PVs in some genes involved in CPS previously reported to be associated with PDAC including STK11, MSH2 MSH6, MLH1, APC and PMS2 in hereditary colorectal cancer, unexpectedly, had relatively low prevalences (<1%) with only APC reaching a cumulative frequency of >1% for any group of patients (FPC). Here, MSH2 is linked to familial colorectal cancer.