儿童肾透明细胞肉瘤的CT与MRI表现

Imaging Findings of Clear Cell Sarcoma of the Kidney in Children

目的 探讨肾透明细胞肉瘤（CCSK）的CT与MRI表现特点。方法 回顾性分析7例手术病理证实的CCSK患者（男3例,女4例,年龄11个月~7岁,平均3.8岁）的影像学资料,其中5例CT检查,2例MRI检查,均完成增强扫描。结果 肿瘤均为单发,均位于肾髓质内,瘤体最大径2~15.8 cm;所有肿瘤内均有不同程度的液化坏死,其密度/信号强度不均匀,2例CT上见点状钙化,3例CT上见瘤体内明显肿瘤血管;2例MRI扩散成像显示病灶呈高信号。所有病灶增强扫描后呈不均匀中等度强化,全部病例均见远处转移：腹膜后淋巴结转移（4例）,骨转移（3例）和肺转移（3例）。结论 肾透明细胞肉瘤有一定影像学特点,通常瘤体大,密度/信号不均,不均匀的中等度强化,可有钙化,CT增强容易显示瘤内肿瘤血管,磁共振扩散成像呈高信号,远处转移早。

Objective To explore the imaging characteristics of clear cell sarcoma of the kidney （CCSK） in children so as to improve the diagnosis. Methods We retrospectively analyzed the images of CCSK confirmed by surgery and pa- thology, in seven patients （3 males, 4 females ; age from 7 months to 1 lyears, mean 3.8 years）. CT scanning was underg- one in 5 patients and MRI in 2 patients. The enhanced scanning was undergone in all patients. Results All of the CCSK tumor was solitaty and occurred in the renal medulla. The maximun diameter of the lession ranged from 2cm to 15.8cm. Mainly attributed to various degree of intra tumoral necrosis, the tumor appeared obviously heterogeneous density or intensity. Calcification （2） and tumoral vessels （3） were seen in CT scan. Hyper intensity of diffusion weighed imaging was seen in all of two MRI scans. The heterogeneously moderate enhancement was seen in all exams. The distant metastases were seen in all patients, including 4 lymph node , 3 bone, and 3 lung metastases. Conclusion The CCSK would have some characteristic imaging findings, including a huge heterogeneous mass, moderate enhancement, calcification, tumoral vessels in contrasted CT scan and hyper intensity in DWI. Also they metastasise early.