Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0017638 (glioma)
 30,880 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In this report we describe a glioblastoma multiforme with focal granular cell change. In most astroglial tumors with granular cells, the granular appearance is due to the presence of periodic acid-Schiff-positive, membrane-bound cellular debris. In the present case the granular appearance was due to the presence of many small Rosenthal fibers, which were immunoreactive for glial fibrillary acidic protein, vimentin, ubiquitin, and heat-shock protein 27, but not for alpha-B crystallin. The ultrastructural characteristics are described. These findings demonstrate that granulofilamentous inclusions with the appearance of Rosenthal fibers in glial tumors are a structurally heterogeneous feature.
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PMID:Rosenthal fibers producing a granular cell appearance in a glioblastoma. 839 34

Ganglioglioma, together with its counterparts-ganglioneuroma and gangliocytoma are relatively uncommon neoplasms of the brain composed of neoplastic neurons (ganglion and ganglioid cells) and glial cells. We report here a case of ganglioglioma studied by electron microscopy. The case was further characterized by peculiar chromosomal alterations, 46,XX[6]/43,XX[1], der(1)t(1;5)(q21;q12), der(8;13)(q10;q10),-9,i(10)(q10). Routine light microscopy revealed mixed neuro-glial tumor composed of pilocytic astrocytes with abundant Rosenthal fibers and relatively numerous ganglion cells. The latter were immunoreactive with antibodies (Abs) against synaptophysin and neurofilament protein (NFP). Anti-NFP Abs also immunostained numerous distorted axons embedded in the tumor mass. Some of these showed bullous swellings and thus were analogous to dystrophic neurites or spheroids. Ganglion cells were characterized by abundant intracytoplasmic dense-core vesicles, absence of intermediate filaments and numerous microtubules. Occasionally a close apposition of ganglion cells and Rosenthal fibers were seen. Dense-cored vesicles were pleomorphic and ranged in diameter from small synaptic vesicles to large lysosome-like neurosecretory granules. The former occasionally formed characteristic dumbbell shapes. Neoplastic astrocytes were identical to those of other glial tumors of astrocytic lineage; numerous Rosenthal fibers were frequently seen.
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PMID:The immunohistochemistry and ultrastructure of ganglioglioma with chromosomal alterations: a case report. 870 69

Standardized postembedding immunoelectron microscopy was performed to demonstrate glial fibrillary acidic protein (GFAP) and vimentin in individual intermediate filaments to determine the diagnostic value of demonstrating ultrastructural and immunophenotypic characteristics of intermediate filaments in routine brain biopsy specimens. Dual expression of GFAP and vimentin was observed in the astroblastoma and astrocytes of Alexander's disease. The antigen availability for vimentin, however, was too low to allow reliable assessment of the GFAP:vimentin ratio in individual intermediate filaments and/or filament bundles. In meningioma, only vimentin positive intermediate filaments were found. GFAP positive intermediate filaments were present in all other specimens except the oligodendroglial components of the mixed glioma, which were devoid of intermediate filaments. GFAP positivity in the filamentous periphery and electron-dense core of Rosenthal fibers was demonstrated. Technical and tissue processing factors had a significant effect on particle density values obtained for individual specimens. Although the number, distribution, and density of glial intermediate filaments varies in different astroglial entities, correlation of particle density values determined by immunoelectron microscopy with relative GFAP concentrations in different lesions requires utmost caution. Nevertheless, application of the postembedding approach to routinely fixed biopsy specimens indicated an association of different entities with the exclusive presence of GFAP and/or vimentin in individual intermediate filaments, thus emphasizing the diagnostic value of intermediate filament typing for pathological characterization.
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PMID:Semiquantitative postembedding characterization of intermediate filaments in central nervous system lesions using immunoelectron microscopy. 904 56

Neuronal differentiation is well documented in cerebellar primitive neuroectodermal tumors but is uncommon in other cerebellar neoplasms. Although rare, gangliogliomas and gangliocytomas have been previously described in the cerebellum. We report a cerebellar ganglioglioma in a 14-year-old boy, which revealed bizzare markedly pleomorphic cells with extremely pronounced nuclear atypia but less than one mitosis per 50 high-power fields and no necrosis. The tumor showed glial as well as neuronal differentiation, with abundant bi- and multinucleated ganglion cells. There were abundant Rosenthal fibers, eosinophilic granular bodies, focal calcification, and perivascular lymphocytic infiltrates. There has been no evidence of tumor recurrence or neurological deterioration 21 years after surgery, although the marked nuclear atypia led to an initial diagnosis of an anaplastic glioma. We stress the need for careful evaluation of cerebellar gliomas in children that show only nuclear atypia or endothelial hyperplasia in the absence of other features of malignancy.
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PMID:Unusual cerebellar ganglioglioma with marked cytologic atypia. 905 64

