儿童肾透明细胞肉瘤CT诊断及误诊分析

CT Diagnosis and Misdiagnosis of Clear Cell Sarcoma of Kidney in Children

目的 分析儿童肾透明细胞肉瘤(CCSK)的影像表现,对本病影像学与临床表现加以总结,探究误诊因素,提高对本病认知度,提升诊断符合率。方法 对河南省儿童医院由病理确诊11名CCSK患者的影像资料展开回顾分析,全部患儿皆行CT扫描,对其影像与临床表现加以总结,并对误诊因素展开分析。结果 肿瘤皆是单侧发病,其中5例为右肾,6例为左肾,11例患儿发病年龄区间为5月9天至10岁,肿瘤多较大,密度混杂,均为单发,10例有囊变;9例肿瘤内见多发细小动脉,实性成分呈延迟渐进性强化,2例伴下腔静脉、同侧肾静脉癌栓。7例肾周血管充盈,骨转移与钙化灶分别为2例,包膜下积液1例。术前误诊10人,其中8人误诊为肾母细胞瘤(WT),1例误诊为先天性中胚叶肾瘤,1例误诊为肾脏横纹肌样瘤。结论 在影像表现上,CCSK和其它儿童肾肿瘤尤其是肾母细胞瘤高度接近,术前误诊几率高,发病年龄小,具不良预后,病程进展快,易发生囊变、坏死,增强肿瘤内多发细小动脉、延迟渐进性强化,还有鱼肉状、虎斑状、条纹状或者云絮状强化,肾周血管充盈多见,有助于与其他肾肿瘤相鉴别。

Objective To analyze the imaging featu res of clear cell sa rcoma of kidney(CCSK) in children,summa rize the imaging and clinical features of CCSK,explore the factors of misdiagnosis,improve the recognition of CCSK,and improve the diagnostic coincidence rate.Methods The imaging data of 11 patients with CCSK diagnosed by pathology in Henan Children s Hospital were retrospectively analyzed.All the children underwent CT scan.The imaging and clinical manifestations were summa rized,and the factors of misdiagnosis were analyzed.Results The tumors were unilateral in all cases,including 5 cases in the right kidney and 6 cases in the left kidney.The age ra nge of the 11 cases was from May 9 to 10 years old.The tumors were mostly large and mixed in density,all of them were single,and 10 cases had cystic change.Multiple small arteries were found in 9 cases,and the solid components showed delayed and progressive enhancement.2 cases were accompanied by inferior vena cava and ipsilateral renal vein cancer embolus.There were 7 cases of perirenal vascular filling,2 cases of bone metastasis and calcification,and 1 case of subcapsular effusion.10 patients were misdiagnosed with nephroblastoma(WT) before operation,of which 8 were misdiagnosed with WT,1 was misdiagnosed with congenital mesoblastoma,and 1 was misdiagnosed with renal rhabdomyoma.Conclusion In terms of imaging manifestations,CCSK is highly similar to other pediatric renal tumors,especially nephroblastoma,with high probability of preoperative misdiagnosis,young age of onset,poor prognosis,ra pid progression of the disease,prone to cystic change and necrosis,enhancement of multiple small a rteries in the tumor,delayed progressive enhancement,and fish,tiger,stripe or cloudy enhancement.Perirenal vascular filling is common and helps to distinguish it from other renal tumors.