肝脏恶性血管周上皮样细胞瘤的CT表现特征

CT features of malignant perivascular epithelioid cell carcinoma of the liver

目的探讨肝脏恶性血管周上皮样细胞瘤(PEComa)患者的CT表现特征。方法回顾性分析2010年1月1日至2022年6月30日在浙江省乐清市人民医院、温州市人民医院和永嘉县人民医院经手术后病理确诊为肝脏恶性PEComa的9例患者的临床资料,其中男性2例,女性7例,年龄47(43,56)岁。记录患者的CT表现,观察肿瘤大小、形态、界限、密度及增强后强化等情况。结果CT平扫结果显示9例肿瘤患者均为单发病灶,其中位于右肝5例,左肝3例,尾叶1例;中位肿瘤最大径为5.7 cm,范围3.3~16.0 cm。9例患者中:肿瘤形状呈圆形3例,类圆形4例,不规则形2例;界限清楚8例,不清楚1例;密度不均匀、低于肝脏9例;肿瘤有假包膜4例,瘤内含脂肪组织1例,坏死囊变6例。CT增强扫描结果显示9例患者于动脉期中度强化2例,显著强化7例;门静脉期及延迟期强化程度均明显下降,呈"快进快出"表现4例;门静脉期及延迟期仍持续强化,呈"快进慢出"表现4例;增强于动脉期、静脉期和延迟期均未见强化,病灶内见条状强化血管影1例;病灶周边可见明显增粗血管影5例。结论PEComa的CT表现为病灶呈圆形、类圆形及不规则形,界限清楚,瘤内易囊变,多呈不均匀低密度,增强动脉期显著强化,门静脉期快速退出或持续强化。

ObjectiveTo study the CT manifestations of malignant hepatic perivascular epithelioid cell carcinoma(PEComa).MethodsClinical data of nine patients undergoing surgery with histologically confirmed malignant hepatic PEComa in Yueqing People's Hospital,Wenzhou People's Hospital,and Yongjia County People's Hospital from January,2010 to June,2022 were retrospectively collected,including two males and seven females with a median age of 47(43,56)years old.The CT findings,including tumor size,shape,boundary,density,and enhancement patterns,were analyzed.ResultsCT scans showed that all nine tumors were single lesions.Five tumors were located in right liver lobe,three in left lobe,and one in caudate lobe.The median tumor diameter was 5.7(range,3.3-16.0)cm.In terms of tumor shape,three were round,four were quasi-circular,and two were irregular.Eight tumors had well-defined boundaries,while one was ill-defined.Nine tumors showed uneven densities and were lower than the adjacent liver parenchyma.Four tumors had a false capsule,one contained fatty tissue,and six had necrotic and cystic changes.In the arterial phase of contrast-enhanced CT scanning,two tumors showed moderate enhancement and seven showed significant enhancement.In the portal venous and delayed phases,enhancement decreased significantly in four cases,showing a"fast-in and fast-out"pattern.In four cases,the enhancement persisted,showing a"fast-in and slow-out"pattern.One case showed no enhancement in any phase but had a strip-like enhanced vessel inside the tumor.Five cases had significantly thickened vessels around the tumor.ConclusionThe CT manifestations of PEComa are as follows:round or quasi-circular lesions with well-defined boundaries,uneven low densities,significant enhancement in the arterial phase and rapid washout or persistent enhancement in the portal venous and delayed phases.