In the present study, we had the opportunity to investigate ATRX in 2 patients with corticotroph tumors, 1 nonaggressive macroadenoma and 1 carcinoma, and Lynch syndrome, a cancer predisposing syndrome with mutations in genes involved in DNA mismatch repair (MLH1, MSH2, MSH6, PMS2, EPCAM). Here, PMS2 is linked to carcinoma.