Wilms tumour: diagnosis and treatment.

Wilms tumour is the most common intra-abdominal solid tumour of childhood. Treatment includes surgical resection and chemotherapy for virtually all affected children and additional radiotherapy for those with advanced disease or adverse prognostic features. This approach leads to cure rates exceeding 80%. During the last decade there have been a number of advances which have increased our understanding of the biology of Wilms tumour. The development of Wilms tumour, for example, involves several genes, including WT1, the Wilms tumour suppressor gene at 11p13, and WT2, the putative Wilms tumour suppressor gene at 11p15. In addition, certain chromosomal regions, most notably 16q and 1p, might predict outcome and hence serve as a prognostic factor, useful for determining the intensity of therapy. This novel information is now being incorporated into current therapeutic protocols. We reviewed the medical literature and present a summary of the advances made, outlining the current treatment of Wilms tumour. Future protocols will continue incorporating biological markers. 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.