Furthermore, we identified upregulation of several WNT pathway effectors in SIX1-Q177R tumors compared to other chemotherapy-naïve blastemal tumors, including positive regulators of non-canonical WNT signaling and negative regulators of canonical WNT/β-catenin-mediated signaling, implicating disruption of this signaling pathway in the maintenance and therapeutic evasion of SIX1-Q177R Wilms tumors. Here, SIX1 is linked to Nephroblastoma.