The inheritance pattern of FPC is mostly autosomal dominant and demonstrates a heterogenous phenotype (Slater et al., 2010) The genetic mutations responsible for the majority of clustering in families with PDAC have yet to be identified, although germline mutations in high-penetrance genes such as BRCA2 and PALB2 have been established along with mutations in p16/CDKN2A, STK11/LKB1, PRSS1, BRCA1, mismatch repair genes (hMLH1, hMSH2, hMSH6), VHL, and ATM (Table 1). Here, STK11 is linked to Familial adenomatous polyposis.