肝脏上皮样血管周细胞瘤的影像学表现及免疫组织化学特点分析

Imaging Manifestations and Immunohistochemistry of Hepatic Perivascular Epithelioid Cell Tumor

目的探讨肝脏上皮样血管周细胞瘤(PEComa)的影像学表现特点,以提高诊断的准确性。方法回顾性分析2014年1月至2020年10月苏州大学附属第一医院经病理证实的11例肝脏PEComa患者的相关CT和MR影像资料,包括发病部位、直径、信号特点、强化方式和病灶周围情况等,并复习相关文献。结果11例患者中,病灶位于肝左叶6例,肝右叶4例,肝左叶及右叶同时发生者1例,共计12个病灶。12个病灶直径范围:5 cm以上4个,1~5 cm 7个,1 cm以下1个。平扫表现:9例CT扫描中5例密度均匀,4例密度不均,9例MRI病例中T1WI均呈低信号,T2WI均为稍高信号,其中T2WI信号不均匀者5例(病灶内出血1例,含脂肪者4例),所有病灶边界均清晰。增强扫描表现:增强后动脉期均可见强化,均质强化3个,不均质强化8个(4个为边缘厚壁环形强化),未见实性强化成分1个;门静脉期至平衡期表现多样,可为高信号或等、低信号;其中5例患者行肝胆特异期增强(普美显)扫描,共计6个病灶,均未见摄取。5个病灶内部或边缘见到迂曲条状的供血血管或明显血管截面强化。免疫组织化学HMB45、Ki-67阳性表达率为100%,Melan-A、SMA、CD34和S-100部分表达阳性。结论肝脏PEComa的影像学表现及免疫组织化学具有一定的特点,对该病的诊断有重要价值。

Objective To investigate the imaging features of hepatic perivascular epithelioid cell tumor(PE Coma)and immunohistochemistry in order to improve the accuracy of diagnosis.Methods CT and MRI images of 11 cases of hepatic epithelioid hemangioblastoma confirmed by pathology were retrospectively analyzed in the First Affiliated Hospital,Soochow University,including the location,diameter,capsule,signal characteristics,enhancement modes,and the situation around the lesion,relevant literatures were reviewed as well.Results A total of 6 cases of tumor was located in the left hepatic lobe,4 cases in right lobe,and 1 case in both of them.The maximum diameter of the tumor was below 1 cm in 1 case,above 5 cm in 4 cases,and between 1-5 cm in 6 cases.The plain scan showed heterogeneous intensity in 4 cases,and intralesional hemorrhage in 1 case,lesion with fatty component in 4 cases.After enhancement,12 cases were enhanced in the arterial phase,3 cases were homogeneous enhancemed,8 cases were heterogeneous enhancemed(4 cases with remarked edge enhancement),and 1 case without solid nodules.In the portal and delayed phase,12 cases showed varied signal intensity,is or slightly hypointensity.There are 5 cases with tortuous vessels or vascular sectional in the center of lesions or at the periphery.Immunohistochemistry examinations showed that HMB45,Ki-67 were positive in all cases,in somecases Melan-A,SMA,CD34 and S-100 expressed positive.Conclusion Imaging manifestations and immunohistochemistry of hepatic perivascular epithelioid cell tumors have certain characteristics,and have good diagnostic value for this disease.