It is not clear whether pancreatic cancer susceptibility genes such as BRCA1, BRCA2, CDKN2A, mismatch repair genes (MLH1, MSH2, MSH6, PMS2, and EPCAM), ATM, PALB2, STK11, and TP53 are associated with IPMNs. Here, MSH6 is linked to familial pancreatic carcinoma.