肝脏血管周上皮样细胞肿瘤的临床病理观察  

Clinicopathological features of hepatic perivascular epithelioid cell neoplasm

作　　者：赵苏苏 余慧萍[2] 徐佳佳[3] 周家华[4] 章宜芬 余泽前[4] ZHAO Susu;YU Huiping;XU Jiajia;ZHOU Jiahua;ZHANG Yifen;YU Zeqian(Department of Pathology,Affiliated Hospital of Nanjing University of Chinese Medicine,Nanjing 210029,China)

机构地区：[1]南京中医药大学附属医院、江苏省中医院病理科,南京210029 [2]南京大学医学院附属鼓楼医院病理科,210008 [3]东南大学附属中大医院病理科,210009 [4]东南大学附属中大医院肝胆胰中心,210009

出　　处：《临床肿瘤学杂志》2023年第3期258-262,共5页Chinese Clinical Oncology

基　　金：国家自然科学基金青年基金资助项目(81502505);江苏省卫生健康委医学科研项目(M2020062)。

摘　　要：目的探讨肝脏血管周上皮样细胞肿瘤(PEComa)的临床病理学特征、诊断、治疗及预后。方法回顾性分析2016年10月至2021年3月10例肝脏PEComa的临床表现、影像学表现、组织病理学特征、治疗及预后。结果10例患者中,女性7例,男性3例;年龄30~74岁;除1例有背部疼痛、腹胀外,其余9例均无相关临床症状;肝左叶2例,肝右叶8例;肿瘤最大径为0.8 cm~6.5 cm,肿块与周围肝脏组织界限均较清楚;术前影像学检查仅2例诊断倾向PEComa;10例患者均接受手术切除;术后随访5~58个月,10例患者中除1例失访外均无复发和转移。显微镜下肿瘤细胞主要由上皮样细胞和脂肪细胞组成,以上皮样细胞占优势。免疫组化检查显示,肿瘤细胞均表达HMB-45、Malen-A、SMA和Vimentin,不表达CK、EMA、AFP和CD117,p53呈野生型,Ki-67均<5%。结论肝脏PEComa在影像学和病理形态学上都可以高度模拟肝细胞来源的肿瘤或者病变,其诊断和鉴别诊断应结合其特殊的影像学、病理形态学和免疫表型。肝脏PEComa患者预后良好。Objective To investigate the clinicopathological features,diagnosis,treatment and prognosis of hepatic perivascular epithelioid cell neoplasm(PEComa).Methods A retrospective analysis was done on 10 cases of hepatic PEComa from October 2016 to March 2021.The clinical features,imaging findings,histopathological findings and immunophenotypes of hepatic PEComa were analyzed.Results There were 7 females and 3 males,and they were aged from 30 to 74;Except 1 case had back pain and abdominal distension,the other 9 cases had no related clinical symptoms;Two cases occurred in the left lobe of liver and 8 cases occurred in the right lobe of liver;The maximum diameter of tumor ranged from 0.8 cm-6.5 cm,and the boundary between the tumor and the surrounding liver tissues was clear;Preoperative imaging examination showed that only 2 cases tended to PEComa;All 10 patients received surgical treatment;Postoperative follow-up ranged from 5 months to 58 months.Except 1 case lost follow-up,there was no recurrence and metastasis in 9 patients.Under the microscope,tumor cells were mainly composed of epithelioid cells and adipocytes,and the epithelioid cells were dominant.Immunohistochemistry showed that HMB-45,Malen-A,SMA and Vimentin were expressed in tumor cells;CK,EMA,AFP and CD117 were not expressed;The expression of p53 was wild-type and the expression of Ki-67 was less than 5%.Conclusion Hepatic PEComa can highly simulate hepatocellular tumors or lesions in imaging and pathomorphology.Its diagnosis and differential diagnosis should be combined with its special imaging,pathomorphology and immunophenotype.Patients usually have good prognosis.

关 键 词：肝脏肿瘤 血管周上皮样细胞肿瘤 临床病理特征 

分 类 号：R735.7[医药卫生—肿瘤]