儿童肾恶性横纹肌样瘤的影像特征及病理学对照  

Correlation of radiological charicteristics of renal malignant rhabdoid tumor in children with pathology

作　　者：薛潋滟 朱铭[1] 钟玉敏[1] 周立霞[2] 

机构地区：[1]上海交通大学医学院附属上海儿童医学中心放射科,上海200127 [2] 河北医科大学第二医院医学影像科

出　　处：《实用放射学杂志》2015年第3期454-457,共4页Journal of Practical Radiology

摘　　要：目的：探讨儿童肾脏恶性横纹肌样瘤（MRTK）的CT、MRI特征并与病理学对照。方法对本院术后病理证实的10例MRTK进行回顾性分析。所有病例术前均行 CT平扫及增强扫描,其中2例另行 MRI检查。10例患儿肿瘤切除术后病理学行常规 HE染色后行免疫组化。结果10例 MRTK病灶呈起源于肾脏的巨大肿块,平均直径约9 cm。肿瘤侵蚀肾脏实质,破坏肾盂,可见“抱球征”、“融冰征”,伴肾被膜受侵及肾周积液。肿瘤内可见多发坏死囊变区,囊变部分密度不均,增强扫描肿瘤呈明显不均匀强化。部分伴肺、肝、腹膜及骨转移。MRI 与 CT 比较,肿瘤强化更显著。HE 染色见肿瘤由单一的横纹肌样细胞组成,表达Vim、EMA阳性,部分CK阳性,INI-1阴性。可见多发出血、坏死区,脉管内可见瘤栓。结论儿童肾脏 MRTK影像学特征性表现为肾脏较大肿块,瘤内出血、坏死,肾被膜及集合系统受累,肾周积液等。Objective To analyze the CT and MRI features of malignant rhabdoid tumor of the children’s kidney （MRTK）retrospectively in comparison with pathology.Methods 10 patients with MRTK confirmed by pathology were retrospectively analyzed.All patients underwent CT plain and enhanced scans,2 of whom underwent MRI.HE and immunohistochemical stainings in all patients were performed after surgery.Results ALL huge lesions were originated from the kidney with an average diameter of 9 cm.Destruction of the renal parenchyma,damage of the renal pelvis,invasion of the renal capsule and perirenal effusion were found with snowmelt sign and holding ball sign.The tumors showed heterogeneous enhancement on CT because of obvious necrosis and cystic degeneration,and the metastases were observed in lung,liver,peritoneum and bone.The enhancement of tumor on MRI was more prominent than that on CT.Pathological staining showed the tumor was composed of single rhabdomyoid cells with positive expressions of Vim and EMA, part positive expression of CK and negative expression of INI-1 .Multiple bleeding,necrosis,blood vessel invasion were seen easily. Conclusion The imaging features of kidney MRTK in children,characterized as larger lump with hemorrhage,necrosis,invasion of the the renal pelvis and perirenal effusion.

关 键 词：肾脏 恶性横纹肌样瘤 计算机体层成像 磁共振成像 病理学 

分 类 号：R737.11[医药卫生—肿瘤] R814.42[医药卫生—临床医学]