Purpose: There have been a number of advances that have increased our
understanding of the biology of Wilms tumor during the last decade. Th
is information is now being incorporated into current pediatric oncolo
gy protocols. We present a summary of these advances and outline the c
urrent treatment of Wilms tumor.Materials and Methods: The medical Lit
erature was reviewed with an emphasis on the molecular biology of Wilm
s tumor. Results: The development of Wilms tumor involves several gene
s, including WT1, the Wilms tumor suppressor gene at 11p13. In additio
n, certain chromosomal regions (16q and Ip) might be used as prognosti
c factors for determining the intensity of therapy. Conclusions: Futur
e protocols conducted by pediatric oncology groups will incorporate bi
ological studies. The goal is to identify patients at low risk for rel
apse which will allow a reduction in treatment intensity and subsequen
t toxicity. Children at an increased risk for relapse can be selected
for more intensive treatment.