Wilms' tumor: diagnosis and treatment in the 1990s

The present therapy of Wilms' tumor is a classic example of the modern multidisciplinary treatment of childhood malignancies, and the organization of the National Wilms' Tumor Studies (NWTS) has served as a prototype for other collaborative studies of childhood tumors. More than 80% of all children with Wilms' tumor can now be expected to have a long term relapse free survival. The NWTS is in large part responsible for this excellent outlook and continues its emphasis on decreasing the morbidity of therapy without sacrificing relapse free survival by tailoring therapy based on histology and accurate surgical staging. The pathological classification of Wilms' and its effect on therapy and prognosis is now being used as a guide in the study of other childhood malignancies. The surgeon continues to play a key role in the multimodality management of the child with a Wilms' tumor since subsequent therapy will be determined by the extent of the tumor at the time of operation. The identification of histological type, the presence or absence of residual tumor within the abdomen following resection, and the status of lymph node metastases are of utmost importance. Advances in cytogenetics and refinement of histopathologic staging will allow the pediatric surgical oncologist, pediatric oncologist, and pediatric radiation therapist to continue to modify therapy for specific patient groups in the future.