子宫血管周上皮样细胞肿瘤6例临床病理分析

Clinicopathological features of uterine perivascular epithelioid cell tumor(PEComa)

目的探讨子宫血管周上皮样细胞肿瘤(PEComa)的病理形态、免疫组化特征及鉴别诊断。方法对6例子宫血管周上皮样细胞肿瘤行HE及免疫组化染色,并结合相关文献进行复习。结果 6例肿瘤均由透明或嗜酸性细胞巢或宽窄不等的细胞索组成,间质血管丰富并伴有不同程度透明变性。免疫组化:6例肿瘤细胞vimentin、HMB45、SMA和desmin均不同程度(+),CK、S-100、melanA和CD10(-)。本组1例失访,其余5例均存活。结论子宫血管周上皮样细胞肿瘤是一种罕见的间叶性肿瘤,具有比较特征性的病理形态及免疫组化特点。

Objective To explore the histological,immunohistochemical characteristics and differential diagnosis of uterine perivascular epithelioid cell tumor（ PEComa）. Methods Histology and immuohistochemical markers of six uterine PEComa cases were analyzed and the correlated literature was reviewed. Results All the six tumors were composed of clear or eosinophilic cells arranged in nests and cords,associated with abundant vessels and hyalinization in the stroma. Immunohistochemically,the tumor cells demonstrated positive staining for vimentin,HMB45,SMA and desmin,but did not express CK,S-100,MelanA and CD10. Five patients followed for a certain period were alive,but one lose to follow up. Conclusion Uterine PEComa is a rare mesenchymal tumor of the gynecologic tract,with distinctive histologic and immunohistochemical features.