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Query: UMLS:C0017636 (glioblastoma)
 18,345 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two patients, in whom visual disturbance (Case 1) and sudden hemiparalysis due to a hemorrhagic lesion (Case 2) had led to craniotomy and histological diagnosis of giant cell glioblastoma, each had an unexpectedly long survival period of 7 and 9 years, respectively. Radiologically, the tumours were well demarcated, but without any distinguishing features, by comparison with glioblastomas in general. The tumours, to a great extent, consisted of cells with large, bizarre multiple nuclei. The highly pleomorphic cells displayed strong cytoplasmic GFAP immunopositivity, which suggested an astroglial origin. Thus, these tumours were considered a variant of glioblastoma ("giant cell glioblastoma") with a more favourable prognosis than experienced by most patients with glioblastoma.
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PMID:Giant cell glioblastoma: a work-up of 2 cases with long survival. 271 38

Giant cell glioblastoma (GCG) is one of a group of rare tumors in which the cell population is abnormally large and includes multinucleated cells of gigantic sizes. Immunohistochemical studies were performed on four GCG cases and found that all giant cells and/or tumor cells were positive for glial fibrillary acidic protein (GFAP), S-100 protein, and vimentin, thus verifying the tumor's glial origin. The nuclei of multinucleated giant cells of three adult cases were frequently immunostained for proteins expressed during the cell cycle (proliferating cell nuclear antigen (PCNA) and Ki-67), thereby demonstrating the proliferative capacity of these cells. By contrast, those of a 12 year old girl expressed these cell cycle markers rather infrequently. Alpha I-antitrypsin was detected with relatively high frequency in the giant cells, and its presence may explain their bizarre sizes and pericellular reticulin fiber formation. A literature review of 32 cases revealed that the GCG that occurs preferentially in young girls is a type of pleomorphic xanthoastrocytoma. By contrast, GCG in adult males has the same age incidence as ordinary glioblastomas and, as these, expresses high levels of cell cycle-related proteins. Thus, GCG, which is subclassified morphologically as ordinary glioblastoma, has distinct biological and clinical characteristics, with that in children requiring re-evaluation because of its similarities to pleomorphic xanthoastrocytoma.
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PMID:Immunohistochemical analysis of giant cell glioblastoma. 755 Sep 96

A dark gray mass 3 cm in diameter replacing the right frontal cortex was found in the brain of a 5-year-old male Doberman Pinscher dog at necropsy. Microscopic studies revealed that the mass consisted of a proliferation of pleomorphic tumor cells: large bizarre or plump eosinophilic cells, multinucleated giant cells, and small lymphocytic cells. These neoplastic cells at the margin of the necrotic area had a psuedopalisade arrangement and tended to proliferate around blood vessels. Immunohistochemically, the tumor cells reacted intensely with the antibody for vimentin and moderately with those for S-100 and glial fibrillary acidic protein. This canine tumor is placed in the category of glioblastoma or undifferentiated astrocytoma, which is analogous to human giant cell glioblastoma.
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PMID:Giant cell glioblastoma in the frontal cortex of a dog. 777 Oct 64

Glioblastoma multiforme (GM) of the cerebellum is a rare tumour. A variant of GM, a multifocal giant cell glioblastoma, initially presenting in the cerebellum, has not previously been reported. A giant cell glioblastoma occurring in a 46-year-old man who presented initially with a cerebellar tumour is described. One month after excision of the tumour, the patient had a grand mal seizure. Computed tomography (CT) showed a low-density lesion in the left temporal lobe. Four months later he developed dysphasia and right-sided hemiparesis. Repeat CT scan revealed a large temporal lobe tumour which was excised, and histologically found to be a giant-cell glioblastoma with histopathological similarities to the original cerebellar tumour. The clinical course, computed tomographic and pathological features of this tumour are described and discussed.
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PMID:Multifocal giant cell glioblastoma: case report. 836 53

