ranking = 0.14285714285714keywords = giant (Clic here for more details about this article) |
10/72. Subependymal giant-cell astrocytoma in tuberous sclerosis: endoscopic images and the implications for therapy.Supratentorial intraventricular tumors are not frequently encountered in childhood. One of the most frequent intraventricular glial tumors is the subependymal giant-cell astrocytoma, mostly associated with tuberous sclerosis. These tumors are diagnosed on computed tomography (CT) or magnetic resonance imaging (MRI) scans. They can occur isolated or multiple and operative resection is advised if these tumors cause symptoms, usually raised intracranial pressure due to obstructive hydrocephalus. However, the number of tumors can be much higher than seen on radiological examination making total resection of all tumors impossible. We demonstrate this with the endoscopic images derived during the endoscopic removal of a subependymal giant-cell astrocytoma obstructing a foramen of Monro in a 15-year-old boy with tuberous sclerosis.- - - - - - - - - - ranking = 0.85714285714286keywords = giant (Clic here for more details about this article) |
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