Molecular basis of Wilms' tumor.

Abstract

Wilms’ tumor, the most common primary malignant renal tumor of childhood, has been a model for the multimodal treatment of pediatric malignant solid tumors. The generally favorable outcome for Wilms’ tumor patients can be partly attributed to medical advances, including refinement in surgical technique, the sensitivity of Wilms’ tumor to irradiation, and the availability of several active chemotherapeutic agents. The successful application of these treatment modalities to Wilms’ tumor has been largely facilitated by the National Wilms Tumor Study Group (NWTSG) which has studied the majority of Wilms’ tumor patients in North America on randomized clinical trials since 1969. In conjunction with these therapeutic trials, data on numerous clinical features and from central pathological review have been collected and analyzed. Such systematic study of a large proportion of the Wilms’ tumor population has contributed not only to the clinical management of affected children but also to our understanding of the etiology and pathogenesis of Wilms’ tumor.