WILMS-TUMOR PATIENTS WITH PULMONARY METASTASES

We have evaluated the clinicopathologic characteristics, survival probabilities, patterns of treatment failure, and late toxicities in a group of 51 Wilms' tumor patients with pulmonary metastases evaluated at our center between 1968 and 1988 and treated intensively with surgery, multiagent chemotherapy, and radiotherapy. Twenty-one patients had pulmonary metasases at diagnosis (Stage IV-p) and 30 had pulmonary relapse after initial treatment for nonmetastatic disease. With a median follow-up time of 83 months, actuarial 5- and 10-year survival probability for the group as a whole is 59%. For the subset of patients with favorable histology Stage IV-p disease, 10-year survival probability is 77%, whereas for the subset with relapsed anaplastic or sarcomatous disease, 10-year survival probability is 22%. The lung was the predominant site of failure even in patients who had received relatively high doses of pulmonary radiotherapy. Late effects seen in patients with over 10 years of follow-up included musculoskeletal and soft tissue growth abnormalities (93% incidence) as well as breast hypoplasia and endocrinologic abnormalities. Second malignant neoplasms of the breast, thyroid, and pancreas were observed at post-treatment intervals of 17, 3.5 and 12 years, respectively.