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Query: UMLS:C0017638 (glioma)
 30,880 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Chordoid glioma of the third ventricle was recently reported as a novel tumor entity of the central nervous system with characteristic clinical and histopathological features (Brat et al., J Neuropathol Exp Neurol 57: 283-290, 1998). Here, we report on a histopathological, immunohistochemical and molecular genetic analysis of five cases of this rare neoplasm. All tumors were immunohistochemically investigated for the expression of various differentiation antigens, the proliferation marker Ki-67, and a panel of selected proto-oncogene and tumor suppressor gene products. These studies revealed a strong expression of GFAP, vimentin, and CD34. In addition, most tumors contained small fractions of neoplastic cells immunoreactive for epithelial membrane antigen, S-100 protein, or cytokeratins. The percentage of Ki-67 positive cells was generally low (<5%). All tumors showed immunoreactivity for the epidermal growth factor receptor and schwannomin/merlin. There was no nuclear accumulation of the p53, p21 (Waf-1) and Mdm2 proteins. To examine genomic alterations associated with the development of chordoid gliomas, we screened 4 tumors by comparative genomic hybridization (CGH) analysis. No chromosomal imbalances were detected. More focussed molecular genetic analyses revealed neither aberrations of the TP53 and CDKN2A tumor suppressor genes nor amplification of the EGFR, CDK4, and MDM2 proto-oncogenes. Our data strongly support the hypothesis that chordoid glioma of the third ventricle constitutes a novel tumor entity characterized by distinct morphological and immunohistochemical features, as well as a lack of chromosomal and genetic alterations commonly found in other types of gliomas or in meningiomas.
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PMID:Chordoid glioma of the third ventricle: immunohistochemical and molecular genetic characterization of a novel tumor entity. 1051

We present a case of chordoid glioma involving the third ventricle in a 42-year-old woman. CT and MR showed a homogeneously enhancing mass occupying the third ventricle, with a cystic component. Chordoid glioma should be included in the differential diagnosis of uncommon masses of the third ventricle in middle-aged women.
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PMID:Chordoid glioma of the third ventricle: CT and MR findings. 1075 4

Chordoid glioma of the third ventricle is a recently characterized primary neoplasm of the central nervous system. We present a case and discuss the pathologic and radiologic features. We are aware of only 16 other cases documented in the world literature. This radiologic-pathologic correlation alerts pathologists and radiologists to recognize chordoid glioma as a distinct clinicopathologic entity restricted to the third ventricular area of adult patients.
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PMID:Chordoid glioma: a novel tumor of the third ventricle. 1114 69

Chordoid glioma is a rare neoplasm occurring in the third ventricle and, as the name implies, having a chordoid appearance. It is currently considered a glial neoplasm of uncertain histogenesis with distinct clinicopathologic features. We report three cases of chordoid glioma with a focus on the ultrastructural appearance. The patients were two men and one woman aged, respectively, 34, 40, and 43 years. Immunohistochemically, all tumors showed strong and diffuse reactivity for glial fibrillary acidic protein and vimentin, whereas immunoreactivity for epithelial membrane antigen and cytokeratin was focal. Ultrastructurally, they showed features of ependymal differentiation for the presence of an apical pole with microvilli and a basal pole characterized, as in normal ependyma, by many hemidesmosomelike structures connecting cell membranes to the underlying basal lamina. Constant features were a submicroscopic cell body zonation (i.e., perinuclear, intermediate, subapical, and apical regions) and the presence of secretory granules. These findings were similar to those described for the secretory ependymal cells of the subcommissural organ, a small structure located in a dorsocaudal region of the third ventricle that undergoes regression after birth in humans. Our observations suggest that chordoid glioma may represent a subtype of ependymoma whose cells resemble the highly specialized ependyma of the subcommissural organ.
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PMID:Chordoid glioma of the third ventricle: an ultrastructural study of three cases with a histogenetic hypothesis. 1122 12

Chordoid glioma, a rare tumour of the third ventricle, has distinctive histological appearances. Fewer than 20 cases have been reported in the literature, all but three in females. This paper describes a 54-year-old man with a chordoid glioma and reviews the clinicopathological features of this lesion.
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PMID:Chordoid glioma: an uncommon tumour of the third ventricle. 1136 Mar 80

