12例中枢神经系统孤立性纤维性肿瘤的临床病理分析

Solitary Fibrous Tumor in Central Nervous System:Clinicopathological Features of 12 Cases

目的探讨中枢神经系统原发的孤立性纤维性肿瘤的临床病理特征及相关鉴别诊断。方法收集华中科技大学同济医学院附属同济医院2005年到2009年间诊断的12例原发及复发的中枢神经系统孤立性纤维性肿瘤临床资料和病理切片,选取典型组织蜡块进行相关标记物的免疫组化染色,检测主要指标包括Vimentin、CD34、Bcl-2、CD99和EMA、S-100等。结果 12例患者中10例为女性,2例为男性;年龄26～72岁,平均年龄46.3岁;12例中2例发生于脑膜,9例发生于邻近脑膜的中枢神经组织,1例发生于椎管内;肿瘤直径1.5～11.0cm,平均直径为5.4cm;肿瘤主要由梭形细胞构成,瘤细胞分布疏密相间,无特殊组织构型。在细胞稀疏的区域,胶原物质沉积增加;在细胞密集区,肿瘤表现为"血管外皮瘤"样的组织学构象;免疫组织化学检测示Vimentin和CD34多呈弥漫阳性,CD99和Bcl-2阳性,而PCK和EMA等上皮标记以及S-100则多为阴性;1例复发病例CD34表达缺失,表现出恶性转化的特点。结论中枢神经系统原发的孤立性纤维性肿瘤较为罕见,有着独特的组织学表现和免疫表型;应和脑膜瘤和神经鞘瘤相鉴别;富于细胞型的孤立性纤维性肿瘤易复发,并有恶变倾向。

Objective To investigate the clinicopathological features and differential diagnosis of solitary fibrous tumor（SFT）in central nervous system（CNS）.Methods Twelve cases of SFT in CNS from Tongji Hospital were analyzed by clinicopathologic study and immunohistochemical staining of Vimentin,CD34,CD99 and Bcl-2.Results There were 10 females and 2 males with age ranging from 26 to 72 years（mean 46.3）.SFT in 2 cases was originated from the meninges,9 from central nervous tissue adjacent to the meninges,and one from intra-vertebral canal.The diameter of tumor was from 1.5 to 11.0 cm（mean 5.4 cm）.SFT was composed of spindle cells arranged in a patternless architecture with alternative of hypocelluar and hypercellular areas.Immunohistochemistry revealed the tumor cells diffusely expressed vimentin,CD34,Bcl-2 and CD99,while PCK,EMA,S-100 always negative.One case had CD34 expression loss and malignant transformation.Conclusion SFT is rare neoplasm in CNS,and should be distinguished from meningioma and Schwannoma by CD34 positive staining.Recurrent case of cellular variant may lose the CD34 expression for malignant transformation.