子宫血管周上皮样细胞肿瘤临床病理特征

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(perivascular epithelioid cell tumor,PEComa)的临床病理特征、诊断及鉴别诊断。方法对1例子宫PEComa进行组织学观察和免疫组化标记,同时复习相关文献。结果患者,女性,51岁,肿瘤位于子宫浆膜下,直径24 cm。肿瘤由圆形和多角形的上皮样细胞围血管排列,呈巢状和片状,细胞无异型,未见核分裂象,胞浆透明或淡嗜酸性。部分区域肿瘤细胞为梭形,呈束状排列。间质内可见不同程度的透明变性。免疫表型:上皮样细胞Vimentin和HMB45阳性。梭形细胞SMA、Actin(HHF35)和desmin阳性。肿瘤细胞不表达CK(AE1/AE3)、EMA、CD10、CD34、CD117和S-100。术后随访26个月未见转移和复发。结论子宫PEComa是PEComa家族中的一个成员,属于恶性潜能未定的肿瘤。

Aim To report a case of perivascular epithelioid cell tumor（PEComa） of uterus and to study its clinicopathological features,diagnosis and differrential diagnosis.Methods One case of uterine PEComa was studied by HE staining and immunohistochemistry,with review of the relevant literatures.Results The patient was a 51-year-old female.The tumor occurred in the subserosa of the uterus.The tumor was partly composed of round to polyonal epithelial cells with abundant clear to slightly eosinphilic granular cytoplasm in a perivascular location.The tumor cells showed no atypia and mitotic figures,and they were strongly immunoactive for HMB45 and Vimentin.The tumor still had areas of spindle-shaped cells.They were positive for SMA,Actin（HHF35）and desmin.The tumor cells were negative for CK（AE1/AE3）,EMA,CD10,CD34,CD117 and S-100.The patient had no evidence of disease recurrence and metastasis 26 months postoperatively.Conclusions Uterine PEComa is a novel member of the family of PEComa.It should be regarded as tumor with an uncertain malignant potential.