外阴孤立性纤维性肿瘤1例临床病理观察及文献复习  

Solitary fibrous tumor of vulva:a clinicopathologic analysis of a case and review of literature

作　　者：陈文静[1] 张江宇[1] 赖日权[2] 张佳立[1] 吴坤河[1] 

机构地区：[1]广东省妇幼保健院/广州医学院附属省妇儿医院病理科,广州510010 [2]广州军区广州总医院病理科,广州510010

出　　处：《临床与实验病理学杂志》2009年第4期396-399,共4页Chinese Journal of Clinical and Experimental Pathology

基　　金：广东省卫生厅资助课题(B2007022)

摘　　要：目的探讨外阴孤立性纤维性肿瘤的临床病理学特点、免疫组化特征及鉴别诊断。方法对1例外阴孤立性纤维性肿瘤进行组织学观察,行EnVision两步法免疫组化CD34、bcl-2、CD99、vimentin、CKpan、HMB-45、α-SMA、CD117、S-100蛋白、ER、PR、Ki-67染色及随访,并复习相关文献。结果患者女,62岁。发现右侧外阴肿物4年余。眼观:肿瘤呈结节状,大小6 cm×5 cm×3 cm,切面灰白、实性,质韧。镜检:肿瘤由交替性分布的富于细胞和稀疏细胞区组成,梭形细胞呈束状、旋涡状或不规则状排列,部分与胶原纤维混杂,部分呈血管外皮瘤样结构。上皮样细胞有的围绕血管周围。细胞间细条索状、粗绳索样或疤痕样胶原纤维不规则性或石棉样的胶原物质沉积。瘤细胞CD34、bcl-2、CD99、vimentin均为阳性,CKpan、HMB-45、α-SMA、CD117、S-100蛋白、ER、PR均为阴性,Ki-67增殖指数<1%。结论外阴孤立性纤维性肿瘤罕见,应与该部位相似形态的病变和血管外皮瘤样的肿瘤相鉴别。Purpose To explore the clinical pathological features, immunohistochemical characteristics and differential diagnosis of solitary fibrous tumor（SFT） of the vulva. Methods A case of SFT of the vulva was examined with light microscopy and immunohistochemistry, including CD34, bcl-2,CI）99,vlmentin,CK （AE1/AE3）, HMB-45,α-SMA, CD117, S-100 protein, ER,PR and Ki-67 with EnVision method,and related literatures were reviewed. Results The patient was a 62-year-old woman. Clinical and radiological examination revealed a tumor in the vulva for 4 years. Grossly, the tumor was nodular with dimension 6 cm × 5 cm × 3 cm,well-circumscribed with grey white color on cut surface. Microscopically, the tumor was composed of bland spindle-shaped cells and dense collagenous bands,with fascicular, storiform, or haphazard arrangements. Focal areas showed a haemangiopericytoma-like structure. Immunohistochemically, the tumor ceils were positive for CD34, bcl-2, CD99 and vimentin, but negative for CK （AE1/AE3）, HMB45, α-SMA, CDllT,S-100 protein, ER and PR. Ki-67 index was less than 1%. Conclusions SFT is a very rare tumor, especially in vulva, and it should be differentiated from the tumors that have features similar to hemangio-perithelioma.

关 键 词：孤立性纤维性肿瘤 外阴肿瘤 梭形细胞型肿瘤 鉴别诊断 免疫组织化学 

分 类 号：R737.35[医药卫生—肿瘤]