肝脏血管周上皮样细胞瘤3例报道及文献复习

Clinical management of hepatic perivascular epithelioid cell tumor:a report of 3 cases with a literature review

目的探讨肝脏血管周上皮样细胞瘤(perivascular epithelioid cell tumor,PEComa)的临床特点、诊断与外科治疗。方法回顾性分析2021年2月至2022年9月武汉大学中南医院肝胆胰外科收治及会诊的3例肝脏PEComa病人的临床特点、影像学资料、手术治疗情况、病理结果及预后情况,复习国内外相关案例报道资料,对肝脏PEComa的临床诊疗要点展开分析。结果3例病人均为女性,年龄39~57岁,均无肝炎、肝硬化病史,肿瘤标志物无异常,术前影像学均误诊为肝细胞癌。3例病人均行腹腔镜下肝切除术,术后恢复良好,随访6~24个月无复发。文献复习共纳入452例肝脏PEComa病例,女性病人占76.55%(346/452),中位发病年龄在30~51岁之间。有临床表现的病人占30.36%(119/392),术前影像学诊断率仅为16.92%(55/325),最易误诊为肝细胞癌(占32.62%)。免疫组织化学结果表明主要的肿瘤标记阳性为HMB45(90.00%)、Melan-A(80.29%)和平滑肌肌动蛋白(SMA)(76.61%)。5.08%的病例出现术后复发。结论肝脏PEComa在临床上罕见,术前诊断难与肝细胞癌相鉴别,具有一定的恶性潜能,确诊依赖于病理检查。手术根治性切除是肝脏PEComa的主要治疗手段,吲哚菁绿荧光导航技术在术中辅助肿瘤定位可能具有一定的优势。

Objective To explore the clinical features,diagnosis and surgery of perivascular perivascular epithelioid cell tumor(PEComa).Methods From February 2021 to September 2022,clinical characteristics,imaging data,surgical approaches,pathological examinations and prognoses were retrospectively reviewed for three female patients with hepatic PEComa aged(39-57)years.And clinical diagnostic and therapeutic points were analyzed along with the relevant case reports at home and abroad.Results None of them had a previous history of hepatitis cirrhosis.There were no abnormalities in tumor markers.Preoperative imaging hinted at hepatocellular carcinoma(HCC).After laparoscopic liver resection,all of them recovered well postoperatively.There was no recurrence during a follow-up period of(6-24)months.A total of 452 cases were retrieved through a literature review.Most subjects were females(76.55%,346/452)with a median onset age of(30-51)years.And 30.36%of subjects had clinical manifestations and preoperative imaging diagnostic rate was merely 16.92%(55/325).The most probable misdiagnosis was HCC(32.62%).Immunohistochemistry revealed that HMB45(90.00%),Melan-A(80.29%)and small muscle action(SMA,76.61%)were three major tumor markers.Postoperative recurrence occurred in 5.08%of subjects.Conclusion Hepatic PEComa is clinically rare and preoperative diagnosis is difficult to distinguish it from HCC.It has some malignant potentials.A definite diagnosis is dependent upon pathological examination.Radical surgical resection is a gold standard for hepatic PEComa.And intraoperative navigation technology of indocyanine green fluorescence offers some advantages in assisting tumor localization.