The mutation rates of the nine target genes in ACC tissue samples from high to low were 54.1% (20/37) of MEN1, 54.1% (20/37) of ZNRF3, 51.4% (19/37) of ARMC5, 51.4% (19/37) of TP53, 35.1% (13/37) of CTNNB1, 32.4% (12/37) of APC, 29.7% (11/37) of RB1, 24.3% (9/37) of PRKAR1A, and 24.3% (9/37) of RPL22. In the 227 mutation sites, the incidences of mutation types were: 74.4% (169/227) of nonsynonymous SNV, 8.4% (19/227) of stopgain mutation, 8.4% (19/227) of frameshift deletions, 8.4% (19/227) of splicing mutation, and 0.4% (1/227) of stoploss mutation. The gene discussed is ZNRF3; the disease is adrenal cortex carcinoma.