title: Hereditary pancreatic cancer: molecular bases and their application in diagnosis and clinical management: a guideline of the TTD group.
abstract: Pancreatic carcinoma (PC) represents the fourth leading cause of cancer death in Spain with a death rate of 2,400 males and 2,000 females per year. Poor outcome related to its silent nature and the lack of reliable secondary prevention measures translate into advanced-stage diagnosis, 75 % of deaths within the first year of diagnosis and 5-year survival rate of <5 %. Family history was first recognized as a risk factor for PC. Further population-based and case-control studies subsequently found that 7.8 % of patients with PC have a family history of the same tumor and individuals with a first-degree relative with PC have a 3.2-fold increased risk of developing PC. Overall, it is estimated that up to 10 % of PC have a familial component. However, known genetic syndromes account for <20 % of the observed familial aggregation of PC. We review the most important aspects in epidemiology, molecular biology and clinical management of familial PC.
authors.affiliation: Medical Oncology, Clinical Hospital San Carlos, Madrid, Spain. perezsegura09@gmail.com
authors.forename: P Pérez
authors.initials: PP
authors.lastname: Segura
authors.forename: C Guillén
authors.initials: CG
authors.lastname: Ponce
authors.forename: T
authors.initials: T
authors.lastname: Ramón Y Cajal
authors.forename: R Serrano
authors.initials: RS
authors.lastname: Blanch
authors.forename: E
authors.initials: E
authors.lastname: Aranda
mesh_terms: D002277:Carcinoma / Q000175:diagnosis* / Q000235:genetics* / Q000473:pathology; D005260:Female; D020022:Genetic Predisposition to Disease; D006801:Humans; D008297:Male; D008403:Mass Screening; D010179:Pancreas / Q000473:pathology*; D010190:Pancreatic Neoplasms / Q000175:diagnosis* / Q000235:genetics* / Q000473:pathology; D017410:Practice Guidelines as Topic; D011230:Precancerous Conditions / Q000175:diagnosis / Q000235:genetics / Q000473:pathology; D012042:Registries; D012307:Risk Factors; D015996:Survival Rate