WILMS-TUMOR AND MULTICYSTIC DYSPLASTIC KIDNEY-DISEASE

Purpose: There is ongoing controversy concerning the management of mul ticystic dysplastic kidney disease, particularly with regard to the po tential for malignant transformation. Our report fuels the debate by a dding the 2 youngest patients in whom malignancy was present from birt h or developed subsequently. Materials and Methods: Two well documente d cases of malignancy associated with multicystic dysplastic kidney di sease are presented in 2 female infants (5 and 3 months old). The 5-mo nth-old female infant was followed for multicystic dysplastic kidney d isease and had no evidence of tumor either antenatally or at birth. Th e 3-month-old presented with hypertension and interventricular septal defect. A renal tumor was present on initial ultrasound. Results: Even though malignant degeneration is rare in multicystic dysplastic kidne y disease, 9 cases have been reported in the Literature so far. Of the se cases 3 were Wilms tumor, 5 were renal cell carcinomas and 1 mesoth elioma. Conclusions: Our 2 cases lend support to the surgical manageme nt of multicystic dysplastic kidney disease, particularly as nephrecto my can now be performed in a day surgery setting with minimal morbidit y. Only the risks of coexisting malignancy and possible malignant dege neration transformation are specifically addressed in this article. Ot her complications of multicystic dysplastic kidney disease such as hyp ertension, infection, abdominal pain, hematuria and persistent dysplas tic renal tissue despite ultrasonographic resolution of multicystic dy splastic kidney disease are additional risk factors to be considered. A recommendation for nephrectomy in all cases of multicystic dysplasti c kidney disease cannot be based only on these 2 cases. Several other factors must be weighed before making that decision.