MSH6 and familial pancreatic carcinoma: Familial pancreatic cancer confers the strongest predictive risk and should be considered if two first-degree relatives have PDAC (RR of 6.4) [10]; Breast Cancer gene 2 (BRCA 2) mutations are the most common inherited genetic risk factor [11], while Lynch syndrome (loss of MLH1, MSH2 and MSH6 DNA repair mechanisms constituting Mismatch repair deficiency (MMRd)) confers an 8.6-fold increased risk of developing pancreatic cancer by age 70 [12].