肝上皮样血管内皮瘤的诊断与治疗

摘要

Objective To study the clinical features and treatment results of hepatic epithelioid hemangioendothelioma (HEHE).Methods A retrospective study was conducted on 26 patients with HEHE who were diagnosed and treated at the Eastern Hepatobiliary Surgical Hospital in Shanghai from November 2003 to December 2015.Results The main clinical manifestations of these 26 patients included abdominal pain (13),dizziness (1),anorexia (1),the other 11 patients were asymptomatic.Ultrasound test revealed mixed echogenicity in 17 patients (17/25,68.0％).CT study showed hypointensity lesions in 10 patients (10/12,83.3％).MRI scans identified T1WI hypointense signals in 12 patients (12/14,85.7％) and T2WI hyperintense signals in 11 patients (11/14,78.6％).Platelet distribution width (PDW) assay indicated 18 patients ＜ 15.5 fl (18/22,81.8％),P ＜0.05.all the patients performed tumor makers test,including AFP,CEA,CA19-9.Slightly elevation of CA19-9 was merely identified in one patient.All the patients were confirmed CD34 positive through immunohistochemistry stains.The 1,3,5-year survival rates of these patients were 87.7％,57.0％,43.4％,respectively.Median survival was 61 months.Conclusions HEHE should be considered based on the suggestive imaging findings for the patients with negative tumor markers after exclusion of hemangioma.PDW assay may have a key role in HEHE diagnosis.%目的 分析肝上皮样血管内皮瘤(HEHE)的临床特征及诊治经验,为其临床诊断提供参考.方法 回顾性分析2003年11月到2015年12月上海东方肝胆外科医院确诊的26例HEHE患者的临床资料.结果 26例患者的主要临床表现为上腹痛(13例)、头晕(1例)、纳差(1例),11例无症状.B超检查为混合回声者17例(17/25,68.0％).CT检查为低密度者10例(10/12,83.3％).MRI检查T1WI为低信号者12例(12/14,85.7％),T2WI为高信号者11例(11/14,78.6％).行血小板分布宽度(PDW)检测＜15.5 fl者18例(18/22,81.8％),P＜0.05.所有患者均行AFP、CEA、CA19-9肿瘤标记物检测.仅1例CA19-9轻度升高.免疫组化检查26例CD34均阳性.26例患者术后1、3、5年生存率分别为87.7％、57.0％和43.4％,中位生存时间61个月.结论 肿瘤标记物阴性,结合影像学特点,在排除血管瘤后,可以考虑诊断HEHE.PDW检查对HEHE临床诊断可能具有一定意义.