Cho Hyun-Yee, Lee Mija, Takei Hidehiro, Dancer Jane, Ro Jae Y, Zhai Qihui J
Department of Pathology, The Methodist Hospital, Weill Medical College of Cornell University, Houston, TX 77030, USA.
Appl Immunohistochem Mol Morphol. 2009 Mar;17(2):131-8. doi: 10.1097/PAI.0b013e3181866a13.
Chordoma originates from embryonic notochordal remnants in the midline along the spinal axis and is characterized by cords and lobules of neoplastic cells arranged within myxoid matrix. Because of histologic similarities with myxoid matrix and overlapping immunohistochemical profile, chondrosarcoma, myxopapillary ependymoma, and chordoid meningioma enter in the histologic differential diagnosis at this site. Therefore, the judicious use of a panel of selected immunostains is unquestionably helpful in diagnostically challenging cases. To find useful immunohistochemical markers for assisting in differential diagnosis between chordoma and other tumors with chordoid morphology, an immunohistochemical study using D2-40, epithelial membrane antigen (EMA), pan-cytokeratin (panCK), glial fibrillary acidic protein (GFAP), S-100 protein, galectin-3, neural cell adhesion molecule (NCAM), beta-catenin, E-cadherin, and carcinoembryonic antigen was performed on 14 chordomas, 7 chondrosarcomas, 9 myxopapillary ependymomas, and 4 chordoid meningiomas. Chordoma typically showed positive for EMA and panCK and negative for D2-40 and GFAP; whereas chondrosarcoma revealed positive for D2-40, and negative for EMA, panCK, and GFAP; myxopapillary ependymoma positive for GFAP and negative for EMA; and chordoid meningioma positive for EMA, and negative for panCK and GFAP. On the basis of our immunohistochemical study, a panel of D2-40, EMA, panCK, and GFAP allowed the correct recognition of all tumors examined. Other immunohistochemical markers including S-100 protein, galectin-3, NCAM, beta-catenin, E-cadherin, and carcinoembryonic antigen were of little value in differential diagnosis. In summary, the best immunohistochemical markers useful for the evaluation of tumors with chordoid morphology were D2-40, EMA, cytokeratin, and GFAP. D2-40 was a true chondroid marker to be useful for the differential diagnosis with chordoma.
脊索瘤起源于沿脊柱轴中线的胚胎脊索残余组织，其特征是肿瘤细胞呈条索状和小叶状排列于黏液样基质中。由于与黏液样基质在组织学上存在相似性且免疫组化谱有重叠，软骨肉瘤、黏液乳头型室管膜瘤和脊索样脑膜瘤均纳入该部位的组织学鉴别诊断。因此，明智地使用一组选定的免疫组化染色对于诊断具有挑战性的病例无疑是有帮助的。为了找到有助于脊索瘤与其他具有脊索样形态肿瘤鉴别诊断的有用免疫组化标志物，对14例脊索瘤、7例软骨肉瘤、9例黏液乳头型室管膜瘤和4例脊索样脑膜瘤进行了使用D2-40、上皮膜抗原（EMA）、全细胞角蛋白（panCK）、胶质纤维酸性蛋白（GFAP）、S-100蛋白、半乳糖凝集素-3、神经细胞黏附分子（NCAM）、β-连环蛋白、E-钙黏蛋白和癌胚抗原的免疫组化研究。脊索瘤通常EMA和panCK呈阳性，D2-40和GFAP呈阴性；而软骨肉瘤D2-40呈阳性，EMA、panCK和GFAP呈阴性；黏液乳头型室管膜瘤GFAP呈阳性，EMA呈阴性；脊索样脑膜瘤EMA呈阳性，panCK和GFAP呈阴性。基于我们的免疫组化研究，一组D2-40、EMA、panCK和GFAP能够正确识别所有检查的肿瘤。其他免疫组化标志物包括S-100蛋白、半乳糖凝集素-3、NCAM、β-连环蛋白、E-钙黏蛋白和癌胚抗原在鉴别诊断中价值不大。总之，用于评估具有脊索样形态肿瘤的最佳免疫组化标志物是D2-40、EMA、细胞角蛋白和GFAP。D2-40是一种真正的软骨样标志物，有助于与脊索瘤进行鉴别诊断。