Two cases of astrocytoma associated with von Recklinghausen's disease (neurofibromatosis type; NF-1) were reported. The first case wes a 60-year-old man who had been diagnosed as von Recklinghausen's disease on the basis of skin findings. Magnetic resonance imaging (MRI) showed a tumor in the left temporal lobe. Partial removal was performed with neuronavigator, and because of the existence of Rosenthal fiber the histological diagnosis was pilocytic astrocytoma. Radiation therapy was performed. The second case was a 6-year-old boy suffering from headache and left hemiparesis including his face. MRI showed a tumor with a cyst in the right thalamus and obstructive hydrocephalus. Initially CT-guided stereotactic biopsy was performed, and the histological diagnosis, on the basis of increased cellularity, pleomorphism and nuclear atypia without necrosis or vascular proliferation, was anaplastic astrocytoma. Radiation and chemo-immuno therapy were carried out after V-P shunt. It is well known that von Recklinghausen's disease (NF-1) is often associated with optic glioma (5-36%). In the literature, the glioma seldom occurs in other parts of the brain, supratentorial glioma especially is rare. Only two familial cases of supratentorial glioma associated with von Recklinghausen's disease have been reported. The prognosis of supratentorial glioma associated with NF-1 was poor in these reports. In this paper, the diagnostic and therapeutic problems are discussed.
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PMID:[Familial astrocytoma associated with von Recklinghausen's disease: report of two cases]. 905 38

Factor analysis of reliably identified histologic features in supratentorial glial tumors yielded five interpretable "factors": Spongy, Fibrillary, Proliferative, Jumbo, and Oligodendroglial. Quantitative scores can be calculated for each factor in a tumor to summarize its heterogeneity. The objective was to investigate whether factor scores are useful for prognostic purposes. The sample consisted of 703 children with supratentorial neuroglial tumors with factor scores for each of the five factors. Data were based on the presence or absence of 26 reliably identified histologic features, plus clinical and survival information. Multivariate proportional hazards models assessed each factor's contribution to survival for children who survived 1 month after operation (n = 609). Patient-specific clinical data were allowed in the models. Increased likelihood of survival is associated with greater tumor removal, later decade of surgery, and high Spongy and high Oligodendroglial factor scores. Decreased likelihood of survival is associated with high Proliferative factor scores and radiation and/or chemotherapy treatment. Gender, age, location, and Jumbo and Fibrillary factor scores did not provide additional prognostic information. Three reliable histologic features, nondefining for any histologic factor, added prognostic information: Rosenthal fibers and glomeruli are associated with improved prognosis; pleomorphic nuclei are associated with worse prognosis. A high Oligodendroglial factor score is associated with a worse prognosis for some classes of astrocytoma but with a better prognosis for oligodendroglial tumors. A high Proliferative score is associated with a worse prognosis for anaplastic astrocytomas, ependymomas, and unclassifiable tumors. A high Spongy score is associated with a better prognosis for anaplastic astrocytomas but with a worse prognosis for pilocytic astrocytomas. For giant cell astrocytomas, gangliogliomas, and miscellaneous tumors, none of the factors is prognostic. Spongy, Oligodendroglial, and Proliferative factors provide important prognostic information for children with supratentorial neuroglial tumors.
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PMID:Prognosis for children with supratentorial neuroglial tumors. 926 88

A 33-year-old pregnant woman presented with intratumoral hemorrhage in an astrocytoma of the left optic nerve and chiasm manifesting as poor vision in her left eye. Visual examination found no light perception in the left eye and deteriorated acuity with temporal hemianopsia in the right eye. Computed tomography demonstrated a round high-density mass in the suprasellar region. T2-weighted magnetic resonance (MR) imaging showed the center of the mass as mildly hypointense and T2-weighted MR imaging as hypointense. Both T1- and T2-weighted MR imaging showed the rim of the mass as hyperintense. She delivered a healthy baby by cesarean section. A left frontotemporal craniotomy was then performed. Incision of the lateral surface of the left optic nerve revealed clotted blood from the left optic nerve and the left side of the chiasm. No vascular malformation was noted in the hematoma cavity. Histological examination revealed a neoplasm composed of well-differentiated astrocytic cells. No Rosenthal fibers were identified. The patient made an uneventful postoperative recovery. Her right visual acuity returned to normal, but the temporal hemianopsia in the right eye and the blindness in the left eye persisted. Gliomas of the anterior visual pathways are rare, especially in adults. The hemorrhage may have been caused by the hypercoagulable state associated with pregnancy. Optic nerve astrocytic glioma with intratumoral hemorrhage should be considered in the differential diagnosis of suprasellar hematoma.
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PMID:Optic nerve glioma manifesting as intratumoral hemorrhage in a pregnant woman--case report. 1470 24

