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)

We studied the relationship of histologic features to survival in a population of 132 children with cerebellar gliomas (excluding medulloblastomas). These children had their initial tumor operation at The Children's Hospital Medical Center of Boston between 1l927 and 1968. A classification system that accentuates differences in survival was derived on the basis of clustering of histologic features and survival. This division was supported by the existence of differentially distributed symptoms and signs and by differences in resectability. The two major subgroups of cerebellar gliomas had different patterns of clinical characteristics. Cerebellar glioma A had any of the four histologic features of microcyst, leptomeningeal deposit, Rosenthal fiber, or focus of oligodendroglia; children with glioma A had a 10-year survival rate of 94%. Glioma B, encompassing the histologic features of perivascular pseudorosette, high cell density, necrosis, mitosis, and calcification, was clearly defined but histologically less uniform; children with glioma B had a 10-year survival rate of 29%. These two groups accounted for 90% of the cerebellar gliomas in childhood.
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PMID:Cerebellar gliomas in children. 84 87

A 26-year-old woman presented with headaches, incoordination and a cerebellar mass (1982). The CT scan revealed dilated ventricles and a hypodense space-occupying lesion adjacent to the fourth ventricle. Neuronal loss, gliosis and masses of Rosenthal fibers were seen in biopsy. There was no evidence of neoplasm. A second biopsy 2 years later was similar to the original specimen. A diagnosis of Alexander's disease was suggested. Later that year the patient's 11-year-old brother manifested a clinical picture initially diagnosed as brainstem glioma, but whose biopsy was characteristic of Alexander's disease. There has been a gradual deterioration of these siblings over the past 6 years (1986-1991). No evidence of neoplasm has appeared.
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PMID:Biopsy diagnosis of familial Alexander's disease. 145 72

Recently the authors have identified a major component of Rosenthal fibers as alpha B-crystallin, a major lens protein. In the current study the authors investigated the expression of alpha B-crystallin in four cultured glioma cell lines and in 115 human neuroectodermal tumors. alpha B-crystallin was expressed differentially by those glioma cell lines, but not by neuroblastoma cell lines. Northern blot analysis revealed two distinct messages for alpha B-crystallin in C-6, whereas only a single message in U-373MG and G26-24. In human surgical specimens positive immunostaining was frequently observed in the following brain tumors: pilocytic astrocytoma of the juvenile type, anaplastic astrocytoma, glioblastoma multiforme, and subependymal giant cell astrocytoma. The astrocytic elements of mixed oligoastrocytomas, glioblastomas with sarcomatous components, and gangliogliomas were likewise strongly stained. In contrast, little immunoreactivity was observed in ependymal and choroid plexus tumors. Thus, alpha B-crystallin is mainly expressed by astrocytic tumors among neuroectodermal neoplasms, without regard to the presence of Rosenthal fibers.
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PMID:Preferential expression of alpha B-crystallin in astrocytic elements of neuroectodermal tumors. 165 7

Immunohistochemically we investigated Rosenthal fibers (RFs) on specimen surgically removed from patients with glioma (three cerebellar astrocytomas, three optic gliomas, two spinal cord astrocytomas, one spinal ganglioglioma). Pathological diagnoses were pilocytic astrocytoma, fibrillary astrocytoma, and ganglioglioma. We utilized sections from the formalin-fixed paraffin-embedded tissues and stained them with H & E, PTAH, PAS as well as with anti-GFAP (glial fibrillary acidic protein) antibody (Ab) and two anti-ubiquitin Abs...anti-PHF (paired helical filament) monoclonal Ab (DF2) which recognizes ubiquitin (H. Mori in Science) and anti-ubiquitin polyclonal Ab provided by Dr. Haas. The primary antibodies were diluted with Tris-saline as follows: anti-GFAP (1:500), DF2 (culture medium without dilution), anti-ubiquitin (2 micrograms/ml). Sections were deparaffinized and incubated with primary antibodies overnight at room temperature. They were visualized by the avidin-biotin-peroxidase complex (ABC) procedure (Vectastain, Vector, USA) and counterstained with hematoxylin. Negative control sections were treated by omitting the primary antibodies. In the representative specimen we compared H & E, anti-GFAP and anti-ubiquitin staining on 3 microcrons serial sections. RFs were eosinophilic (bright red on H & E), purply-stained with PTAH (metachromasia), black with Heidemhein's iron-hematoxylin, and negative with PAS. Anti-GFAP Ab stained glial filaments diffusely in the cytoplasm and cell process of astrocytomas in every case. The peripheral parts of most RFs were intensely stained with anti-GFAP. The whole part of some RFs showed dark staining, and no part of a few RFs showed positive reactivity.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Immunohistochemical study on Rosenthal fibers in gliomas using anti-GFAP and anti-ubiquitin antibodies]. 169 68

