Approximately 3–5 % of all patients with PDAC have a genetic predisposition that results in an increased risk of developing the tumor [2] and a substantial proportion of these patients carry an underlying gene defect in CDKN2A/p16-Leiden (Familial Atypical Multiple Mole Melanoma, FAMMM syndrome), STK11 (Peutz-Jeghers syndrome), the BRCA1/2 genes (Hereditary breast cancer) or one of the MMR genes (Lynch syndrome) [3]. Here, CDKN2A is linked to Peutz-Jeghers syndrome.