肝血管平滑肌脂肪瘤的临床特征与预后分析  

Clinical characteristics and prognostic analysis of hepatic angiomyolipoma

作　　者：陈伦 何柳青 吴静[3] 何超[1] CHEN Lun;HE Liuqing;WU Jing;HE Chao(Department of General Surgery,the Second Xiangya Hospital,Central South University,Changsha 410011,China;Department of Pathology,the Second Xiangya Hospital,Central South University,Changsha 410011,China;Department of Radiology,the Second Xiangya Hospital,Central South University,Changsha 410011,China)

机构地区：[1]中南大学湘雅二医院普通外科,湖南长沙410011 [2]中南大学湘雅二医院病理科,湖南长沙410011 [3]中南大学湘雅二医院放射科,湖南长沙410011

出　　处：《中国普通外科杂志》2024年第7期1078-1090,共13页China Journal of General Surgery

基　　金：湖南省自然科学基金资助项目(2022JJ30809)。

摘　　要：背景与目的:肝血管平滑肌脂肪瘤(HAML)是一种罕见的肝脏良性肿瘤,部分HAML较难与其他肝脏良、恶性肿瘤相鉴别。其临床诊断困难,治疗策略及预后仍不清楚。本研究探讨HAML的临床、影像与病理特征,以及治疗策略和预后情况,以期为该病的临床诊治提供参考。方法:回顾性分析中南大学湘雅二医院普通外科2012年1月—2023年12月收治的46例HAML患者的临床特征、病理、影像及随访资料。结果:46例HAML患者中,男性12例(26.1%),女性34例(73.9%);发病年龄20~70岁(中位年龄46岁)。29例发病时无明显临床症状,17例表现为腹部不适,6例合并乙型病毒性肝炎,7例合并肾血管平滑肌脂肪瘤(RAML)。无病例合并结节性硬化症。肿瘤标记物(AFP、CEA、CA19-9、异常凝血酶原Ⅱ)均无明显异常。根据其中4例HAML患者的临床数据,HAML平均增长速度0.46 cm/年,中位增长速度0.39 cm/年。影像学表现多样,超声、CT和MRI对HAML术前影像学诊断的准确率分别为3.8%、34.1%、40.9%。46例样本中,共有左肝病变26例(56.5%),右肝病变18例(39.1%),尾状叶病变2例(4.3%)。1例为多发且局限于左肝,余45例均为单发。肿瘤直径为1~15 cm(中位直径4.3 cm)。41例病理结果报告为良性,5例病理结果报告为恶性;上皮样型HAML有10例。免疫组化显示,HMB-45、Melan-A阳性率均为100%,SMA阳性率为95.1%,CD34阳性率为89.7%,S-100阳性率为54.5%。随访0.25~12年,失访16例。1例术后19个月复发,2年后死于肺腺癌(本中心术后病理证实),另1例术后5年死于肺癌(外院病理证实);2例死亡均与HAML无关,余28例患者均健康,无复发转移。结论:HAML好发于中年女性,常无明显症状,肿瘤生长缓慢,通过影像学诊断容易误诊。病理诊断为HAML诊断的金标准,HMB-45、Melan-A阳性是HAML特异性指标。绝大多数HAML病理为良性,少数为恶性。患者整体预后良好,上皮样型HAML和恶性HAML有复发风险,建议积极�Background and Aims:Hepatic angiomyolipoma(HAML)is a rare benign liver tumor that can be difficult to distinguish from other benign and malignant liver tumors.The clinical diagnosis is challenging,and the treatment strategies and prognosis remain unclear.This study was performed to explore the clinical,imaging,and pathological characteristics,as well as the treatment strategies and prognosis of HAML,to provide a reference for its clinical diagnosis and treatment.Methods:The clinical,pathological,imaging,and follow-up data of 46 cases of HAML treated in the Department of Hepatobiliary and Pancreatic Surgery of the Second Xiangya Hospital of Central South University from January 2012 to December 2023 were retrospectively analyzed.Results:Among the 46 HAML patients,12 were male(26.1%)and 34 were female(73.9%),with an age range of 20 to 70 years(median age of 46 years).Twenty-nine cases were asymptomatic at onset,17 presented with abdominal discomfort,6 had concurrent hepatitis B,and 7 had concurrent renal angiomyolipoma(RAML).No cases were associated with tuberous sclerosis.Tumor markers(AFP,CEA,CA19-9,and abnormal prothrombinⅡ)were not significantly abnormal.Based on clinical data from 4 HAML patients,the average growth rate of HAML was 0.46 cm/year,with a median growth rate of 0.39 cm/year.Imaging characteristics were diverse,with the accuracy rates of preoperative imaging diagnosis of HAML being 3.8%for ultrasound,34.1%for CT,and 40.9%for MRI.Among the 46 samples,26 cases(56.5%)had left liver lesions,18 cases(39.1%)had right liver lesions,and 2 cases(4.3%)had caudate lobe lesions.One case had multiple lesions confined to the left liver,and the remaining 45 cases had single lesions.Tumor diameters ranged from 1 to 15 cm,with a median diameter of 4.3 cm.Pathological results indicated that 41 cases were benign,while 5 cases were malignant;10 cases were epithelioid HAML.Immunohistochemistry showed 100%positivity for HMB-45 and Melan-A,95.1%for SMA,89.7%for CD34,and 54.5%for S-100.Follow-up ranged from 0.25 to 12 ye

关 键 词：肝肿瘤 血管肌脂瘤 预后 

分 类 号：R735.7[医药卫生—肿瘤]