肝脏血管周上皮样细胞瘤16例临床分析

Clinical analysis of 16 cases of perivascular epithelioid cell tumor of liver

目的探讨肝脏血管周上皮样细胞瘤(perivascular epithelioid cell tumor,PEComa)的临床特点、诊断与治疗。方法回顾性分析2010年1月—2019年9月经郑州大学第一附属医院收治的16例肝脏PEComa患者的临床资料,总结其临床表现、病理及免疫组化特点、治疗方案及效果。结果16例患者平均年龄49岁,男女比例1∶7。肿瘤平均直径3.5 cm。16例患者均行肝部分切除术,其中5例同时行胆囊切除术,术后病理诊断均为肝脏PEComa,免疫组化HMB-45、Melan-A均阳性,CK8/18、Hepatocyte均阴性。平均随访时间18.8个月,其中1例出现肝内复发,余病例无转移和复发。结论肝脏PEComa发病率低,好发于中年女性,影像学表现具有一定特异性,但确诊率仍较低,误诊及漏诊率较高,确诊依靠病理及免疫组化,手术切除是目前首选的治疗方法,m-TOR通路抑制剂西罗莫司有一定的临床疗效。

Objective To investigate the clinical features,diagnosis and treatment of perivascular epithelioid cell tumor(PEComa)of the liver.Methods The clinical data of 16 patients with PEComa in the First Affiliated Hospital of Zhengzhou University from January 2010 to September 2019 were analyzed retrospectively,summarize its clinical manifestations,pathological and immunohistochemical characteristics,treatment plan and effect.Results The average age of 16 patients was 49 years old(35~64 years old),and the ratio of male to female was 1∶7.The average diameter of tumor was 3.5 cm(0.8 cm^8.3 cm).All the 16 patients underwent partial hepatectomy,5 of them underwent cholecystectomy at the same time.The pathological diagnosis was that the liver was PEComa,the immunohistochemistry HMB-45 and Melan-A were positive,and CK8/18 and hepatecyte were negative.The average follow-up time was 18.8 months,in which 1 case had intrahepatic recurrence and the rest had no metastasis or recurrence.Conclusion The incidence of PEComa in liver is low,which is common in middle-aged women.The imaging performance has certain specificity,but the diagnosis rate is still low,The misdiagnosis and missed diagnosis rate are high.The diagnosis depends on pathology and immunohistochemistry.Surgical resection is the preferred treatment method at present.Sirolimus,an inhibitor of m-tor pathway,has a certain clinical effect.