ZNRF3 and adrenal cortex carcinoma: Recent next-generation sequencing studies have revealed recurrent alterations present in patients with ACC.3–5 These studies have identified mutations in the WNT signalling pathway to be one of the most frequent alterations, with CTNNB1-activating mutations present in up to 16% and inactivating changes in ZNRF3, a pathway repressor, in up to 21%.3 Other pathways that are altered in ACC patients include epigenetic regulation and p53/RB1 and PKA signalling.5