title: Pancreatic cancer risk in Peutz-Jeghers syndrome patients: a large cohort study and implications for surveillance.
abstract: BACKGROUND
  Although Peutz-Jeghers syndrome (PJS) is known to be associated with pancreatic cancer (PC), estimates of this risk differ widely. This hampers counselling of patients and implementation of surveillance strategies. We therefore aimed to determine the PC risk in a large cohort of Dutch PJS patients.
  
  
  METHODS
  PJS was defined by diagnostic criteria recommended by the WHO, a proven LKB1 mutation, or both. All patients with a presumptive diagnosis of pancreatic, ampullary or distal bile duct cancer were identified. Cases were reviewed clinically, radiologically and immunohistochemically. Cumulative PC risks were calculated by Kaplan-Meier analysis and relative risks by Poisson regression analysis.
  
  
  RESULTS
  We included 144 PJS patients (49% male) from 61 families (5640 person years follow-up). Seven (5%) patients developed PC at a median age of 54 years. Four patients (3%) were diagnosed with distal bile duct (n=2) or ampullary cancer (n=2) at a median age of 55 years. The cumulative risk for PC was 26% (95% CI 4% to 47%) at age 70 years and relative risk was 76 (95% CI 36 to 160; p<0.001). The cumulative risk for pancreatico-biliary cancer was 32% (95% CI 11% to 52%) at age 70 years, with a relative risk of 96 (95% CI 53 to 174; p<0.001).
  
  
  CONCLUSIONS
  PJS patients have a highly increased risk for pancreatico-biliary cancer. Therefore, patients are eligible for surveillance within well defined research programmes to establish the benefit of such surveillance.
authors.affiliation: Department of Gastroenterology & Hepatology, Erasmus MC University Medical Centre, Rotterdam, The Netherlands. s.korsse@erasmusmc.nl
authors.forename: Susanne E
authors.initials: SE
authors.lastname: Korsse
authors.forename: Femme
authors.initials: F
authors.lastname: Harinck
authors.forename: Margot G F
authors.initials: MG
authors.lastname: van Lier
authors.forename: Katharina
authors.initials: K
authors.lastname: Biermann
authors.forename: G Johan A
authors.initials: GJ
authors.lastname: Offerhaus
authors.forename: Nanda
authors.initials: N
authors.lastname: Krak
authors.forename: Caspar W N
authors.initials: CW
authors.lastname: Looman
authors.forename: Wendy
authors.initials: W
authors.lastname: van Veelen
authors.forename: Ernst J
authors.initials: EJ
authors.lastname: Kuipers
authors.forename: Anja
authors.initials: A
authors.lastname: Wagner
authors.forename: Evelien
authors.initials: E
authors.lastname: Dekker
authors.forename: Elisabeth M H
authors.initials: EM
authors.lastname: Mathus-Vliegen
authors.forename: Paul
authors.initials: P
authors.lastname: Fockens
authors.forename: Monique E
authors.initials: ME
authors.lastname: van Leerdam
authors.forename: Marco J
authors.initials: MJ
authors.lastname: Bruno
mesh_terms: D000328:Adult; D015331:Cohort Studies; D016208:Databases, Factual; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009426:Netherlands / Q000453:epidemiology; D010190:Pancreatic Neoplasms / Q000150:complications* / Q000453:epidemiology*; D010580:Peutz-Jeghers Syndrome / Q000150:complications* / Q000453:epidemiology; D011159:Population Surveillance; D012306:Risk; D055815:Young Adult