1/42. 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.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
2/42. Sudden death due to a central neurocytoma.The central neurocytoma is a common, usually intraventricular tumor with bland histologic features. We report a case of a 51-year-old man who died suddenly. At autopsy, a neurocytoma with acute hemorrhage filled the anterior left lateral ventricle. The tumor matrix and surrounding brain tissue contained accumulations of hemosiderin. Previously, 2 cases of central neurocytoma with associated hemorrhage have been reported. hemorrhage appears to be a serious complication associated with these neoplasms.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
3/42. Atypical central neurocytoma: report of a case.Central neurocytomas are rare, relatively benign intraventricular neoplasms composed of uniform round cells with neuronal differentiation. The majority of previously reported central neurocytomas did not recurr after tumor removal and the patients had favorable postoperative outcomes. Only a few cases with malignant histopathology or malignant behavior have been noted. Atypical central neurocytoma is a new entity that was first described in the literature in 1997. The tumors have been noted to exhibit a Ki-67 labeling index of 2% or more, or vascular proliferation, mitoses, and necrosis, or both. Atypical histologic findings are usually associated with a somewhat less favorable clinical course and requires postoperative radiotherapy. We report a unique case of a 33-year-old man with a large intraventricular central neurocytoma. The characteristic histopathologic picture, the immunoreactivity for both synaptophysin and neuron-specific enolase, and the ultrastructural features of neuronal differentiation distinguished it from ependymoma and oligodendroglioma. The mitotic activity (up to 3 mitoses/10 high power field) and the high percentage of Ki-67-staining tumor cells (labeling index, 5.0%) in our case were consistent with the atypical variant of central neurocytoma. The patient underwent craniotomy and partial resection of the tumor. Unfortunately, he died of hydrocephalus and brain edema, the next day.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
4/42. 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").- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
5/42. Surgical management of intractable epilepsy associated with cerebral neurocytoma.Neuronal neoplasms of the CNS constitute a rarely encountered group of tumors. This report concerns the surgical management of seizures encountered in four cases (ranging from 2 to 10 years-of-age at onset; consisting of two males and two females) of a recently recognized morphologically unique tumor, called 'cerebral neurocytoma'. All patients were associated solely with intractable complex partial seizures. The tumor involved the temporal lobe in two cases, and the frontal in two. magnetoencephalography (MEG) clearly demonstrated an accumulation of equivalent current dipoles originating from the interictal spikes on the cortex around the tumor. On intra-operative electrocorticography (ECoG), the epileptogenic zone was topographically distinct from the region of the tumor. No definite ECoG activities were observed at the tumor site, although this tumor did consist of small mature neuronal cells. Either a complete or a subtotal resection of the tumor and the epileptogenic cortex was performed and, post-operatively, universal freedom from seizures was demonstrated in all patients. A histological examination of the epileptogenic cortex revealed the presence of minute cortical dysplasia or tumor involvement in the hippocampus. A resection of the epileptogenic cortex along with the tumor was thus found to improve the seizure outcome in patients with neurocytoma-associated epilepsy without inducing any identifiable neurological deficits attributable to the incremental resection.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
6/42. Papillary glioneuronal tumor.Tumors of mixed glioneuronal type are well recognized in the central nervous system. The most common examples of these lesions include gangliogliomas and dysembryoplastic neuroepithelial tumors. Recently, unusual examples of these lesions have been described, including the papillary glioneuronal tumor. This report describes a histologically similar-appearing lesion arising in the left parieto-occipital lobe of an 18-year-old man who presented with headaches and difficulties with vision. Imaging studies noted a large cystic neoplasm marked by a peripheral rim of enhancement. The patient underwent gross total resection of the tumor, which histologically was marked by a mixture of glial (glial fibrillary acidic protein-positive) and neural (synaptophysin-positive) components. Architecturally, the tumor was notable for a focal pseudopapillary pattern. Papillae were lined by predominantly glial cells, with intervening areas occupied by neurally differentiated cells. Mitotic activity, vascular proliferation, and necrosis were not noted. A MIB-1 labeling index of 1.1% was seen. p53 immunoreactivity was not observed. This report adds further evidence supporting the existence of this unusual mixed glioneuronal tumor of the central nervous system.- - - - - - - - - - ranking = 4.4403481856587keywords = central nervous system, neoplasm, nervous system (Clic here for more details about this article) |
