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

• 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
• Journal Title: BRAIN PATHOLOGY
• Volume: Volume 19, Number 3
• Publisher: WILEY | 2009-07-01, Pages 439-448
• Type of Work: Article | Final Publisher PDF
• Publisher DOI: http://doi.org/10.1111/j.1750-3639.2008.00196.x
• Subjects: Health Sciences, Pathology
• Keywords: Science & Technology; Life Sciences & Biomedicine; Clinical Neurology; Neurosciences; Pathology; Neurosciences & Neurology; array comparative genomic hybridization; chordoid glioma; fluorescence in situ hybridization; OF-THE-LITERATURE; CHROMOSOMAL IMBALANCES; 3RD-VENTRICLE; FEATURES; TUMORS; ENTITY; DIFFERENTIATION; EXPRESSION; ORIGIN; GENE

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.