[Unusual presentation of nephroblastomatosis]

Abstract

Background: Nephrogenic rests generally constitute precursor lesions of Wilms' tumor. We report a case of right nephroblastomatosis with dysmorphic features.

Case report: 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.

Conclusion: 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.