UNUSUAL PRESENTATION OF NEPHROBLASTOMATOS IS

Background. - Nephrogenic rests generally constitute precursor lesions of Wilms' tumor. We report a case of right nephroblastomatosis with d ysmorphic features. Case report. - An enlargement of the right kidney was incidentally discovered in a 1-year-old girl with dysmorphic featu res but normal psychomotor development. Combined ultrasonography and c omputerized tomography (CT) scan showed right cortical nephroblastomat osis. Chemotherapy using actinomycin D and vincristin was successful; however, an hyperechogenic nodule was subsequently found, necessitatin g a right nephrectomy. Conclusion. - The relationship between nephrobl astomatosis and Wilms' tumor is discussed. This case report reminds us of the importance of a long-term follow-up including echography and C T scan in cases of nephroblastomatosis. (C) 1998 Elsevier, Paris.