儿童MiT家族异位性肾细胞癌的影像表现

Children MiT Family Translocation Renal Cell Carcinoma:Imaging Manifestations

目的分析儿童MiT家族异位性肾细胞癌(MiT-RCC)[包括Xp11.2易位/TFE3基因融合相关性肾细胞癌(Xp11.2 RCC)和t(6;11)(p21;q12)/TFEB基因融合相关性肾细胞癌t(6;11)RCC]的影像学表现特点,提高对这类疾病的认识及诊断水平。方法回顾性分析13例经病理证实的MiT-RCC患儿的临床及影像资料,Xp11.2 RCC 12例,t(6;11)RCC 1例。13例行CT扫描,8例行MRI扫描,分析并总结CT及MRI表现特点。结果12例Xp11.2 RCC患儿CT图像肿块大小不一,影像表现可分为4种类型,肿瘤实性部分密度偏高,可见出血、坏死、钙化,钙化多见,淋巴结转移多见,腹膜后转移淋巴结常伴有钙化,增强扫描延迟期强化程度稍下降。MRI信号混杂,可见坏死、出血、钙化等信号,大部分T1呈稍高信号,T_(2)呈稍低信号,DWI呈稍高信号,平均ADC值下降。1例t(6;11)RCC CT上表现为类圆形等密度影,边界清晰,密度均匀。结论MiT-RCC是儿童RCC的主要亚型,影像学表现具有一定特点。

Objective To analyze the imaging features of MiT family translocation renal cell carcinoma[including Xp11.2 RCC and T(6;11)RCC],and improve the understanding and diagnosis of this kind of disease.Methods The clinical and imaging data of 13 children with pathologically confirmed MiT family translocation renal cell carcinoma[12 cases of Xp11.2 RCC,1 case of t(6;11)RCC]were retrospectively analyzed.CT scans were performed in 13 cases and MRI scans were performed in 8 cases,and the features of CT and MRI were summarized.Results The CT images of 12 children with Xp11.2 RCC could be divided into 4 types according to the size,shape and lymph node metastasis of the tumor.The density of solid part of the mass was high,with bleeding,necrosis and calcification.Calcification was more common,and lymph node metastasis was more common.Retroperitoneal metastatic lymph nodes were often accompanied by calcification,and the degree of enhancement decreased in the delayed period after enhancement.MRI signals were mixed,including cystic degeneration,hemorrhage,calcification and other signals.Most T1showed slightly higher signal,T_(2)showed slightly lower signal,DWI showed slightly higher signal,and the average ADC value decreased.1 case of t(6;11)RCC CT showed circular iso-density shadow with clear boundary and uniform density.Conclusion MiT family translocation renal cell carcinoma is the main subtype of pediatric renal cell carcinoma,and its imaging findings have certain characteristics.