MLH1 and adrenal cortex carcinoma: Aberrations in DNA mismatch repair (MMR) genes, leading to microsatellite instability, are implicated in a subset of ACC cases.32 Lynch syndrome, caused by germline variants in MMR genes (MLH1, MSH2, MSH6, PMS2, and EPCAM), is associated with ACC development.33 MMR-deficient tumors possess a high somatic mutation burden resulting in increased tumor-specific T-cell responses making them potential targets for immunotherapy programmed death-ligand 1 (PD-L1).34