In stage IV ACC tumors, there was a higher rate of TP53 mutations (47% versus 13%), and of CTNNB1 gain-of-function mutations (24% versus 11%), along with a higher rate of LGR5 and MDM2 CNV amplifications (24% versus 1%, and 14% versus 3%, respectively, compared with localized tumors. This evidence concerns the gene LGR5 and adrenal cortex carcinoma.