Santiago J, Bouvier R, Pouillaude J M, Thiesse P, Bergeron C, Chapuis J P, Cochat P, Lespinasse J
Service de pédiatrie, Centre Hospitalier, Albertville, France.
Arch Pediatr. 1998 Jun;5(6):627-32. doi: 10.1016/s0929-693x(98)80166-8.
Nephrogenic rests generally constitute precursor lesions of Wilms' tumor. We report a case of right nephroblastomatosis with dysmorphic features.
An enlargement of the right kidney was incidentally discovered in a 1-year-old girl with dysmorphic features but normal psychomotor development. Combined ultrasonography and computerized tomography (CT) scan showed right cortical nephroblastomatosis. Chemotherapy using actinomycin D and vincristin was successful; however, an hyperechogenic nodule was subsequently found, necessitating a right nephrectomy.
The relationship between nephroblastomatosis and Wilms' tumor is discussed. This case report reminds us of the importance of a long-term follow-up including echography and CT scan in cases of nephroblastomatosis.
肾源性残留通常是威尔姆斯瘤的前驱病变。我们报告一例具有畸形特征的右肾母细胞瘤病病例。
一名1岁女童具有畸形特征但精神运动发育正常，偶然发现右肾增大。超声检查和计算机断层扫描（CT）显示为右皮质肾母细胞瘤病。使用放线菌素D和长春新碱进行化疗成功；然而，随后发现一个高回声结节，需要进行右肾切除术。
讨论了肾母细胞瘤病与威尔姆斯瘤之间的关系。本病例报告提醒我们，对于肾母细胞瘤病患者，包括超声检查和CT扫描在内的长期随访很重要。
