UTERINE ANOMALIES IN WILMS-TUMOR SURVIVORS

BACKGROUND. The association between Wilms' tumor (WT) and genitourinar y (GU) anomalies has long been appreciated; however, associated GU ano malies have been described almost exclusively in males. METHODS. To in vestigate whether females with WT also have an increased prevalence of GU anomalies, the authors evaluated the uterine anatomy of 24 WT surv ivors using magnetic resonance imaging and ultrasonography. RESULTS. T wo of 24 female survivors (8%) had anomalies. One had a septate uterus , and a limited molecular analysis of her constitutional DNA revealed no mutations or deletions of the tumor suppressor gene WT1. Another su rvivor with the WAGR syndrome (WT, aniridia, GU anomalies, and retarda tion), with the characteristic 11p13 deletion including WT1, had a ute rine anomaly (hypoplastic vs. unicornuate). CONCLUSIONS. Because uteri ne malformations are rare in the general population, this finding sugg ests an association between WT and uterine malformations and also may partially explain the fertility deficit previously demonstrated in adu lt female WT survivors. Pelvic ultrasonography in adult female WT surv ivors can alert survivors and clinicians to possible fertility problem s that may lead to problem pregnancies and adverse pregnancy outcomes. (C) 1996 American Cancer Society.