PURPOSE: There have been a number of advances that have increased our understanding of the biology of Wilms tumor during the last decade. This information is now being incorporated into current pediatric oncology protocols. We present a summary of these advances and outline the current treatment of Wilms tumor. MATERIALS AND METHODS: The medical literature was reviewed with an emphasis on the molecular biology of Wilms tumor. RESULTS: The development of Wilms tumor involves several genes, including WT1, the Wilms tumor suppressor gene at 11p13. In addition, certain chromosomal regions (16q and 1p) might be used as prognostic factors for determining the intensity of therapy. CONCLUSIONS: Future protocols conducted by pediatric oncology groups will incorporate biological studies. The goal is to identify patients at low risk for relapse which will allow a reduction in treatment intensity and subsequent toxicity. Children at an increased risk for relapse can be selected for more intensive treatment.
PURPOSE: There have been a number of advances that have increased our understanding of the biology of Wilms tumor during the last decade. This information is now being incorporated into current pediatric oncology protocols. We present a summary of these advances and outline the current treatment of Wilms tumor. MATERIALS AND METHODS: The medical literature was reviewed with an emphasis on the molecular biology of Wilms tumor. RESULTS: The development of Wilms tumor involves several genes, including WT1, the Wilms tumor suppressor gene at 11p13. In addition, certain chromosomal regions (16q and 1p) might be used as prognostic factors for determining the intensity of therapy. CONCLUSIONS: Future protocols conducted by pediatric oncology groups will incorporate biological studies. The goal is to identify patients at low risk for relapse which will allow a reduction in treatment intensity and subsequent toxicity. Children at an increased risk for relapse can be selected for more intensive treatment.