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

The authors present a case of malignant fibrous histiocytoma (MFH) involving a thoracolumbar myelomeningocele. The patient died of metastases 11 months after diagnosis, in spite of extensive surgery, radiation therapy, and chemotherapy. Meningeal involvement by MFH is very rare; 11 additional cases are reviewed. Glial fibrillary acidic protein staining is critical for proper histological diagnosis, as this tumor can be easily confused with pleomorphic xanthoastrocytoma or glioblastoma involving the meninges. Malignant degeneration within a myelomeningocele is also very rare. Eight additional cases from the literature are reviewed. Our case is the only one with MFH. Chronic irritation to the unrepaired congenital defect appears to be a key to the development of the malignancy. Prophylactic repair in infancy might prevent the occurrence of malignant degeneration.
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PMID:Meningeal malignant fibrous histiocytoma arising from a thoracolumbar myelomeningocele. Case report. 629 83

In order to study the clinicopathological features, histogenesis and prognosis, 12 cases of gliosarcoma were reported representing 0.4% of a series of 2743 patients undergoing biopsy for CNS tumors. All the tumors originated from the cerebral hemispheres with a predilection for the temporal lobes. Half of the cases show more firm consistency and are rather well demarcated from brain tissue. Clinically, they are sometimes mistaken for meningiomas. Of the 10 patients with follow-up, 9 have died. The mean survival period after operation was 8 months, 1 cases is still alive and well for 3.2 years. There were some cases in which the origin of spindle cell populations could not be determined by H & E staining. Glioblastoma and malignant fibrous histiocytoma (MFH) element of the tumor was confirmed by electron microscopical examination and immunohistochemical stains for GFAP, Mac 387, VIM, FV III RA, etc. Osteosarcoma component in the tumor was detected in one case. It was accepted that MFH arose from the primitive uncommited mesenchyme.
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PMID:[A clinical and pathological study of gliosarcoma]. 927 62

The clotting factor XIIIa (FXIIIa) has been shown to be present both in tumor cells and in tumor-associated macrophages of different neoplasms such as Hodgkin's disease, giant cell tumor of bone, malignant fibrous histiocytoma, meningeal tumors, and hemangiopericytoma. The biological significance of these findings, however, are still unclear. This study investigates the immunohistochemical distribution of FXIIIa in 186 tumors of the central nervous system (CNS) in order to evaluate its possible diagnostic or prognostic significance in neuro-oncology. High-grade gliomas such as glioblastoma, gliosarcoma, astrocytoma (grade III WHO), and ependymoma (III) as well as meningiomas and meningeal hemangiopericytomas consistently contained factor XIIIa-positive cells, whereas low-grade glial tumors did not do so. One desmoplastic medulloblastoma and one anaplastic schwannoma also showed FXIIIa-positive cells. With the exception of hemangiopericytomas, however, the major source of FXIIIa expression in all these tumors consisted of a subpopulation of tumor-associated macrophages, the exact role of which still remains unclear. Because of its non-discriminatory staining in a wide variety of CNS tumors, the differential diagnostic contribution of FXIIIa in neuro-oncology seems to be limited.
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PMID:Factor XIIIa-immunoreactivity in tumors of the central nervous system. 956 29

We observed three neoplasms with completely different histologies: malignant fibrous histiocytoma (MFH), atypical meningioma (AM), and glioblastoma (GB), developing in a patient with Li-Fraumeni syndrome. By using a combined molecular approach we performed molecular characterization of all three tumours. Data obtained showed an interesting molecular background of the AM and GB. AM showed TP53mutations and a 22q loss of heterozygosity (LOH). GB showed epidermal growth factor receptor (EGFR) amplification and TP53 mutations, whereas P16, PTEN, Rbwere intact in terms of LOH and/or multiplex PCR (polymerase chain reaction) analysis. Additionally, GB has a 1q LOH, which is an extremely rare alteration in glioblastomas. Identical 1q LOH was also observed in MFH.
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PMID:Atypical molecular background of glioblastoma and meningioma developed in a patient with Li-Fraumeni syndrome. 1571 70

Pleomorphic xanthoastrocytoma (PXA) is an uncommon, usually low-grade, astrocytic tumor. Characteristic histological features include tumor cell pleomorphism and lipidization of tumor cells. Albeit prognosis in PXA is generally good, cases with histological signs of anaplasia have been observed. In these cases, the differential diagnosis needs to exclude other malignancies, for example, glioblastoma or malignant fibrous histiocytoma. Immunocytochemical detection of GFAP may support exclusion of non-glial neoplasms resembling PXA. However, GFAP expression in PXA may be faint or focal, although complete lack of GFAP has not been described. A 43-year-old woman was operated on for a left occipital parasagital tumor attached to the dura. Histopathology showed a pleomorphic tumor with moderate mitotic activity and necrosis, lack of GFAP immunoreactivity and ultrastructural detection of premelanosome-like structures. These features led to the tentative diagnosis of amelanotic melanoma, and the patient was irradiated. Three years later she had local tumor recurrence and underwent another operation. The recurrent tumor showed similar plain histology as the first specimen. In contrast, anti-GFAP immunoreactivity was now detectable in pleomorphic tumor cells. Anti-GFAP staining of the first biopsy was repeated using monoclonal and polyclonal antibodies in combination with prolonged tissue pretreatment. Focal GFAP staining of tumor cells was now achieved. We conclude that non-standard GFAP staining protocols may enhance sensitivity and thus lead to detection of a low level of GFAP expression in tumor specimens, in which PXA is considered in the differential diagnosis. This may avoid misleading diagnostic considerations that impact on postoperative patient management.
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PMID:Pleomorphic xanthoastrocytoma with anaplastic features presenting without GFAP immunoreactivity: implications for differential diagnosis. 1619 42

Gliosarcoma, a variant of isocitrate dehydrogenase-wildtype glioblastoma, is largely a lobar surfacing neoplasm often with dural attachment. In this biphasic neoplasm, the sarcomatous component usually takes the form of fibrosarcoma or malignant fibrous histiocytoma. Heterologous sarcomatous differentiation is a rare phenomenon. Here, we present a case of gliosarcoma with liposarcomatous and myosarcomatous differentiation in a 68-year-old man which was purely intraventricular. This is the first report of such a morphologic pattern in this location. Varied histological components with their immunohistochemical profile are discussed. Of note was the presence of a p53 negative giant cell glioblastoma component, as was the expression in the rest of the tumor.
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PMID:Intraventricular gliosarcoma with dual sarcomatous differentiation: A unique case. 2826 69