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[Unusual presentation of nephroblastomatosis]
J Santiago1, R Bouvier, J M Pouillaude
1Service de pédiatrie, Centre Hospitalier, Albertville, France.
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.