儿童肝脏未分化胚胎性肉瘤的影像学表现及临床特征  

Imaging and Clinical Features of Undifferentiated Embryonal Sarcoma of the Liver in Children

作　　者：董雪 李金凝 倪沈军 陆征海 汪登斌[1] 刘欢欢[1] DONG Xue;LI Jinning;NI Shenjun(Department of Radiology,Xinhua Hospital,Shanghai Jiao Tong University School of Medicine,Shanghai 200092,P.R.China)

机构地区：[1]上海交通大学医学院附属新华医院放射科,200092

出　　处：《临床放射学杂志》2025年第2期356-361,共6页Journal of Clinical Radiology

基　　金：上海申康医院发展中心市级医院新兴前沿技术联合攻关项目(编号:SHDC12021119);国家自然科学基金项目(编号:82001762)。

摘　　要：目的探讨儿童肝脏未分化胚胎性肉瘤(UESL)的影像学表现及临床特征。方法回顾性分析经手术病理证实的10例UESL患儿的临床和影像学资料,分析总结其特征表现。结果10例UESL中,9例行CT检查,1例同时行CT和MRI检查;7例位于肝右叶,1例位于肝左叶,2例同时位于肝左、右叶。5例CA125升高,1例AFP升高,2例CA19-9升高,2例发生肺多发转移。影像学表现为单房或多房巨大囊实性肿块,9例单发且形态规整,1例多发、形态不规则;7例包膜完整,3例包膜破溃;其中3例病灶边缘见点状、条状钙化,7例病灶内部见出血。增强扫描动脉期10例病灶均呈条片状不均匀、轻-中度强化,其中4例病灶以边缘强化为主;5例病灶动脉期见增粗、迂曲血管,延迟期病灶均呈持续、不均匀强化;1例呈渐进性强化;6例强化范围增大,其中1例边缘持续强化且由周边向中心充填。结论儿童UESL影像表现具有一定特征性,结合临床资料,有助于提高术前诊断准确率。Objective To investigate the imaging and clinical features of undifferentiated embryonal sarcomas of the liver(UESL)in children.Methods The clinical data and imaging features of 10 children with UESL confirmed by surgical pathology were analyzed retrospectively and summarized.Results Among the 10 patients of UESL,9 patients had undergone CT examination and 1 case undergone both CT and MRI examinations.A total of 7 cases were located in the right lobe of the liver,1 case in the left lobe,and 2 cases in both the left and right lobes of the liver.Elevated CA125,elevated AFP,elevated CA19-9 were detected in 5,1,and 2 cases,respectively.There were 2 cases with multiple pulmonary metastases.The imaging findings were single or multilocular giant intrahepatic cystic solid mass,while 9 cases had a single lesion with regular shape,and 1 case with multiplelesionsin irregular shape.Totally 7 cases had intact capsules and 3 cases showed capsule rupture.Spot and strip calcifications were detected at the edge of the lesions in 3 cases,and hemorrhage was found in 7 cases.During the arterial phase,10 lesions showed slightly or moderately heterogeneous strip and/or patchy enhancement.Among them,4 cases displayed marginal enhancement and 5 were detected with thickened and tortuous vessels.The lesions presented continuously heterogeneous enhancement,with consistent enhancement at the edge and filling from the periphery to the center in 1 case,and progressive enhancement being found in 1 case in the delayed phase.Conclusion The imaging findings of UESL in children have certain characteristics.Combined with clinical data,they are helpful in improveing the accuracy of preoperative diagnosis.

关 键 词：肝脏未分化胚胎性肉瘤 体层摄影术 X线计算机 磁共振成像 

分 类 号：R73[医药卫生—肿瘤]