脊索样形态特征肿瘤的临床病理分析

目的探讨中枢神经系统内具有脊索样形态特征肿瘤的临床病理特征。方法对42例脊索瘤、6例脊索样脑膜瘤及2例第三脑室脊索样胶质瘤的临床资料和组织形态学资料进行回顾性分析。结果脊索瘤好发于颅底及骶尾部；镜下肿瘤组织呈分叶状结构，瘤细胞以条索状、簇状或单细胞结构漂浮在淡蓝色粘液样基质中，胞浆内可见空泡，瘤内可见液滴样细胞；瘤细胞细胞角蛋白（CK）、上皮膜抗原（EMA）及S-100表达阳性。脊索样脑膜瘤好发于幕上，与脑膜关系密切；组织学大部分类似于脊索瘤，但可找到典型的脑膜瘤结构；瘤细胞EMA、的和波形蛋白（Vim）表达阳性，CK及胶质纤维酸性蛋白（GFAP）阴性。第三脑室脊索样胶质瘤几乎只发生于第三脑室和下丘脑，组织学特点为条索状或簇状排列的上皮样细胞漂浮于粘液样基质中，伴淋巴细胞和浆细胞浸润及胶质细胞增生；肿瘤细胞GFAP、Vim和CD34表达阳性。结论中枢神经系统“脊索样”改变是脊索瘤、脊索样脑膜瘤及第三脑室脊索样胶质瘤共同的组织学特征，但三者组织形态、免疫表型及发病部位又各有特点。其诊断及鉴别诊断需对临床、组织学形态及免疫表型综合分析。

Analysis of clinicopathology of tumors with chordoid features in the central nervous system

Objective To study the clinicopathological features of tumors with chordoid features in the central nervous system （CNS）. Method The clinical and histopathological data of 50 patients with tumors with chordioid features in CNS including 42 patients with chordomas, 6 with chordoid meningiomas and 2 with chordoid gliomas of the third ventricles, were analyzed retrospectively. Results The chordomas occurred usually in the skull base and sacrococcygeal region. The chordomas were lobulated tumors with tumorous cells arranged in the cord or cluster, or single cell floating in the abundant mueoid matrix. The multivacuolated and ＂physalliphorous＂ cells were observed in the chordomas, which were of immunoreactivity to cytokeratins （CK）, epithelial membranee antigen （EMA）, and S-100 protein. The chordoid meningiomas, which were in the supratentorial region and closely related to the meninges, were similar to the chordomas in the histological structure. However, the typical structure of meningioma was found in the chordoid meningiomas where EMA and vimentin （Vim） expressions were positive, and CK and glial fibrillary acidic protein （GFAP） expressions were negative. The chordoid glioma, which almost occurred only in the third ventricle and hypothalamus, was histologically similar to the chordoma. The epithelioid cells arranged in the cords and clusters were embedded in the mucoid matrix of the chordoid glioma, where there were lymphocytes, and plasmaeyotes infiltrations and glial cells proliferation. The tumorous cells strongly expressed GFAP, Vim and CD34 in the chordoid glioma. Conclusions Chordoid-like change is the common features of ehordoma, chordoid meningioma and chordoid glioma of the third ventricle. However, they have distinct features respectively in the histomorphology, immunophenotype and involved region. Their diagnosis and differential diagnosis depend on the comprehensive analysis of the clinic, histomorphology and immunophenotype.