Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0017636 (glioblastoma)
 18,345 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Pleomorphic xanthoastrocytoma (PXA), a tumor most often presenting superficially over the cerebral hemisphere of young subjects, has certain morphological similarities to fibrous histiocytoma (or fibrous xanthoma) of the meninges and brain, namely the occurrence of lipid-laden neoplastic cells and, frequently, a dense reticulin fiber network. The detection of glial fibrillary acidic (GFA) protein in the tumor cells helped to establish its astrocytic derivation, but it has been advanced that, in spite of this agreed observation, the tumor should still be regarded as a fibrous xanthoma of meningeal origin. Although many patients have a long symptom-free postoperative survival, local recurrences at varying intervals after surgery have been noted in some instances. Weldon-Linne et al. first reported that such a recurrence had the morphology of a small-cell glioblastoma. We are reporting three further examples of locally recurrent neoplasms in patients whose original meningocerebral tumors had the typical features of PXA; the recurrences (developing 7 months, 7 years and 15 years, respectively, after surgery) were small-cell glioblastomas. The rich reticulin network present in the initial tumor was mostly lost in the recurrences. This anaplastic evolution further confirms the astrocytic nature of the PXA.
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PMID:Histopathological features of recurrent pleomorphic xanthoastrocytomas: further corroboration of the glial nature of this neoplasm. A study of 3 cases. 281

Three cases of primary intracranial tumours fulfilling the clinical and histological criteria of pleomorphic xanthoastrocytoma are presented. As is typical, they occurred in young people, aged 15, 17, and 22 years, and were composed of lipid-laden pleomorphic cells with frequent bizarre multinucleated forms and a prominent reticulin network. However, subsequent immunohistochemistry, further histological review, and clinical follow-up suggest that these tumours were different entities. In one case the tumour cells were negative for GFAP but positive with a panel of histiocytic markers. The lesion, which extended rapidly and caused death within 6 months, was assumed to be a true meningeal fibrous histiocytoma. The remaining two cases were positive for glial fibrillary acidic protein (GFAP) and S100 protein. One of these was mitotically active, contained areas of necrosis and vascular proliferation, and also led rapidly to death. This, we concluded, was a glioblastoma. The third case showed little mitotic activity and the patient remains well; this is probably a true pleomorphic xanthoastrocytoma. These results indicate that tumours with light-microscopic appearance of pleomorphic xanthoastrocytoma require detailed immunohistochemical investigation. Only those lesions with low mitotic activity and undoubted evidence of glial origin should be accepted as true pleomorphic xanthoastrocytoma. Extensive necrosis in a tumour with GFAP-positive, lipid-rich cells indicates a lipidised glioblastoma, while positive histiocytic immunocytochemistry should suggest a fibrous histiocytoma.
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PMID:Pleomorphic xanthoastrocytoma. Immunohistochemical methods for differentiation from fibrous histiocytomas with similar morphology. 301 Jul 49