肝血管周上皮样细胞瘤24例诊治分析  

Analysis of the diagnosis and treatment of 24 cases of hepatic perivascular epithelioid cell tumor

作　　者：刘奔 周文逸 肖玉婷 成昱昊 葛一亨 聂盛丹[2] 吕品[1] Liu Ben;Zhou Wenyi;Xiao Yuting;Cheng Yuhao;Ge Yiheng;Nie Shengdan;Lyu Pin(Department of Hepatobiliary Surgery,The First Affiliated Hospital of Hunan Normal Hospital of Hunan Normal University/Hunan Provincial People's Hospital,changsha 410005,China;Clinical trial research Center,The First Affiliated Hospital of Hunan Normal Hospital of Hunan Normal University/Hunan Provincial People's Hospital,changsha 410005,China)

机构地区：[1]湖南师范大学附属第一医院/湖南省人民医院肝胆外科,长沙410005 [2]湖南师范大学附属第一医院/湖南省人民医院临床试验研究中心,长沙410005

出　　处：《中华肝脏病杂志》2022年第8期889-894,共6页Chinese Journal of Hepatology

基　　金：国家自然科学基金(82070857)。

摘　　要：目的探讨肝血管周上皮样细胞瘤(PEComa)的诊断与治疗方案。方法对2015年9月至2020年9月期间确诊的24例PEComa患者的临床表现、血清甲胎蛋白(AFP)、癌胚抗原(CEA)、糖类抗原19-9(CA19-9)指标、影像学检查结果、手术方式、手术后住院时间、病理结果及预后进行回顾性分析。结果女性发病较多(79.2%),年龄(41.5±11.4)岁,肿瘤多位于右肝(50.0%),其中多数患者(76.7%)无明显临床症状;AFP、CEA、CA199指标均为阴性。CT多提示瘤体于平扫期呈现低密度,增强期强化,门静脉期与平衡期呈现强化减弱(66.7%);MRI多表现为肿瘤呈低T1WI信号与高T2WI信号(72.7%);B超多提示瘤区高回声团,其内点状血管影(52.9%);行腹腔镜手术者13例,术后住院时间(9.0±2.4)d,行传统开腹手术(下称开腹手术)者10例,术后住院时间(13.4±6.3)d,行微波消融术患者1例,术后住院时间3 d;术后病理结果均有HMB45阳性、Melan-A阳性。随访结果:21例患者正常生存,近期体检结果未提示肿瘤复发,2例患者分别于术后2年与3年出现肿瘤复发并死亡,1例失访。结论肝PEComa好发于中年女性,肿瘤标志物与临床表现无特异性表现,部分影像学检查结果呈现特异性,可联合其特点作为诊断依据,术后病理学检查结果可明确诊断,手术仍为该病首选治疗方案,因微波消融术及腹腔镜手术较开腹手术术后时间短,创伤小,推荐作为优先选择。Objective To investigate hepatic perivascular epithelioid cell tumor(PEComa)diagnosis and treatment plan.Methods 24 cases diagnosed with PEComa clinical manifestations,serum alpha fetoprotein(AFP),carcinoembryonic antigen(CEA),carbohydrate antigen 199(CA199),imaging findings,surgical methods,postoperative hospital stay,pathological results and prognosis were analyzed retrospectively from September 2015 to September 2020.Results Majority of patients were females(79.2%),aged 41.5±11.4 years.Tumors were predominantly located in the right liver(50.0%).76.7%of the cases were mostly clinically asymptomatic.AFP,CEA and CA199 indices were all negative.CT mostly showed low density tumor in the plain scan phase,enhanced in the enhancement phase,and enhanced and weakened in portal venous and equilibrium phase(66.7%).MRI manifestations of most tumors were hypointense on T1WI and hyperintense on T2WI(72.7%).B-ultrasound mostly showed hyperechoic mass in the tumor area with punctate vascular shadow(52.9%).Postoperative hospital stay was 9.0±2.4 days for laparoscopic surgery patients(n=13),13.4±6.3 days for traditional laparotomy(hereinafter referred to as laparotomy,n=10),and 3 days for 1 patient with microwave ablation.All postoperative pathological results were positive for HMB45 and Melan-A.Follow-up results:21 cases survived normally,with no tumor recurrence in the recent physical examination;two cases had tumor recurrence and they died two and three years after surgery,and one case was lost to follow-up.Conclusion Hepatic PEComa more commonly occurs in middle-aged women,with no specific features for tumor markers and clinical manifestations.Some imaging findings are specific,so its features can be combined as a basis for diagnosis.Postoperative pathological examination results can confirm the diagnosis.Therefore,surgery remains the initial treatment plan.Microwave ablation and laparoscopic surgery are recommended as the preferred option because of shorter hospital stays and less trauma than open surgery.

关 键 词：血管周上皮样细胞肿瘤 PEComa诊断 PEComa治疗 

分 类 号：R735.7[医药卫生—肿瘤]