儿童肾恶性横纹肌样瘤CT表现

CT manifestations of malignant rhabdoid tumor of kidney in children

目的观察儿童肾恶性横纹肌样瘤(MRTK)的CT表现。方法回顾性分析经病理证实的19例MRTK患儿,观察其CT表现。结果19例MRTK中,11例以血尿为主诉就诊;病变位于左肾10例、右肾9例,均为单发病灶,位置多邻近肾门,最大径4.53~15.32 cm,平均(8.42±2.77)cm;其中11例呈类圆形,8例为不规则形;12例边缘呈分叶状,7例边缘基本光滑;13例边界不清,6例边界清楚;CT平扫呈混杂密度肿块,增强后肿瘤实性部分强化程度低于正常肾实质,内见多发坏死囊变;其中14例见较明显的偏心性囊变,囊实性交界区多模糊不清;12例瘤体内见出血,5例见细线状或砂砾状钙化;10例肾周包膜下积液/血,6例肾包膜增厚;9例侵犯肾盂或肾窦。术后病理提示9例侵犯肾包膜,4例淋巴结转移;免疫组织化学染色19例整合酶相互作用蛋白1(INI-1)均(-)。结论儿童MRTK的CT表现具有一定特征性,有助于诊断。

Objective To observe the CT manifestations of malignant rhabdoid tumor of kidney(MRTK)in children.Methods Data of 19 pediatric MRTK patients confirmed by pathology were retrospectively analyzed.Results Among 19 children,hematuria was the chief complaint symptom in 11 cases.Single MRTK lesions developed in left kidney in 10 cases,in right kidney in 9 cases,mostly located near the renal hilum,with the maximum diameter of 4.53-15.32 cm and the average diameter of(8.42±2.77)cm.The lesions shaped quasi-circularly 11 cases and irregularly in 8 cases,with lobed edge in 12 cases and smooth in 7 cases,the boundary was unclear in 13 cases and clear in 6 cases.Plain CT showed mixed density masses,and the enhancement degree of solid part was lower than that of normal renal parenchyma,and multiple necrotic cystic lesions were observed.Obvious eccentric cystic lesions were observed in 14 cases,and most of the cystic solid junction area was ambiguous.Hemorrhage was found in 12 cases,and fine line or gravel calcifications were found in 5 cases.Renal subcapsular fluid/blood accumulation were detected in 10 cases,renal capsule thickening in 6 cases,and renal pelvis or sinuses invasion were noticed in 9 cases.Pathological examination showed 9 cases with renal capsule invasion and 4 cases with lymph node metastasis.Immunohistochemical staining showed negative integrase interactor protein 1(INI-1)in all 19 cases.Conclusion CT manifestations of MRTK in children had certain characteristics.