The results showed that the mutation rates of 6 genes detected in ACC tissues were significantly higher than those in ACA and normal adrenal gland tissues, which were ZNRF3, TP53, ARMC5, APC, RB1, and PRKAR1A. We regarded the 6 genes as high-risk genes associated with ACC and proposed a novel concept named sum of high-risk mutation genes (SHGM), defined as the count of mutations of above 6 high-risk genes detected in ACC tissues. Here, PRKAR1A is linked to adrenal cortex carcinoma.