MLH1 and Lynch syndrome: In addition, germline PVs confer a high risk of PDAC in patients carrying PVs in other genes including CDKN2A (RR 13–39), the Lynch syndrome genes MLH1, MSH2, and MSH6 (RR of 8.6–11), and ATM, in which a RR of 3.92 (95% CI, 0.44–14.2) has been reported [5].