小儿非中枢神经系统恶性横纹肌样瘤CT和MRI误诊分析  

Analysis of CT and MRI Misdiagnosis of Extracranial Rhabdoid Tumor in Children

作　　者：徐守军[1] 干芸根[1] 杨春兰[2] 曹娟[3] 刘龙平 刘根 徐华[4] 罗娜[1] 侯明君 XU Shoujun;GAN Yungen;YANG Chunlan;Cao Juan;LIU Longping;LIU Gen;XU Hua;LUO Na;HOU Mingjun(Department of Radiology,Shenzhen Children's Hospital 2 Department of Hematology and Oncology,Shenzhen Childr;erfs Hospital;Department of Pathology,Shenzhen Children's Hospital;Department of Statistics Room,Shenzhen Children's Hospital)

机构地区：[1]深圳市儿童医院放射科 [2]深圳市儿童医院血液肿瘤科 [3]深圳市儿童医院病理科 [4]深圳市儿童医院统计室

出　　处：《中国医学计算机成像杂志》2022年第2期177-182,共6页Chinese Computed Medical Imaging

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

摘　　要：目的:观察并总结小儿非中枢神经系统恶性横纹肌样瘤(ERT)的CT及MRI表现,并对误诊病例进行分析,以提高该病诊断正确率。方法:回顾性分析2013年10月至2020年9月我院收治的10例ERT患儿(12个病灶)的病例资料,术前均行CT平扫及增强检查,其中3例3个病灶同时行MRI平扫、增强及DWI检查。结果:10例患儿中,病灶位于左颈部1例(1个病灶),左肾1例(1个病灶),右肾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)。病灶呈梭形1个,分叶状3个,类圆形8个。瘤体均较大,最大径线为34~149mm,平均(72.58±31.74)mm。CT平扫主要表现为以实性为主的不均质囊实性肿块,边界不清;增强后病灶不均匀强化,其中7个呈渐进性强化,1个呈花瓣样强化。MRI显示为不均匀T1WI低信号影,T2WI高信号影,实性部分弥散受限。增强扫描不均匀强化。12个病灶中4个瘤内见钙化、2个瘤内出血、4个瘤周积液、2个邻近骨质破坏、3个向椎管内延伸、3例有局部或远处侵犯、转移。因病灶多发于脊柱旁,恶性程度高,易破坏邻近骨质及向椎管内延伸,血清神经元烯醇化酶升高等,10例(12个病灶)中6例(7个病灶)误诊为神经源性肿瘤,另1例(1个病灶)位于左颈部脊柱旁,误诊为神经源性肿瘤或横纹肌肉瘤;1例(1个病灶)位于右肾,误诊为肾母细胞瘤,1例(2个病灶)位于左前臂及肝左叶,未定性。结论:小儿ERT的CT和MRI表现多样,且与小儿其他常见恶性肿瘤具有许多相似性,容易误诊,但有一定的特征,全面了解ERT的影像学表现,分析误诊原因,有助于准确诊断。Purpose:To observe and summarize the CT and MRI imaging manifestations of extracranial rhabdoid tumor(ERT)in children,and analyze the misdiagnosed cases,aiming to improve the diagonsitic accuracy for this disease.Methods:Ten children with ERT(12 lesions)confirmed by pathological results in our hospital from October2013 to September 2020 were enrolled in this study.All 10 cases(12 lesions)were examined with plain and enhanced CT scans preoperatively,and 3 cases(3 lesions)underwent plain and enhanced MRI scans,as well as DWI,simultaneously.Results:Among the 10 children,1 case(1 lesion)was located in the left side of neck,1(1 lesion)in the left kidney,1(1 lesion)in the right kidney,2(2 lesions)in the mediastinum,3(4 lesions)in the retroperitoneum,1(2 lesions)in the left forearm and the left lobe of liver,and 1(1 lesion)in pelvic cavity.Among the 12 lesions,1 was fusiform,3 lobulated and 8 quasi-circular.The tumors were all large,and the maximum diameter of the lesions ranged from 34 mm to 149 mm,with an average of(72.58±31.74)mm.The main findings were heterogeneous solid mass,and the boundary was not clear on plain CT;and the lesions showed uneven enhancement on contrastenhanced CT,with 7 progressive enhancement and 1 petal-like enhancement.MRI showed inhomogeneous hypointense on T1WI and hyperintensity on T2WI,and slightly hyperintensity on DWI in the solid part.Uneven enhancement was shown on contrast-enhanced scanning.Among the 12 lesions,calcification was found in 4 lesions,hemorrhage in 2 lesions,peritumoral effusion in 4 lesions,destruction of adjacent bone in 2 lesions,extension to spinal canal in 3 lesions,and local or distant invasion and metastasis in 3 lesions.As the lesions are frequently located in the spinal column,the degree of malignancy is high,easy to destroy adjacent bone and extend into the spinal canal,the serum neuronal enolase is elevated,of the 10 cases(12 lesions),6 cases(7 lesions)were misdiagnosed as neurogenic tumor,1 case(1 lesion)was misdiagnosed as neurogenic tumor or rhabdomyosarcoma

关 键 词：恶性横纹肌样瘤 非中枢神经系统恶性横纹肌样瘤 计算机体层成像 磁共振成像 误诊分析 

分 类 号：R445.2[医药卫生—影像医学与核医学]