ABSTRACT

Nephroblastoma, or Wilms’ tumor, is an embryonal tumor that develops from remnants of immature kidney. It is the most common renal tumor of childhood. Current survival of children with Wilms’ tumor is excellent, and emphasis is now on reducing the morbidity of treatment for low-risk patients. Unfortunately, there is still a subset of high-risk patients with poor survival for whom new treatment strategies are needed. This chapter reviews the epidemiology, pathology, and recent advances in the understanding of the biology of Wilms’ tumor. The current management of children with all stages of nephroblastoma is discussed in detail.