title: Familial pancreatic cancer.
abstract: Familial pancreatic cancer (FPC) includes those kindreds that contain at least two first-degree relatives with pancreatic ductal adenocarcinoma. At least 12 known hereditary syndromes or genes are associated with increased risk of developing pancreatic cancer, the foremost being BRCA2 and CDKN2A. Research into the identification of mutations in known cancer predisposition genes and through next-generation sequencing has revealed extensive heterogeneity. The development of genetic panel testing has enabled genetic risk assessment and predisposition testing to be routinely offered. Precision oncology has opened the possibility of "incidental" germline mutations that may have implications for family members. However, in both cases, evidence-based recommendations for managing patients and at-risk family members in light of genetic status remain emergent, with current practice based on expert opinion.
authors.affiliation: Department of Health Sciences Research, Mayo Clinic Cancer Center, Rochester, MN. Electronic address: Petersen.gloria@mayo.edu.
authors.forename: Gloria M
authors.initials: GM
authors.lastname: Petersen
mesh_terms: D019941:Cyclin-Dependent Kinase Inhibitor p16; D050763:Cyclin-Dependent Kinase Inhibitor p18 / Q000235:genetics; D052218:Fanconi Anemia Complementation Group C Protein / Q000235:genetics; D000076164:Fanconi Anemia Complementation Group N Protein; D024522:Genes, BRCA2; D020022:Genetic Predisposition to Disease; D006801:Humans; D009687:Nuclear Proteins / Q000235:genetics; D010190:Pancreatic Neoplasms / Q000209:etiology / Q000235:genetics*; D025521:Tumor Suppressor Proteins / Q000235:genetics
chemical_list: C000614131:CDKN2A protein, human; D019941:Cyclin-Dependent Kinase Inhibitor p16; D050763:Cyclin-Dependent Kinase Inhibitor p18; C497651:FANCC protein, human; D052218:Fanconi Anemia Complementation Group C Protein; D000076164:Fanconi Anemia Complementation Group N Protein; D009687:Nuclear Proteins; C512790:PALB2 protein, human; D025521:Tumor Suppressor Proteins
keywords: BRCA2; CDKN2A; Genetic testing; Pancreatic cancer