Recent studies have shown that there are distinct genetic pathways leading to the most malignant astrocytic neoplasm, the glioblastoma. Primary (de novo) glioblastomas are characterized by amplification/overexpression of the EGF receptor (EGFR) and, less frequently, of the MDM2 gene. Another pathway, operative in the progression of low-grade or anaplastic astrocytomas to secondary glioblastomas, is characterized by the frequent occurrence of p53 mutations. In this study, we assessed p53 mutations and EGFR expression in the giant cell glioblastoma. This rare variant is characterized by unusually large, multinucleated giant cells, but tends to be more confined and has been reported to carry a somewhat more favorable prognosis. We analyzed biopsies from 16 patients (mean age at clinical manifestation, 40 years). DNA sequencing revealed that 12 of 16 (75%) giant cell glioblastomas contained a p53 mutation. In 7 patients with two or more surgical interventions, the p53 mutation was already detected in the first biopsy. Focal EGFR overexpression, including multinucleated giant cells, was observed immunohistochemically in 9 of 16 (56%) tumors. However, most tumor areas lacked immunoreactivity, indicating that EGFR overexpression does not play a significant role in the evolution of this glioblastoma variant. These results suggest that giant cell glioblastomas develop de novo with a short preoperative history (mean, 47 +/- 40 days), but contain genetic alterations similar to those observed in secondary glioblastomas.
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PMID:p53 mutations versus EGF receptor expression in giant cell glioblastomas. 937 Feb 34

Giant cell glioblastoma is a rare glioblastoma variant characterized by the presence of large, bizarre, multinucleated giant cells. This glioblastoma subtype develops clinically de novo after a short clinical history and contains a high frequency of p53 mutations. In this study, we screened a series of 18 giant cell glioblastomas for additional genetic alterations. PCR-SSCP followed by DNA sequencing revealed PTEN mutations in 5 of 15 tumors (33%). Of these, two mutations were located in exon 5, two mutations in exon 6, and one mutation each in exons 1 and 9. Four mutations were point mutations and two mutations were deletions. One neoplasm contained two PTEN mutations (exons 5 and 6). None of the giant cell glioblastomas showed a homozygous deletion of PTEN orp16, or amplification of MDM2. Immunohistochemically, MDM2 overexpression was either not observed or detected in only a minor fraction of tumor cells. Differential PCR revealed EGFR amplification in only one of 17 tumors (6%). These results indicate that giant cell glioblastomas occupy a hybrid position, sharing with primary (de novo) glioblastomas a short clinical history, the absence of a less malignant precursor lesion and a 30% frequency of PTEN mutations. With secondary glioblastomas that develop through progression from low-grade astrocytomas, they have in common a younger patient age at manifestation and a high frequency (>70%) of p53 mutations.
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PMID:Genetic profile of the giant cell glioblastoma. 1006 1

Astrocytic brain tumors are the most frequent human gliomas and they include a wide range of neoplasms with distinct clinical, histopathologic, and genetic features. Diffuse astrocytomas are predominantly located in the cerebral hemispheres of adults and have an inherent tendency to progress to anaplastic astrocytoma and (secondary) glioblastoma. The majority of glioblastomas develop de novo (primary glioblastomas), without an identifiable less-malignant precursor lesion. These subtypes of glioblastoma evolve through different genetic pathways, affect patients at different ages, and are likely to differ in their responses to therapy. Primary glioblastomas occur in older patients and typically show epidermal growth factor receptor (EGFR) overexpression, PTEN mutations, p16 deletions, and, less frequently, MDM2 amplification. Secondary glioblastomas develop in younger patients and often contain TP53 mutations as their earliest detectable alteration. Morphologic variants of glioblastoma were shown to have intermediate clinical and genetic profiles. The giant cell glioblastoma clinically and genetically occupies a hybrid position between primary (de novo) and secondary glioblastomas. Gliosarcomas show identical gene mutations in the gliomatous and sarcomatous tumor components, which strongly supports the concept that there is a monoclonal origin for gliosarcomas and an evolution of the sarcomatous component due to aberrant mesenchymal differentiation in a highly malignant astrocytic neoplasm.
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PMID:Phenotype vs genotype in the evolution of astrocytic brain tumors. 1066 4

