小儿肾外非中枢神经系统恶性横纹肌样瘤临床及影像学表现  

The Imaging and Clinical Characteristics of Extrarenal Extracranial Rhabdoid Tumor in Children

作　　者：徐守军[1] 干芸根[1] 杨春兰[2] 曹娟[3] 郭学军[5] 刘龙平 刘根 徐华[4] 罗娜[1] 侯明君 XU Shoujun;GAN Yungen;YANG Chunlan(Department of Radiology,Shenzhen Childrens Hospital,Shenzhen,Guangdong Province 518026,P.R.China)

机构地区：[1]深圳市儿童医院放射科,518026 [2]深圳市儿童医院血液肿瘤科,518026 [3]深圳市儿童医院病理科,518026 [4]深圳市儿童医院统计室,518026 [5]北京大学深圳医院医学影像科,深圳518036

出　　处：《临床放射学杂志》2022年第2期308-313,共6页Journal of Clinical Radiology

基　　金：深圳市医疗卫生三名工程项目资助(编号:SZSM202011005,SZSM201612036)。

摘　　要：目的探讨小儿肾外非中枢神经系统恶性横纹肌样瘤(EERT)的临床及影像学表现。方法回顾性分析2013年10月至2020年9月本院收治的8例EERT患儿(10个病灶)的病例资料。8例10个病灶术前均行CT平扫及增强扫描,其中3例3个病灶同时行MRI平扫、增强扫描及扩散加权成像(DWI)检查。结果 8例患儿中,病灶位于左颈部1例(1个病灶),纵隔2例(2个病灶),腹膜后3例(4个病灶),左前臂及肝左叶1例(2个病灶),盆腔1例(1个病灶)。病灶1个呈梭形,2个分叶状,7个类圆形。瘤体均较大,病灶最大径线为34~149 mm,平均(73±35)mm。CT平扫主要表现为以实性为主的不均质囊实性肿块,囊实性分界不清,呈渐变性改变。MRI主要显示为不均匀T_(1)WI等或稍低信号,T_(2)WI高或稍高信号,实性部分DWI呈高信号。增强扫描不均匀强化,其中6个病灶呈渐进性强化,囊变及坏死区无强化。部分病灶伴钙化、出血、瘤周积液、邻近骨质破坏及向椎管内延伸、局部或远处侵犯、转移等。患儿短期内可出现贫血、电解质紊乱、低蛋白血症、恶病质以及感染等。结论小儿EERT恶性程度高、并发症多、进展迅速,影像学表现具有一定的特征性,对提高该病诊断的敏感性和准确性具有重要意义。Objective To investigate the imaging and clinical characteristics of extrarenal extracranial rhabdoid tumor(EERT) in children.Methods We conducted a retrospective analysis of the 8 children with EERT(10 lesions) con-firmed by pathologically in our hospital from October 2013 to September 2020. All 8 cases(10 lesions) were examined withplain and enhanced CT scans preoperative, 3 cases(3 lesions) underwent plain and enhanced MRI scans,as well as DWI,simultaneously.Results Among the 8 children,1 with EERT located in the left neck(1 lesion),2 in the mediastinum(2 lesions),3 in the retroperitoneum(4 lesions), 1 in the left forearm and the left lobe of liver(2 lesions)and 1 in pelviccavity(1 lesion). Among the 9 lesions,1 was fusiform,3 were lobulated and 5 were quasi-circular. The tumors were alllarge,and the maximum diameter of the lesions ranged from 34 mm to 149 mm,with an average of(73 ± 35)mm. The mainfindings were heterogeneous solid mass,mostly with cystic degeneration,and the boundary between cystic and solid was notclear,Characterized by changing gradually on Non-enhanced CT. MRI mainly showed inhomogeneous isointensity or hypoin-tense on T_(1)WI and slightly hyperintensity or hyperintensity on T_(2)WI,and hyperintensity on DWI in the solid part. Showeduneven enhancement after contrast-enhanced scanning,6 lesions showed were gradual strengthening,and no enhancement incystic degeneration and necrotic areas. In addition,some lesions were accompanied by calcification,hemorrhage,effusion a-round the tumor,destruction of adjacent bone and extension to the spinal canal,local or distant invasion and metastasis. A-nemia,electrolyte disturbance,hypoproteinemia,cachexia and infection can occur in a short period of time.Conclusion EERT has high malignant degree,many complications and rapid progress,the imaging manifestations of EERT in childrenare of great significance for improving the sensitivity and accuracy of EERT diagnosis.

关 键 词：恶性横纹肌样瘤 肾外非中枢神经系统恶性横纹肌样瘤 体层摄影术 X线计算机 磁共振成像 

分 类 号：R737.11[医药卫生—肿瘤]