肝脏血管周上皮样细胞瘤11例临床诊治分析  

Clinical analysis of hepatic perivascular epithelioid cell neoplasm： a report of eleven cases

作　　者：王国禹 赵闯[1] 侯登峰[1] 杨明威 胡晓儒 刘志昊 卜献民[1] Wang Guoyu;Zhao Chuang;Hou Dengfeng;Yang Mingwei;Hu Xiaoru;Liu Zhihao;Bu Xianmin(Department of Hepatobiliary and Splenic Surgery,Shengjing Hospital Affiliated to China Medical University,Shenyang 110000,China)

机构地区：[1]中国医科大学附属盛京医院肝胆脾外科,沈阳110000 [2]中国医科大学附属盛京医院病理科,沈阳110000

出　　处：《中华医学杂志》2018年第34期2715-2717,共3页National Medical Journal of China

摘　　要：目的 探讨肝血管周上皮样细胞瘤（PEComa）的临床特征、诊断与治疗方法.方法 回顾性分析2012年4月至2017年10月中国医科大学附属盛京医院收治的11例肝脏PEComa的临床资料,对其临床特点、影像学特征、诊断、治疗、病理学特性及预后进行总结.结果 11例患者年龄35～55岁（平均年龄47岁）,3例为男性,8例为女性.2例患者就诊主诉为上腹部疼痛,其余患者无自觉症状.1例患者合并丙型肝炎病毒感染.11例患者术前影像学诊断率仅为9.09％（1/11）.患者均接受手术治疗,确诊依赖于术后病理及免疫组织化学结果.免疫组化一抗因病例而异,其中Melan A阳性率达100％（10/10）、黑色素瘤抗体（HMB）45阳性率达90％（9/10）、平滑肌肌动蛋白（SMA）阳性率达77.8％（7/9）,S-100阳性率达33.3％（3/9）,Ki-67阳性指数为1％ ～10％.1例患者因术后出血死亡,其余10例患者（随访5～71个月）均未肿瘤转移及复发.结论 肝脏PEComa是一种罕见的能够表达黑色素细胞和肌细胞标志物的间叶性肿瘤,常见于中年女性,无特异性临床表现,术前难以诊断,确认依赖于病理及免疫组化结果.手术及长期密切随访是目前公认的治疗方法.Objective To investigate the clinical features , diagnosis and therapy of hepatic perivascular epithelioid neoplasms （PEComa）.Methods The clinical data of eleven patients with hepatic PEComa who received surgical treatment at Shengjing Hospital Affiliated to China Medical University from April 2012 to October 2017 were collected.The clinical manifestations, imaging features, diagnostic and therapeutic strategies, pathologic features, prognosis were analyzed.Results The patients aged from 35 to 55 years （mean： 47 years ,3 males and 8 females）.Two patients had epigastric pain, the others rarely had any clinical symptom.Hepatitis C virus （HCV） infection was present in one patient 9.09%（1/11）,the rate of correct diagnosis by imageological examination before operation was only 9.09%（1/11） .All patients received a surgical resection, the final diagnosis of hepatic PEComa was made with pathology and immunohistochemistry.The antibodies used for immunohistochemistry varied from patient to patient .The positive rates of Melan A,HMB45, smooth muscle actin and S-100 were 100%（10/10）,90%（9/10）, 77.8%（7 /9）and 33.3%（3 /9） respectively, the Ki-67 positive index was 1% -10%.One patient died after surgery because of hemorrhage ,other ten patients received long -term follow-up（5 -71 months）,and no recurrence or metastasis was observed.Conclusion Hepatic PEComa is a rare mesenchymal neoplasm which expresses both melanocytic and myogenic markers .Middle aged females are susceptive to hepatic PEComa , and patients rarely have any specific clinical presentation .It′s difficult to make a correct diagnosis before operation.The diagnosis finally depends on the pathological examination .Surgical resection and close follow -up are the principal methods for the management of hepatic PEComa .

关 键 词：肝脏 血管周上皮样细胞瘤 诊断 治疗 

分 类 号：R735.7[医药卫生—肿瘤]