Studies have shown somatic mutations of the cationic trypsinogen gene (PRSS1) in patients with (hereditary) chronic pancreatitis and pancreatic cancer.31 Although the risk for pancreatic cancer may be lower than previously thought,31 it may serve as a model for the role of pathological intracellular activation of trypsin that also play a role in pancreatic cancerogenesis.32–34 However, direct mutation in the gene is unlikely to be the explanation in the vast majority of patients with PDAC, although being a likely key component in the risk of families with hereditary pancreatitis.31,35. This evidence concerns the gene PRSS1 and familial pancreatic carcinoma.