CT诊断儿童畸胎瘤样肾母细胞瘤与文献复习

CT diagnosis and literature review of teratoid Wilms tumor in children

目的观察畸胎瘤样肾母细胞瘤(TWT)的CT表现、分型及其临床价值。方法收集本院7例CT资料完整、经病理证实的儿童TWT,并以"teratoid Wilms tumor"及"teratoid nephroblastoma"为主题词,在PubMed及Embase上检索相关文献,回顾性分析儿童TWT的CT表现,并进行CT分型。结果本院7例(单发)、文献22例(19例单发、3例双肾病变)儿童TWT共32个病灶,其中肾内27个、肾外5个(腹膜后间隙4个、骶尾部1个),CT均表现为轻度非均质强化的混杂密度肿块;其中Ⅰ型11个(11/32,34.38%),4个(4/32,12.50%)Ⅰa型明确显示瘤内脂肪组织及钙化灶,7个(7/32,21.88%)Ⅰb型瘤内未见明确钙化灶;Ⅱ型21个(21/32,65.63%),瘤内未见明确脂肪组织但可测出负CT值脂肪成分,6个(6/32,18.75%)Ⅱa型显示瘤内钙化灶,15个Ⅱb型(15/32,46.88%)瘤内未见钙化灶。结论儿童TWT的CT表现颇具特征性,可分为2个类型、4个亚型,对术前诊断和鉴别诊断具有重要价值。

Objective To observe CT features and classification of teratoid Wilms tumor(TWT) and their clinical values. Methods CT data of 7 children with pathologically proved TWT in our hospital were collected. Meanwhile, related literature were searched through Pubmed and Embase by putting into keywords of "teratoid Wilms tumor" and "teratoid nephroblastoma". CT manifestations of children TWT were retrospectively analyzed and classified. Results Totally 32 lesions of children TWT were enrolled, including 7 cases of single lesion in our hospital and 22 cases from literature(19 cases with single lesion and 3 with birenal lesions), 27 were renal and 5(4 in retroperitoneal space and 1 in sacrococcygeal site) were extrarenal lesions. On CT, lesions of children TWT appeared all as mixed-attenuation and slightly heterogeneous enhanced mass with different types. Type Ⅰ was identified in 11 lesions(11/32, 34.38%), showing clearly intratumoral adipose tissue with(type Ⅰa, 4 lesions, 12.50%) or without(type Ⅰb, 7 lesions, 21.88%) calcifications, while type Ⅱ in 21 lesions(21/32, 65.63%) showed no fat but negative CT value components in tumor with(type Ⅱa, 6 lesions, 18.75%) or without(type Ⅱb, 15 lesions, 46.88%) intratumoral calcifications. Conclusion CT images of children TWT can be characteristically classified into 2 types and 4 subtypes, which is of great value for preoperative diagnosis and differential diagnosis of this disease.