Genetics and Molecular Biology

Abstract

A conundrum of genetic and epigenetic pathways has been implicated in the etiopathogenesis of Wilms’ tumor (WT). The WT1, WT2, and WTX genes are commonly associated with syndromic WT. The current concepts in the molecular biology of WT are complex and evolving. This chapter would discuss the genetic events in the normal and pathological nephrogenesis, Knudson double-hit hypothesis, the various other genetic pathways, and the known genetic mutations associated with WT. The learning objectives of this chapter would include to know about the genes and genetic pathways especially WT1 and WT2 genes, present status of Knudson’s two-hit hypothesis, current conceptual molecular basis of biological events of WT, role of genetics and molecular markers in prognostication, therapeutic manipulation of genetic steps, and the biological basis of future treatment of WT. The syndromes associated with WT are discussed elsewhere.