Introduction: Extrarenal Wilms' tumors are rare, with only 55 well-documented cases in the literature and only 4 confined to the uterus. A fifth case along with review of the current literature is presented.
Case: A 13-year-old girl presented with a 2-month history of irregular vaginal spotting. A 6.5 x 4.5 x 2.5-cm polypoid mass meeting criteria for an extrarenal Wilms tumor was removed from the superior aspect of the cervix, but recurred 8 months later on the posterior lip. The patient underwent a Schauta procedure, lymph node dissection, and bilateral oophoropexy. The surgery was followed by typical renal Wilms' tumor chemotherapy. The patient is without evidence of disease 5 years after surgery.
Discussion: This case uniquely suggests that limited excision of the original tumor has inherent risks for relapse. Oophoropexy appears to be reasonable when fertility preservation is desired and reconfirms that treatment mimicking renal nephroblastomas offers good long-term survival.
Copyright 2000 Academic Press.