Although the numbers are small, the most common germline mutations in pancreatic cancer related to these syndromes are breast cancer 2 (BRCA2), breast cancer 1 (BRCA1), partner and localiser of BRCA2 (PALB2), cyclin-dependent kinase inhibitor 2A (CDKN2A), ataxia telangiectasia mutated (ATM), tumour protein p53 (TP53) and mismatch repair genes mutL homolog 1 (MLH1), mutS homolog 2 (MSH2) and mutS homolog 6 (MSH6). The gene discussed is MSH2; the disease is familial pancreatic carcinoma.