In addition, it has been recognized that 3–18% of ACC cases are associated with somatic or germline mutations of DNA mismatch repair genes, such as ML1, MSH2, MSH6, and PMS2 mutations (e.g., ACC-associated Lynch syndrome), and these mutations lead to microsatellite instability [40,41,42]. Here, MSH6 is linked to adrenal cortex carcinoma.