Chordoid Glioma: A Case Report and Molecular Characterization of Five Cases

• Type of Material: Article
• Authors: Craig Horbinski, University of Pittsburgh; Sanja Dacic, University of Pittsburgh; Roger E McLendon, Duke University; Kathy Cieply, University of Pittsburgh; Michael Datto, Duke University; Daniel Brat, Emory University; Charleen T Chu, University of Pittsburgh
• Language: English
• Date: 2009-07-01
• Publisher: WILEY
• Publication Version: Final Published Version
• Copyright Statement: © 2008 The Authors; Journal Compilation © 2008 International Society of Neuropathology
• License: Creative Commons Attribution 4.0 International
• Final Published Version (URL): http://dx.doi.org/10.1111/j.1750-3639.2008.00196.x
• Title of Journal or Parent Work: BRAIN PATHOLOGY
• Volume: 19
• Issue: 3
• Start Page: 439
• End Page: 448
• Grant/Funding Information: Support for this work came from the Pathology Departments of the University of Pittsburgh and Duke University.
• Keywords: ENTITY; Science & Technology; Pathology; EXPRESSION; FEATURES; DIFFERENTIATION; array comparative genomic hybridization; fluorescence in situ hybridization; Clinical Neurology; TUMORS; CHROMOSOMAL IMBALANCES; chordoid glioma; ORIGIN; 3RD-VENTRICLE; OF-THE-LITERATURE; Life Sciences & Biomedicine; GENE; Neurosciences; Neurosciences & Neurology
• Research Categories: Health Sciences, Pathology

Abstract

Chordoid gliomas are rare, slow-growing neoplasms of the anterior third ventricle. We reported a case of chordoid glioma in a 41-year-old man with obstructive hydrocephalus. Histologically, the tumor consisted of polygonal epithelioid cells admixed with elongated cells in a myxoid stroma. A prominent lymphoplasmacytic infiltrate was present. The tumor cells expressed glial fibrillary acidic protein (GFAP), epithelial membrane antigen (EMA), vimentin, CD31, CD34, epidermal growth factor receptor (EGFR) and S100 but were negative for pankeratin and E-cadherin. The percentage of Ki67 positive cells was approximately 3%. Weak p53 immunoreactivity was seen in less than 10% of the cells. Array comparative genomic hybridization performed on this case, as well as on four other archived cases, showed losses at several loci. Fluorescence in situ hybridization (FISH) confirmed consistent genetic alterations at 9p21 and 11q13. These are the fifth through ninth reported cases of chordoid gliomas with molecular characterization suggesting a distinct genetic origin from other gliomas. © 2008 International Society of Neuropathology.