We report 2 cases of an unusual central nervous system lesion involving the gyrus rectus and characterized by well-differentiated epithelial and mesenchymal components. One patient was a 30-year-old woman, and the other was a 42-year-old man. Both presented with seizures. Radiologically, the lesions were solid, with ill-defined margins and increased signal on T2-weighted MRI. Both were contrast enhancing. There was no significant mass effect or edema. Histopathologically, the epithelial component was characterized by a tubule-forming, stratified epithelium. The lumina of the tubules were lined, in some areas, by a distinct layer of mature keratinocytes. Smooth muscle actin-immunoreactive myoepithelial cells were present in the basal layer, indicating glandular differentiation. Some lumina were cystically dilated and contained anucleate squames and/or PAS-positive material. In some areas, the epithelial structures were separated by reactive glial tissue resembling pilocytic astrocytoma, with prominent, piloid processes, Rosenthal fibers, and gemistocytic astrocytes. In other areas, glial nuclei formed small clusters reminiscent of subependymoma, but no glioma was identified in either case. Tissue resembling mature adipose was present focally. No mitotic figures were identified. Follow-up interval of up to 2 years showed no evidence of recurrent tumor. By location and histologic features, this lesion appears to represent a rare, distinct clinicopathologic entity, perhaps derived from misplaced cranial neural crest cells.
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PMID:Complex choristoma of the gyrus rectus: a distinct clinicopathologic entity? 1669 17

Hemangioblastomas of the central nervous system are often accompanied by a cyst exhibiting an extensive astroglial reaction. The cyst's wall might be composed of various astroglial elements including reactive pilocytic or gemistocytic and hypertrophic astrocytes. The small tissue samples composed of compact gliotic tissue are sometimes nonrepresentative for primary hemangioblastoma tumour and might be confused with both pilocytic and diffuse infiltrative astrocytoma. Moreover, vascular anomalies of hemangioblastoma-like pattern could be combined with true neoplastic glial proliferation. Such association of glioma with certain types of vascular anomalies has been designated as angioglioma. In the current study we evaluated a series of hemangioblastomas accompanied by advanced astrogliosis of adjacent brain tissue. In some cases the histopathological features of pilocytic gliosis with numerous Rosenthal fibres and eosinophilic granular bodies strongly suggest the diagnosis of pilocytic astrocytoma. One tumour was identified as an angioglioma exhibiting a combination of hemangioblastoma-like tissue and pilocytic astrocytoma. The recognition of such an entity is important in differential tumour diagnosis and prognosis.
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PMID:Advanced reactive astrogliosis associated with hemangioblastoma versus astroglial-vascular neoplasm ("angioglioma"). 1784 62

A 16-year-old male presented to an emergency room after falling on his head while inebriated. The patient had only a history of recent fatigue and demonstrated no focal neurological deficit. MRI revealed a cystic and solid, enhancing midline cerebellar lesion. A suboccipital craniotomy was performed. Histologically, the mass showed large bizarre cells arranged in sheets with admixed small lymphocytes. The pleomorphic population had ample glassy eosinophilic cytoplasm and intranuclear inclusions. An infiltrating component resembling diffuse astrocytoma could be found in areas. Rosenthal fibers were particularly abundant in the areas of infiltrating glioma. Mitotic activity was very low, and necrosis was absent. Reticulin fibers between individual cells were focally abundant. Glial fibrillary acidic protein and vimentin were strongly expressed in many cells, while synaptophysin and neurofilament protein were not. Ki-67 showed a very low proliferation index. The pathologic diagnosis was pleomorphic xanthoastrocytoma (PXA) of the cerebellum. PXA is a diagnosis typically regarded as a superficial meningocerebral neoplasm. This case is one of sixteen cerebellar PXAs reported in the literature.
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PMID:A 16-year-old male with a cerebellar mass. 1907 85


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