肝上皮样血管内皮瘤文献复习及临床特点分析  

A review of literatures on clinical manifestations of hepatic epithelioid hemagioendothelioma

作　　者：孙淑杰[1] 迮兴宇[1] 赵新颜[1] 

机构地区：[1]首都医科大学附属北京友谊医院肝病中心,北京100050

出　　处：《临床和实验医学杂志》2012年第9期654-656,共3页Journal of Clinical and Experimental Medicine

基　　金：北京市科技新星计划(2009B45)

摘　　要：目的回顾分析国内曾报道过的肝上皮样血管内皮瘤病例,旨在提高对该病的认识和诊治水平。方法利用中国知识仓库总库检索2000～2011年文献报告的肝上皮样血管内皮瘤病例38例(文献21篇),入选病例均为病理组织学证实。结果男女比例为1∶2.3,平均年龄41.8±16.9岁(8～75岁)。常见的症状及体征包括:右上腹饱胀不适75.0%(15/20),右上腹痛73.7%(14/19),肝大50.0%(3/6),脾大50.0%(3/6),ALT、AST轻度升高者占50.0%(6/12)。单结节型占36.8%(14/38),多结节型占55.3%(21/38),弥漫型7.9%(3/38)。首诊误诊为原发性肝癌10例,肝转移癌10例,肝血管瘤2例。手术肿瘤切除为主要治疗措施,平均生存期1～24个月。结论我国肝上皮样血管内皮瘤的特点基本与国外类似,该病少见,好发于中年女性,肝组织病理免疫组化染色是确诊本病的关键。目前国内主要采取手术切除治疗本病,预后较好。Objective Hepatic epithelioid hemangioendothelioma（HEHE） is a rare disease with vascular origin.The aim of this study is to summarize and analyze the clinical features of HEHE in Chinese patients,thus it may contribute to early diagnosis and properly management for this disease.Methods All data of patients with hepatic epithelioid hemangioendothelioma were collected from China National Knowledge Infrastructure.These literatures were published in Chinese.Results Altogether 21 papers including a total of 38 cases of HEHE had been reported in 2000 to 2011.All these patients confirmed by pathological examination are Chinese.The ratio of male with female was 1：2.3 with the mean age of 41.8±16.9 years（ranging from 8-75 yr）.The common clinical manifestations include right upper quadrant discomfort in 75.0%（15/20）of patients,right upper quadrant pain in 73.7%（14/19）,hepatomegaly in 50.0%（3/6）and splenomegaly in 50%（3/6）,and mildly elevated serum levels of ALT and AST were seen in 50%（6/12）of patients.There were three patterns of HEHE identified including solitary in 36.8%（14/38）,multiple in 55.3%（21/38）and diffuse in 7.9%（3/38）.The initial diagnosis of this disease was usually as hepatocellular carcinoma in 10 patients,metastatic liver carcinoma in 10 patients and hemangioma in 2 patients.These patients were mainly treated by liver resection with mean survival time of 1 to 24 months.Conclusion Hepatic epithelioid hemangioendothelioma in China has similar features as those of previously reported western cases.It mainly affects middle aged women without specific symptoms.Liver resection is widely adopted option for treatment in this country and it has a relatively good clinical outcome.

关 键 词：肝上皮样血管内皮瘤 原发性肝癌 肝血管瘤 转移性肝癌 

分 类 号：R735.7[医药卫生—肿瘤]