A 50-year-old female patient died of an untreatable glioma apoplecticum. At autopsy a strongly vascularized glial tumour was found. The criteria for malignancy according to the WHO classification were only partially fulfilled by this tumour which displayed morphological features of an astrocytoma but could not be further subclassified. By light microscopy, angioma-like vascular proliferations, large cells with brightly eosinophilic cytoplasm, and small cells with hyperchromatic nuclei were found. Most large cells had vesicular, excentrically placed nuclei and contained fibrillary whorls or amorphous, irregular cytoplasmic inclusions. By immunohistochemical staining, using antibodies to glial fibrillary acidic protein (GFAP) the fibrillary whorls were identified as aggregates of glial filaments. The amorphous inclusions lacked GFAP immunoreactivity and appeared in the electron microscope as electron dense material surrounded by a dense network of glial filaments. The abnormal perikaryal inclusions of these atypical astrocytoma cells appeared to be peculiar alterations of Rosenthal fibres closely mimicing Mallory bodies.
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PMID:Peculiar changes in Rosenthal fibres in an atypical astrocytoma. 234

A case of intracerebral multifocal gliomas with von Recklinghausen's disease is reported. A 12-year-old boy was admitted to our hospital with an episode of convulsive attacks which were uncontrolled by anticonvulsants. CT scan and MRI revealed small well circumscribed tumors in the left frontal cortex and left parietal subcortex. Both of the tumors revealed low density in plain CT scan and low intensity in T1 weighted MRI. The vascularity of these tumors was poor in cerebral angiography. In other words these tumors were of a similar nature. The histology of the frontal tumor, which was totally removed surgically, showed typical pilocytic astrocytoma. The other tumor in the left parietal subcortex must also be included in the category of low grade glioma. In the sixth month after the operation, we could find neither recurrence of the frontal glioma nor enlargement of the parietal tumor, on CT and MRI findings. Immunohistochemically, the outer portion of the Rosenthal fiber in this tumor was positive for GFAP and S-100 protein, but the inner portion was negative, because the GFAP and S-100 protein there had degenerated. The cytoplasm of this tumor's cell was abundant with mitochondria and Golgi's bodies compared to the fibrillary astrocytoma. This case may be the first case of multifocal gliomas in the same cerebral hemisphere. We suggest that multifocal gliomas grow naturally, and over the years, tumors combine with each other and finally constitute a large type diffuse glioma.
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PMID:[Multifocal gliomas in cerebral hemisphere associated with von Recklinghausen's disease: case report]. 249 19

