Purpose of review: Wilms' tumor accounts for nearly 6% of all pediatric cancers and more than 95% of all kidney tumors in children. Fortunately, survival for patients with Wilms' tumor is generally excellent. This review will outline the results of prior clinical trials that have led to this excellent outcome and how information gleaned from these trials has led to the development of the current series of clinical trials for the management of children with Wilms' tumor.
Recent findings: Tumor stage and histologic subtype have long been recognized as important prognostic factors in Wilms' tumor. More recent evidence suggests that, in certain instances, patient age, tumor size, response to therapy, and genetic abnormalities, specifically the loss of genetic material on chromosomes 1p and 16q, provide additional prognostic information. These factors have, therefore, been incorporated into a new risk stratification system that is currently being used to assign patients with Wilms' tumor to specific protocol-based therapies.
Summary: Survival for patients with Wilms' tumor when considered as a whole, once less than 30%, is currently greater than 90%, with this dramatic improvement being due, in part, to the systematic manner in which the approach to therapy has evolved. Further refinement in therapy is being undertaken, with the current trials aiming to maintain the excellent survival for children being treated for Wilms' tumor, while minimizing therapy-related toxicity.