肝脏血管周上皮样细胞肿瘤临床诊治分析  

Clinical diagnosis and treatment of liver perivascular epithelioid cell neoplasms

作　　者：曹丽娟 宋涛[1] 姚英民[1] 廉洁[3] 刘青光[1] 韩少山[1] Cao Lijuan;Song Tao;Yao Yingmin;Lian Jie;Liu Qingguang;Han Shaoshan(Department of Hepatobiliary Surgery,the First Affiliated Hospital of Xi’an Jiaotong University,Xi’an 710061,China;Department of General Surgery,Affiliated Hospital of Shaanxi University of Traditional Chinese Medicine,Xianyang 712000,China;Department of Pathology,the First Affiliated Hospital of Xi’an Jiaotong University,Xi’an 710061,China)

机构地区：[1]西安交通大学第一附属医院肝胆外科,西安710061 [2]陕西中医药大学附属医院普外科,咸阳712000 [3]西安交通大学第一附属医院病理科,西安710061

出　　处：《中华肝胆外科杂志》2022年第10期721-725,共5页Chinese Journal of Hepatobiliary Surgery

基　　金：国家自然科学基金(81602566);陕西省自然科学基金(2022JM-535);中央高校科研业务基金(xzy012020050)。

摘　　要：目的:总结分析肝脏血管周上皮样细胞肿瘤(PEComa)的临床特征及预后。方法:回顾性分析西安交通大学第一附属医院2011年10月至2021年11月病理确诊为肝脏PEComa的12例患者的临床资料,其中男性1例,女性11例,中位年龄44岁,年龄范围20~63岁。收集总结患者临床表现、实验室检查、影像学特征、治疗方法、术后病理特征及治疗转归。术后采用电话随访或查询患者复诊记录方式进行随访。结果:12例肝脏PEComa患者中无症状者8例(66.7%),单发病灶者11例(91.7%)。均采取手术治疗,其中局部肿瘤切除10例(83.3%)、扩大半肝切除2例(16.7%)。增强CT示病灶为类圆形均匀低密度肿块,动脉期强化,有肝动脉分支走行其中,门静脉期及延迟期强化程度减低。12例患者术后病理均提示良性,免疫组化结果表明黑色素瘤细胞源标志物HMB45阳性率100.0%(12/12)、Melan-A阳性率83.3%(10/12)、平滑肌肌动蛋白阳性率91.7%(11/12)。中位随访时间为27个月,随访期内均未发现复发及转移。结论:肝脏PEComa好发于女性,症状隐匿,多为单发,与肝炎无相关性,影像学易与原发性肝癌及肝转移癌相混淆,良性率高,表达黑色素细胞源性及平滑肌细胞源性双重标志物,根治性手术切除治疗效果良好。Objective To analyze the clinical features and prognosis of liver perivascular epithelioid cell neoplasms(PEComa).Methods The clinical data of 12 patients with liver PEComa diagnosed by pathology at the First Affiliated Hospital of Xi'an Jiaotong University from October 2011 to November 2021 were retrospectively analyzed.There were 1 male and 11 females,with a median age of 44(range 20 to 63)years old.The clinical manifestations,laboratory examinations,imaging features,treatment methods,postoperative pathological features and treatment outcomes of these patients were collected and analysed.Postoperative follow-up by telephone or patient's follow-up records.Results Among the 12 patients with hepatic PEComa,8 patients(66.7%)were asymptomatic and 11 patients(91.7%)had a single lesion.All patients underwent surgical treatment,including local tumor resection in 10 patients(83.3%)and extended hemihepatectomy in 2 patients(16.7%).Enhanced CT showed the lesion to be a quasi-round homogeneous low-density mass,enhanced in arterial phase with hepatic artery branches in the lesion,and decrease in enhancement degrees in portal vein phase and delayed phase.Postoperative pathology of the lesions in all the 12 patients was benign.Immunohistochemical results showed that the positive rates of melanoma cell markers HMB45,Melan-A and smooth muscle actin were 100.0%(12/12),83.3%(10/12)and 91.7%(11/12)respectively.The median follow-up period was 27 months,and no recurrence or metastasis was found.Conclusion Hepatic PEComa occurred commonly in women with obscure symptoms.The lesion was mainly single and it had no correlation with hepatitis.It is easily confused with primary liver cancer and liver metastasis on medical imagings.PEComa expressed markers of both melanocyte and smooth muscle cell,and radical surgical resection resulted in good results.

关 键 词：肝脏 血管周上皮样细胞肿瘤 临床特征 影像学表现 治疗 

分 类 号：R735.7[医药卫生—肿瘤]