Abstract
Wilms' tumor, or nephroblastoma, arises from the progenitor population that gives rise to normal nephrons in the developing kidney. As such, it is the prototypical case of a developmental process that has become dysregulated, leading to malignancy. Consideration of the pathogenesis of Wilms' tumor therefore instructs about the relationship between development and cancer and how oncogenic processes often co-opt regulatory pathways that drive growth and differentiation. The chapter will review current knowledge of the molecular basis for Wilms' tumor and discuss how these tumors may arise from aberrant regulation of the same processes that regulate normal kidney development.
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