Horbinski Craig, Dacic Sanja, McLendon Roger E, Cieply Kathy, Datto Michael, Brat Daniel J, Chu Charleen T
Department of Pathology, University of Pittsburgh School of Medicine, Pittsburgh, PA 15213, USA.
Brain Pathol. 2009 Jul;19(3):439-48. doi: 10.1111/j.1750-3639.2008.00196.x. Epub 2008 Jul 23.
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.
脊索样胶质瘤是第三脑室前部罕见的、生长缓慢的肿瘤。我们报告了一例41岁患有梗阻性脑积水的男性脊索样胶质瘤病例。组织学上，肿瘤由多边形上皮样细胞与黏液样基质中的细长细胞混合组成。存在显著的淋巴浆细胞浸润。肿瘤细胞表达胶质纤维酸性蛋白（GFAP）、上皮膜抗原（EMA）、波形蛋白、CD31、CD34、表皮生长因子受体（EGFR）和S100，但细胞角蛋白和E-钙黏蛋白呈阴性。Ki67阳性细胞百分比约为3%。不到10%的细胞可见弱p53免疫反应性。对该病例以及其他四个存档病例进行的阵列比较基因组杂交显示多个位点存在缺失。荧光原位杂交（FISH）证实9p21和11q13存在一致的基因改变。这些是第五至第九例报告的具有分子特征的脊索样胶质瘤病例，提示其起源于与其他胶质瘤不同的独特基因。
