Individuals which are mutation carriers of the following genes have an increased lifetime risk of pancreas carcinoma (variable from 1 to 30%), beside other tumor entities within other syndromes: Adenomatous Polyposis Coli (APC), Ataxia-Telangiesctasia Mutated (ATM), BReast Cancer (BRCA) 1 and 2, Cyclin Dependent Kinase Inhibitor 2A (CDKN2A), MutL Homolog 1 (MLH1), MutS Homolog 2 (MSH2), MutS Homolog 6 (MSH6), PMS1 Homolog 2 (PMS2), Epithelial Cell Adhesion Molecule (EPCAM), Partner And Localizer of BRCA2 (PALB2), Serine/Threonine Kinase 11 (STK11), Tumor Protein p53 (TP53) [8]. Here, MSH6 is linked to exocrine pancreatic carcinoma.