Chordoid glioma is a rare benign neoplasm of uncertain histogenesis occurring in the third ventricle/ /suprasellar region. Recently, data have emerged suggesting that chordoid glioma is a variant of ependymoma related to a specialised ependyma of the subcommisural organ or the lamina terminalis area. In this study, we report clinicopathological and ultrastructural findings in two chordoid glioma cases. In case 1, a tumour (1.5 cm in diameter) in a 62-year-old man invaded the anterior-basal part of the third ventricle in the lamina terminalis region. In case 2, a large tumour in a 51-year-old woman occupied the whole third ventricle. The tumour attached to the medio-basal hypothalamic region. Histologically, both cases revealed a distinct chordoma-like pattern and glial immunophenotype of tumour cells. Under the electron microscope the tumour cells exhibited microvilli, intercellular lumina, intermediate type junctions and focal basal lamina formations. These findings were similar to those previously reported in the chordoid glioma cases. Moreover, the intracytoplasmic cilia and subplasmalemmal pinocytic vesicles or caveoles were observed. The study supports the view of ependymal derivation of chordoid glioma. Its relation to lamina terminalis or infundibular/median eminence area presumably reflecting tumour origin from the modified ependyma of circumventricular organs of the third ventricle is discussed.
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PMID:Clinicopathological and ultrastructural study in two cases of chordoid glioma. 1460

Chordoid glioma has been recently described as a slow-growing neoplasm with chordoid appearance, occurring exclusively in the regions of the third ventricle and hypothalamus of middle-aged women. We experienced a case of a 48-year-old woman with a suprasellar tumor composed of chordoid glioma and Rathke's cleft cyst, which was confirmed by histopathological, immunohistochemical and electron microscopic examinations. Histologically, chordoid glioma comprised the major part of the tumor, and the prominent Rathke's cleft cysts were distributed focally in the same tumor tissue without any transitions. Chordoid glioma was immunoreactive for glial fibrillary acidic protein, S-100 protein and vimentin, and focally positive for epithelial membrane antigen and CD34, while cytokeratin highlighted epithelial cells lining Rathke's cleft cysts. Ultrastructural examination of the chordoid glioma revealed short cytoplasmic processes, intermediate filaments, intercellular junctions of zonular adherens type, basal lamina, secretory granules and pinocytic vesicles. The ultrastructural observations of the current case are similar to those of the subcommisural organ, although cell body zonation or microvilli were not evident. The coexistence of chordoid glioma and Rathke's cleft cyst has not been reported previously and may represent a collision tumor.
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PMID:Suprasellar chordoid glioma combined with Rathke's cleft cyst. 1462 3

Chordoid glioma is an uncommon low-grade brain neoplasm arising in the third ventricular region, predominantly in middle-aged women. It characteristically shows chordoma-like histologic features and glial fibrillary acidic protein immunoreactivity. We present a case of chordoid glioma in a previously healthy 56-year-old woman admitted to our hospital because of a cranial trauma subsequent to an incidental fall. Radiologic examinations revealed a well-demarcated, partially cystic, enhancing mass at the level of the lamina terminalis. The lesion was surgically removed. The patient remained alive and well 8 months after the surgery. Histologically, the tumor consisted of clusters and cords of epithelioid cells embedded in a mucinous matrix. Lymphoplasmacytic infiltrates and Russell bodies were prominent. Immunohistochemically, the tumor cells were positive for glial fibrillary acidic protein, neurofilaments, and neuron-specific enolase, suggesting a divergent neuronal and glial differentiation. The Ki-67 index was low. The clinicopathologic profile and the differential diagnosis of this tumor are discussed.
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PMID:Chordoid glioma: clinicopathologic profile and differential diagnosis of an uncommon tumor. 1550 76

Chordoid glioma of the third ventricle is an infrequent brain tumour that was described for the first time by Brat et al. in 1998; since then, only 39 cases have been reported. We present a new case of chordoid glioma of the third ventricle in a 51-year-old-man that was treated with total surgical removal, with a good initial postoperative evolution. Sudden death, most likely due to a massive pulmonary embolism, occurred in the third postoperative day. We present the histological characteristics of the tumour and review the literature regarding this entity.
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PMID:[Chordoid glioma of the III ventricle. Case report and revision of the literature]. 1749 57

Chordoid glioma, which generally occurs in adults, is a rare CNS tumor arising in the anterior part of the third ventricle. We report two cases of chordoid glioma of the third ventricle in a 42-year-old woman and a 51-year-old man, respectively. Both tumors showed essentially the same histological and immunohistochemical features; the tumors were composed of cords and nests of epithelioid, GFAP-immunoreactive cells in a mucinous stroma with lymphoplasmacytic infiltrates at the tumor periphery. Ultrastructural examination in one case revealed that the tumor cells were characterized by the presence of hemidesmosomes and associated focal basal lamina formation, intermediate junctions, microvilli and cilia, and intercellular microrosettes with microvilli. Of interest was that small blood vessels with fenestrated endothelial cells were present in the stroma. In the brain, the presence of fenestrated endothelial cells is a feature of the circumventricular organs (except the subcommissural organ), among which the organum vasculosum of the lamina terminalis is located in the anterior part of the third ventricular floor that is lined by specialized ependymal cells known as tanycytes. These findings further strengthen the hypothesis that chordoid glioma may represent a peculiar clinicopathological subtype of ependymoma (chordoid ependymoma) originating from the lamina terminalis area.
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PMID:Chordoid glioma of the third ventricle: a report of two cases, one with ultrastructural findings. 1849 85


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