ANIRIDIA AND WILMS-TUMOR - 2 CASES OF FET AL RHABDOMYOMATOUS NEPHROBLASTOMA

Background. - Wilms tumor is associated in 7 to 10% of patients with c ongenital abnormalities Among those, aniridia is the most constant fea ture of the WAGR syndrome that includes, in one third of cases, Wilms tumor We report two cases of aniridia associated with fetal rhabdomyom atous nephroblastoma. Case reports. - Case 1. A one-year old girl with congenital aniridia was admitted for macroscopic hematuria. Abnormal ultrasonography and tomodensitometry revealed a large bilateral, kidne y tumor The patient was given actinomycin and vincristine, without eff icacy. Bilateral tumorectomy was performed 6 months later and the hist ological study showed a fetal rhabdomyomatous nephroblastoma. This pat ient is in remission at the age of 5. Case 2. A boy, also with congeni tal aniridia, presented with macroscopic hematuria at the age of 2 yea rs revealing a nephroblastoma located on his right kidney. Preoperativ e chemotherapy remained uneffective and the nephrectomy performed 1 mo nth later permitted the diagnosis of fetal rhabdomyomatous nephrolasto ma. The patient is well 4 years later Conclusion. - Both cases of feta l rhabdomyomatous nephroblastoma, a histological variant of Wilms tumo r, seem to be the first reported in the WAGR syndrome.