Glioblastomas, the most malignant human brain tumors, are characterized by marked aneuploidy, suggesting chromosomal instability which may be caused by a defective mitotic spindle checkpoint. We screened 22 glioblastomas for mutations in the mitotic spindle check-point genes hBUB1, hBUBR1 and hBUB3. DNA sequencing revealed a silent mutation at codon 144 of hBUB1 (CAG-->CAA, Gln-->Gln) in one glioblastoma, a silent mutation at codon 952 of hBUBR1 (GAC-->GAT, Asp-->Asp) in another glioblastoma, and a silent mutation at codon 388 of the hBUBR1 gene (GCG-->GCA, Ala-->Ala) in 8 glioblastomas. We also observed a known polymorphism at hBUBR1 codon 349 (CAA/CGA, Gln/Arg), with an allelic frequency of 0.75 for Gln and 0.25 for Arg, which is similar to that among healthy Caucasian individuals (0.73 vs 0.27). The coding sequence of the hBUB3 gene did not contain any mutation, but in 4 glioblastomas (18%), a C-->T point mutation was detected at position -6 (6 nucleotides upstream of the ATG initiator codon). Analysis of blood DNA of these patients showed identical sequence alterations, indicating that this is a polymorphism. Again, the frequency in glioblastomas was similar to that in healthy Caucasians (15%). We further screened hBUB1 in 18 cases of giant cell glioblastoma, a variant characterized by a predominance of bizarre, multinucleated giant cells. There were no changes, except for a silent mutation at codon 144 in two cases. These results suggest that mutations in these mitotic spindle checkpoint genes do not play a significant role in the causation of chromosomal instability in glioblastomas.
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PMID:Mutation analysis of hBUB1, hBUBR1 and hBUB3 genes in glioblastomas. 1135

Neurotrophins and their receptors of the Trk family play a critical role in proliferation, differentiation and survival of the developing neurons. There are reports on their expression in neoplasms too, namely, the primitive neuroectodermal tumours of childhood, and in adult astrocytic gliomas. The involvement of Trk receptors in tumour pathogenesis, if any, is not known. With this end in view, the present study has examined 10 tumour biopsy samples (identified as astrocytoma, pilocytic astrocytoma and glioblastoma) and peritumoral brain tissue of adult patients, for the presence of Trk A and Trk B receptors, by immunohistochemistry. The nature of the tumour samples was also confirmed by their immunoreactivity (IR) to glial fibrillary acidic protein. In the peritumoral brain tissue, only neurons showed IR for Trk A and Trk B. On the contrary, in the tumour sections, the IR to both receptors was localized in the vast majority of glia and capillary endothelium. There was an obvious pattern of IR in these gliomas: high levels of IR were present in the low-grade (type I and II) astrocytoma; whereas in the advanced malignant forms (WHO grade IV giant cell glioblastoma and glioblastoma multiforme) the IR was very weak. These findings suggest that Trk A and Trk B are involved in tumour pathogenesis, especially in the early stage, and may respond to signals that elicit glial proliferation, and thus contribute to progression towards malignancy.
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PMID:Expression of the neurotrophin receptors Trk A and Trk B in adult human astrocytoma and glioblastoma. 1271 10

Glioblastomas (World Health Organization, (WHO), grade IV) are the most frequent and malignant neoplasms of the human nervous system. Giant cells glioblastomas, a subtype of these, account for less than 1% of all brain toumors and up to 5% of glioblastomas. We present the case of a female who was diagnosed and treated for a right intra and paraventricular giant cell glioblastoma. We enfatize the importance of histological features of this toumor related to its prognosis.
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PMID:[Giant cell glioblastoma. Case report]. 1525 47


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