Interestingly, the Wnt/β-Catenin pathway is also one of the most frequently altered pathways in adrenocortical carcinomas (ACC), mainly harboring alterations in CTNNB1, APC Regulator of WNT Signaling Pathway (APC), and Zinc and Ring Finger 3 (ZNRF3), suggesting that alterations in this pathway are, in part, shared events in both benign and malignant adrenocortical tumors [79,80]. This evidence concerns the gene ZNRF3 and adrenal cortex carcinoma.