15例具有血管周上皮样细胞分化的肿瘤临床病理分析

目的探讨具有血管周上皮样细胞分化的肿瘤(perivascular epithelioid cell tumor,PEComa)临床病理特征及免疫表型。方法对15例PEComa行EnVision两步法免疫组化及特殊染色,并分析其临床表现、病理学特征和免疫组化特点。结果 15例PEComa中13例为女性,且术后无复发。肿瘤可发生在身体任何部位,以肝脏和肾脏最多见,有包膜,多为实性,可有出血。特征性组织学改变是肿瘤细胞围绕血管生长。PAS染色显示该肿瘤细胞中有糖原物质沉积,弹力纤维染色显示该肿瘤中的血管壁缺乏弹力层。免疫组化显示所有PEComa病例均明显表达HMB-45、Melan-A、SMA,Ki-67阳性率不高。结论 PEComa是一组具有恶性潜能的肿瘤,多发于女性,来源于血管周上皮样细胞。大部分肿瘤呈良性过程,生物学分级极低,预后良好;极少部分病例可发生转移,预后不佳。免疫组化检测对其组织来源、生物学行为具有提示意义,并可辅助诊断。

The clinical and pathological features of perivascular epithelioid cell tumor of 15 cases

Purpose To investigate the clinicopathologic features and immunological phenotype of perivascular epithelioid cell tumor(PEComa).Methods Immunohistochemistry and special staining were used to observe the 15 cases of PEComa in order to further recognize and understand the tumor.Results Among 15 patients,13 were female,and no postoperative recurrence.Tumors can occur in any part of the body,but mostly in liver and kidney.Most of these tumors were encapsulated,solid,and bleeding.The growth of tumor cells aroun...