BILATERAL WILMS-TUMOR - REVIEW OF OUTCOME, ASSOCIATED ABNORMALITIES, AND LATE EFFECTS IN 36 PEDIATRIC-PATIENTS TREATED AT A SINGLE INSTITUTION

Background. Patients with bilateral Wilms tumor have an increased inci dence of associated abnormalities and nephrogenic rests and require in dividualized multimodality therapy for cure. The authors reported the associated abnormalities, outcome, complications, and late effects of treatment in a group of children with bilateral Wilms tumor treated at St. Jude Children's Research Hospital, Memphis, Tennessee, over a 28- year period. Methods. The records of 36 consecutive pediatric patients diagnosed with bilateral Wilms tumor between 1962-1990 were analyzed. Biopsy material was also reviewed, with particular emphasis on charac terization of nephrogenic rests and histology. Results. Twenty-nine pa tients had synchronous tumors and 7 had metachronous lesions. Associat ed physical abnormalities were present in 12 patients and involved the genitourinary, cardiovascular, integumentary, and musculoskeletal sys tems. The overall survival for patients with metachronous tumors (71%) was similar to that for those with synchronous tumors (70%). There wa s no effect of age or the presence of nephroblastomatosis. Two patient s with synchronous tumors initially treated with nephrectomy eventuall y required bilateral nephrectomies for contralateral recurrence after chemotherapy and radiation therapy. Nephrogenic rests were present in 22 of 30 evaluable patients. Two of three patients with metachronous t umors had intralobar nephrogenic rests. Bilateral renal salvage proced ures were demonstrated to be technically feasible and effective in con trolling disease without compromising renal function or survival. Late effects included scoliosis in three patients treated before 1970, car diomyopathy in one patient who received 300 mg/m2 doxorubicin and 12 G y pulmonary irradiation, and benign tumors in two patients, one of who m also had a bowel obstruction. Serum creatinine, urea nitrogen, and b lood pressure were normal in 23 of 26 survivors. Conclusions. The auth ors' experience supports a favorable outcome with minimal late effects for patients with bilateral Wilms tumor who receive individualized th erapy at pediatric oncology centers.