子宫血管周上皮样细胞肿瘤临床病理分析

Clinicopathological Analysis of Perivascular Epithelioid Cell Tumor

目的探讨子宫血管周上皮样细胞肿瘤（PEComa）的临床病理及免疫组化特征、鉴别诊断及预后。方法对3例子宫PEComa进行临床、光镜及免疫组化的研究并复习相关文献。结果 3例子宫PEComa发病年龄从33至43岁,1例剖宫产偶然发现,2例表现为阴道异常出血,肿瘤大小从1.5cm×1.2cm×0.8cm至4cm×3.5cm×3cm。镜检：肿瘤内广泛分布分支状毛细血管网至透明变性的厚壁大血管,上皮样瘤细胞围绕血管周围呈放射状排列,未见核分裂象及坏死灶,其中1例可见脉管浸润。免疫组化：HMB-45、Melan-A、SMA、Actin及Desmin胞浆阳性。3例均手术切除,随访5-8年不等均无复发或转移。结论子宫PEComa是具有独特的病理学特征的罕见肿瘤,免疫组化黑色素和肌源性标记物双向表达阳性有助于诊断,良性肿瘤手术切除预后好。

OBJECTIVE To investigate the clinieopathological and immunohistochemical features, differential diagnosis and prognosis of Perivascular epithelioid cell tumor （PEComa） of the uterus. METHODS 3 cases of uter- ine PEComa were studied through clinical features,microscopic and immunohistochemical observation and the related literature were reviewed. RESULTS The patients＇ ages ranged from 33 to 43 years, one case was found accidently by cesarean section,two cases presented abnormal vaginal bleeding. The tumors size ranged from 1.5cm x1. 2cm x 0. 8cm to 4cm x 3.5cm x 3cm. Microscopically,Tumors were widely distributed with the branching capillaries net to blood vessels of hyaline degeneration, the epithelioid cells surrounded vessels and radiated out from vessels with no mitotic and necrosis,and 1 case presented with lymphovascular invasion. Immunohistochemically, HMB-45, Melan-A, SMA,Aetin and Desmin were positive in the cytoplasm. 3 cases of tumors were removed surgically, the followed up period varied from 5 to 8 years and all were no recurrence or metastasis. CONCLUSION Uterine PEComa is a rare tumor with unique pathological feature, coexpression of melanocytic and muscle markers could help in diagnosis of u- terine PEComa, the prognosis of benign tumors after surgery were well.