7/42. 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.- - - - - - - - - - ranking = 2.7828402424285keywords = central nervous system, nervous system (Clic here for more details about this article) |
8/42. Cerebellar liponeurocytoma: immunohistochemical and ultrastructural study of a case.The clinical, histopathological, and ultrastructural features of a cerebellar liponeurocytoma are reported. The tumor, a 3-cm mass localized in the right cerebellar hemisphere, was resected from a 61-year-old man clinically presenting with symptoms of intracranial hypertension. The lesion was composed of small, in some areas closely packed, medulloblastoma-like cells with prominent areas of lipidization phenomena. Moreover, mitoses, cellular atypia, and numerous vascular structures were focally observed. Glial and neuronal differentiation was immunohistochemically noted (glial fibrillary acidic protein, synaptophysin, neurofilaments, and neuron-specific enolase positivity). The p53 oncoprotein was detected in the majority of neoplastic cells and a moderate proliferation activity, evaluated by Mib-1 antibody, was focally appreciated. Ultrastructural study did not show evident neuritic processes, synapses, or dense core neuroendocrine granules. This cerebellar tumor previously called lipidized medulloblastoma and recently renamed cerebellar liponeurocytoma is considered an adult neoplasm with excellent prognosis histologically presenting prominent lipidized areas and, at the immunohistochemical and ultrastructural level, both glial and neuronal differentiation. The present study provides the first description of a less differentiated and histologically more aggressive form of this unusual tumor.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
9/42. Management of neurocytomas: case report and review of the literature.The purpose of this report is to review the available literature on the presentation, pathology, and treatment of central nervous system (CNS) neurocytomas. A case report of an extraventricular neurocytoma is presented along with a comprehensive literature search of patients with a diagnosis of CNS neurocytoma. CNS neurocytomas are rare neoplasms, with fewer than 240 cases reported in the literature. The majority of neurocytomas are found in the ventricular system of the brain. immunohistochemistry is frequently used to help distinguish this tumor from other CNS neoplasms. MIB-1 proliferation index is commonly used in an attempt to predict biologic behavior. Little is known about the management of patients with this tumor, because most reports are from the pathologic literature and contain sparse information regarding clinical management. Neurocytomas are rare CNS tumors with varied biologic behavior. MIB-1 index may help direct adjuvant therapy. An excellent prognosis can be expected if a gross total resection is achieved. Postoperative radiation therapy (RT) may be considered after subtotal resection. Otherwise, RT is an option for medically inoperable or recurrent disease.- - - - - - - - - - ranking = 4.7828402424285keywords = central nervous system, neoplasm, nervous system (Clic here for more details about this article) |
10/42. Cerebellar liponeurocytoma. Case report and review of the literature.Cerebellar liponeurocytoma is a rare tumor of the posterior fossa that has many morphological similarities to medulloblastoma and neurocytoma. Recently the world health organization working group for classification of central nervous system neoplasms adopted the term "cerebellar liponeurocytoma" to provide a unified nomenclature for a tumor variously labeled in the literature as lipomatous medulloblastoma, lipidized medulloblastoma, medullocytoma. neurolipocytoma, lipomatous glioneurocytoma, and lipidized mature neuroectodermal tumor of the cerebellum. The rarity of this tumor and paucity of pertinent information regarding its biological potential and natural history have resulted in the application of various treatment modalities. It is suggested in the available literature that these lesions have a much more favorable prognosis than typical medulloblastomas, and that adjuvant therapy for liponeurocytoma need not be as extensive as that administered for medulloblastomas.- - - - - - - - - - ranking = 3.7828402424285keywords = central nervous system, neoplasm, nervous system (Clic here for more details about this article) |
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