409例肝上皮样血管平滑肌脂肪瘤汇总分析

409 patients with hepatic epithelioid angiomyolipoma： A pooled analysis

目的总结肝上皮样血管平滑肌脂肪瘤（HEAML）的诊疗经验，提高对该病的认识，降低误诊率。方法收集2000年1月至2018年3月PubMed、Medline、万方、维普数据库中所有关于HEAML的报道并进行汇总分析。结果共收集97篇文献、409例患者，男女比例为1：4．84，年龄12～80岁，中位年龄44．0岁。其中61．9％（205／331）的患者为无症状体检发现，34．7％（115／331）表现为上腹部或右上腹胀痛不适，少数表现为腹部包块、胃肠道反应、低热、体重减轻等。其余78例患者临床症状文献未提及。HEAML误诊率高达40．3％（165／409）。HEAML影像学表现无特异性，多为单发。超声造影、CT、MRI动脉期多表现为强化征象，多可见“中心血管影”及“早期引流静脉”。增强扫描表现多样，“快进快出”、“快进慢出”、“延迟强化”的发生比率大致为4：5：1。HEAML确诊有赖于病理发现病灶内的上皮样细胞以及免疫组织化学染色HMB45、SMA、Melan—A、Actin等表达。HEAML恶性率为3．9％（16／409），手术切除是最主要的治疗方式。结论HEAML属罕见病例，且极易误诊，结合临床资料、影像学、病理及免疫组化检查有助于明确诊断。

Objective To summarize our experience in the diagnosis and treatment of hepatic epithelioid angiomyolipoma （HEAML）, with the aim to reduce the future misdiagnosis rate. Methods The PubMed, Medline, China Science Periodical Database （CSPD）, and VIP Databases were searched from January 2000 to March 2018 on all reports on HEAML. Results There were 409 cases of HEAML in 97 reports. The ratio of men to women was 1：4.84. The age ranged from 12 to 80 years and the median age was 44 years. 61.9% of patients （205/331） were asymptomatic, while 34.7% （115/331） had upper or fight upper quadrant abdominal discomfort. Some patients presented with abdominal mass, gastrointestinal reaction, low grade fever or weight loss. The clinical symptoms in 78 patients were not mentioned in the reports. The misdiagnostic rate of HEAML was as high as 40.3% （165/409）. The imaging findings of HEAML were non- specific. Ultrasound, CT and MRI scan usually showed contrast enhancement in the arterial phase. Most lesions were accompanied by central vessels with early drainage veins. The enhanced scans showed varied characteristics. The ratios of fast wash- in and fast wash- out, to fast wash- in and slow wash-out, and to delayed enhancement were roughly 4： 5： 1. A definitive diagnosis of HEAML is based on the pathological findings of epithelioid cells in the lesions and the expressions of HMB45, SMA, Melan-A and Actin on immunohistochemical staining. HEAML had a relatively low malignant rate of 3.9%. Surgical resection was the main treatment for HEAML. Conclusion HEAML was a rare and easily misdiagnosed disease. ,which could be diagnosed by taking into account the clinical course, imaging, pathological and immunohistochemical findings.HEAML