title: Familial pancreatic cancer: a review.
abstract: The cause of pancreatic cancer remains elusive. The most consistently identified epidemiological risk factor is cigarette smoking. Genetic factors are known to play a significant role in perhaps 5% of the total pancreatic cancer burden. Recent discoveries in molecular biology, particularly germline mutations in inherited conditions which feature pancreatic cancer as an integral part of the tumor spectrum such as in adenomatosis polyposis and hereditary nonpolyposis colorectal cancer, provide powerful incentive to search for other "cancer genes" in this heterogeneous disease. Early detection of this dreadful disease is crucial because its mortality rate approximates its incidence; the ability to identify high-risk patients on the basis of genetic analysis would significantly enhance the potential for early diagnosis. This review addresses the genetic epidemiology of pancreatic cancer and updates our views on screening, surgery, chemotherapy, and genetic counseling, all of which must be used to gain value from genetic predictability of risk status.
authors.affiliation: Creighton University School of Medicine, Department of Preventive Medicine, Omaha, NE 68178, USA.
authors.forename: H T
authors.initials: HT
authors.lastname: Lynch
authors.forename: T
authors.initials: T
authors.lastname: Smyrk
authors.forename: S E
authors.initials: SE
authors.lastname: Kern
authors.forename: R H
authors.initials: RH
authors.lastname: Hruban
authors.forename: C J
authors.initials: CJ
authors.lastname: Lightdale
authors.forename: S J
authors.initials: SJ
authors.lastname: Lemon
authors.forename: J F
authors.initials: JF
authors.lastname: Lynch
authors.forename: L R
authors.initials: LR
authors.lastname: Fusaro
authors.forename: R M
authors.initials: RM
authors.lastname: Fusaro
authors.forename: P
authors.initials: P
authors.lastname: Ghadirian
mesh_terms: D011125:Adenomatous Polyposis Coli / Q000235:genetics; D003110:Colonic Neoplasms / Q000235:genetics; D005817:Genetic Counseling; D006801:Humans; D015994:Incidence; D008967:Molecular Biology; D009154:Mutation / Q000235:genetics; D009857:Oncogenes / Q000235:genetics; D010190:Pancreatic Neoplasms / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics* / Q000517:prevention & control / Q000601:surgery; D010375:Pedigree; D012004:Rectal Neoplasms / Q000235:genetics; D012307:Risk Factors; D012907:Smoking / Q000009:adverse effects; D015996:Survival Rate