title: Familial pancreatic cancer.
abstract: The dismal prognosis of ductal pancreatic adenocarcinoma is mainly attributable to advanced tumor stages at the time of diagnosis. Meanwhile, familial pancreatic cancer is an established hereditary tumor entity that is responsible for approximately 3% of pancreatic cancer (PC) cases. Therefore, analysis of the family history may help to identify individuals at increased risk of developing PC. These include members of families with a history of PC as well as those of families with distinct hereditary cancer syndromes such as Peutz-Jeghers syndrome, hereditary pancreatitis, familial atypical multiple mole melanoma syndrome, hereditary breast and ovarian cancer syndrome and hereditary non-polyposis colorectal cancer. In future, the identification of germline mutations in genes predisposing to PC, together with the analysis of exogenous risk factors, could be used for a more precise risk assessment for the development of PC. This may allow the application of invasive screening methods for the identification of early PC or, even better, its precursor lesions in high-risk individuals, providing the option of timely curative pancreatectomy.
authors.affiliation: Institute of Clinical Genetics, Marburg, Germany.
authors.forename: Harald
authors.initials: H
authors.lastname: Rieder
authors.forename: Detlef K
authors.initials: DK
authors.lastname: Bartsch
mesh_terms: D000328:Adult; D017677:Age Distribution; D000368:Aged; D001943:Breast Neoplasms / Q000453:epidemiology / Q000235:genetics; D021441:Carcinoma, Pancreatic Ductal / Q000453:epidemiology* / Q000235:genetics* / Q000628:therapy; D003123:Colorectal Neoplasms, Hereditary Nonpolyposis / Q000453:epidemiology / Q000235:genetics; D005260:Female; D020022:Genetic Predisposition to Disease / Q000453:epidemiology*; D005858:Germany / Q000453:epidemiology; D006801:Humans; D015994:Incidence; D008297:Male; D008875:Middle Aged; D010051:Ovarian Neoplasms / Q000453:epidemiology / Q000235:genetics; D010190:Pancreatic Neoplasms / Q000453:epidemiology* / Q000235:genetics* / Q000628:therapy; D010375:Pedigree; D011379:Prognosis; D018570:Risk Assessment; D017678:Sex Distribution; D016019:Survival Analysis