Investigation of GFAP (Glial Fibrillary Acidic Protein) in 175 brain tumours showed varying amounts of fibrillary acidic protein in every glioma. In ependymal and oligodendroglial tumours a high number of positive neoplastic elements were detected, GFAP positive were also the peri-vascular cells of a so-called astroblastoma. In pilocytic astrocytomas, Rosenthal fibers were in part GFAP positive, in part negative. In giant cells gliomas, giant cells were GFAP negative or weakly positive. Intraleptomeningeal growing tumour cells presented usually a very strong positivity. In 8 recurring oligodendrogliomas, the number of GFAP positive tumour cells was the same in the primary tumour and in its recurrence. These results demonstrate that GFAP is not a specific astrocytic, but a glial-specific protein. Although GFAP is usually present in greater concentration in differentiated, slow growing gliomas, absolute reliable predictions on biological behaviour of the individual tumour are not possible, because a high GFAP content can be detected also in malignant tumours. GFAP investigation does not seem reliable for solving the pathogenetic problems of undifferentiated tumours: the results obtained in 50 medulloblastomas showed that the investigation of small tumour samples or the positivity of a single cell are inadequate data for a correct evaluation of the findings, especially bearing in mind that GFAP of degenerated astrocytes can be phagocytised by other cells, these findings giving rise to misinterpretations.
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PMID:GFAP in brain tumor diagnosis: possibilities and limitations. 403 33

A case of a 14 month old Japanese female infant presenting with nasal glioma is reported. The tumor had been noticed at the nasal radix since birth and had slowly and progressively enlarged. There was no communication between the tumor and the cranial cavity on radiological examination. The tumor was macroscopically anchored to the nasal septum by a fibrous stalk, and histologically consisted of nests or trabeculae of either polygonal or spindle cells with plump eosinophilic cytoplasm and oval nuclei, separated by vascular-rich connective tissue intermingled with multinucleated giant cells. These tumor cells were immunohistochemically positive for glial fibrillary acidic protein as well as for S-100 protein and vimentin. An electron microscopic examination revealed collagen fibers and basal lamina between the tumor cells and the fibroblasts. Tumor cells possessed abundant intermediate filaments, which showed occasional Rosenthal fiber-like structures, in their cytoplasm and processes. A few oligodendrocytes and cilia of 9 microtubule doublets either with or without 2 central microtubules were also noted. These clinicopathological findings suggested that this tumor was once an encephalo(meningo)cele, which probably degenerated as a result of the loss of intracranial communication and then appeared to be isolated from the intracranial tissue.
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PMID:Nasal glioma: an immunohistochemical and ultrastructural study. 764 37

A 31-year old woman died after 10 years of progressive dysautonomia and cerebellar and pyramidal symptoms. CT scan showed pontine, bulbar and cerebellar atrophy. Post-mortem examination revealed Rosenthal's fibers widespread throughout the CNS, but especially in the subependymal and perivascular regions. White matter cavitations involving peri-ventricular regions, hilum of dentate nuclei and pons were observed, leading to a diagnosis of adult form of Alexander's disease. At the age of 5, the patient had been operated upon for a chiasmatic tumor. Microscopic examination revealed a pilocytic astrocytoma without Rosenthal's fibers. No complementary radiotherapy had been done. Her mother has been operated upon in 1972, for a high-grade glioma and is still alive 20 years later. This suggests diffuse cerebral gliomatosis. This family history may suggest a relation between these different diseases. They might be the result of a transmissible astrocytic abnormality with varying expression.
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PMID:[Alexander's disease in adults and diffuse cerebral gliomatosis in 2 members of the same family]. 799 38

To understand the significance of the accumulation of alpha B-crystallin in Rosenthal fibers within astrocytes, the expression and metabolism of alpha B-crystallin in glioma cell lines were examined under the conditions of heat and oxidative stress. alpha B-crystallin mRNA was increased after both stresses, and alpha B-crystallin protein moved from a detergent-soluble to a detergent-insoluble form. In addition, Western blotting of Alexander's disease brain homogenates revealed that the 27-kd heat shock protein (HSP27), which is related to alpha B-crystallin, accumulates along with alpha B-crystallin. The presence of HSP27 in Rosenthal fibers was directly demonstrated by immunohistochemistry. Our results suggest that astrocytes in Alexander's disease may be involved in an as yet unknown kind of stress reaction that causes the accumulation of alpha B-crystallin and HSP27 and results in Rosenthal fiber formation.
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PMID:Alpha B-crystallin and 27-kd heat shock protein are regulated by stress conditions in the central nervous system and accumulate in Rosenthal fibers. 839 18


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