儿童肾横纹肌样瘤的影像表现及误诊分析

Imaging Features and Misdiagnosis Analysis of Malignant Rhabdoid Tumor of the Kidney in Children

目的分析儿童肾横纹肌样瘤(MRTK)的影像特征,总结误诊原因,加深认识,提高诊断符合率。方法回顾本院经病理证实的10例儿童MRTK的影像资料,所有病例均行CT扫描,其中1例另行MRI检查,总结其影像及临床特点,分析误诊原因。结果10例患儿发病年龄区间为2月11天至3岁4个月,肿瘤均为单侧发病,其中右肾4例,左肾6例,肿瘤体积多较大,密度混杂,瘤体内存在不同程度的液化坏死;7例肾包膜受累增厚;6例肾盂受侵;5例存在包膜下积液;3例瘤内见新鲜出血;2例肿瘤内见钙化灶。5例术前存在误诊,其中4例误诊为肾母细胞瘤,1例误诊为肾透明细胞肉瘤。结论MRTK与其他儿童肾肿瘤影像所见极为相似,术前易被误诊,发病年龄小,预后差,病程进展快,集合系统受累,瘤内出血及肾周积液为其主要特征,有助于MRTK的鉴别及诊断。

Objective Objective to analyze the imaging features of renal rhabdomyoma(MRTK)in children,summarize the causes of misdiagnosis,deepen the understanding and improve the diagnostic coincidence rate.Methods the imaging data of 10 children with MRTK confirmed by pathology in our hospital were reviewed.All cases underwent CT scan,and 1 case underwent MRI examination.Results the age range of 10 cases was from 2 months and 11 days to 3 years and 4 months.The tumors were unilateral in 4 cases of right kidney and 6 cases of left kidney.The tumor volume was large and the density was mixed.There were different degrees of liquefaction necrosis in the tumor body.7 cases had renal capsule thickening;6 cases had renal pelvis invasion;5 cases had subcapsular effusion;3 cases had fresh hemorrhage;2 cases had calcification.5 cases were misdiagnosed preoperatively,including 4 cases of Wilms tumor and 1 case of clear cell sarcoma of the kidney.Conclusion MRTK is very similar to other renal tumors in children.It is easy to be misdiagnosed before operation.It is characterized by small age,poor prognosis,rapid progress of disease course,involvement of collecting system,intratumoral hemorrhage and perirenal effusion,which is helpful for the differential diagnosis of MRTK.