Epigenetic Regulation in Wilms Tumor

Wilms Tumor, or nephroblastoma, is the most common pediatric renal malignancy, with a complex genetic and epigenetic landscape. While mutations in genes such as WT1, CTNNB1, and WTX have been well-characterized, accumulating evidence suggests that epigenetic dysregulation plays a pivotal role in WT pathogenesis. This review synthesizes the various epigenetic mechanisms implicated in WT, including DNA methylation, histone modifications, chromatin remodeling, and non-coding RNA-mediated regulation. We discuss how epigenetic mechanisms contribute to tumor initiation, progression, and heterogeneity, alongside their implications for diagnosis and targeted therapy. We also highlight recent advances in epigenomic profiling, discuss the interplay between epigenetics and developmental gene expression programs, and evaluate potential therapeutic strategies targeting epigenetic regulators.