title: Progression and Management of Duodenal Neoplasia in Familial Adenomatous Polyposis: A Cohort Study.
abstract: OBJECTIVE
  To describe the natural history and outcomes of surveillance of duodenal neoplasia in familial adenomatous polyposis (FAP).
  
  
  BACKGROUND
  Duodenal cancer is the most common cause of death in FAP.
  
  
  METHODS
  Cohort study of patients prospectively enrolled in an upper endoscopic surveillance protocol from 1982 to 2012. The duodenum was assessed by side-viewing endoscopy and classified as stage 1 to 5 disease. Endoscopic and/or operative interventions were performed according to stage.
  
  
  RESULTS
  There were 218 patients in the protocol (98 with advanced stage). They had a median of 9 endoscopies (range: 2-25) over a median of 11 years (range: 1-26). Median age at diagnosis of stage 3 disease (adenoma: 2.1-10 mm) was 41 years and stage 4 disease (adenoma >10 mm) was 45 years. Median time from first esophagogastroduodenoscopy to stage 4 disease was 22.4 years. The risk of stage 4 disease was 34.3% [95% confidence interval (CI) 23.8-43.4] at 15 years. In multivariate analysis, sex, type of colorectal surgery, years since colorectal surgery, and stage were significantly associated with risk of progression to stage 4 disease. Five of 218 (2.3%) patients developed duodenal cancer at median age of 58 years (range: 51-65). The risk of developing duodenal cancer was 2.1% (95% CI: 0-5.2) at 15 years.
  
  
  CONCLUSIONS
  Patients with advanced duodenal polyposis progress in the severity of disease (size and degree of dysplasia); however, the rate of progression to carcinoma is slow. Aggressive endoscopic and surgical intervention, especially in the presence of large polyps and high-grade dysplasia, appears to be effective in preventing cancer deaths in FAP.
authors.affiliation: *Department of Surgery, McMaster University, Hamilton, Ontario, Canada †Samuel Lunenfeld Research Institute, Mount Sinai Hospital, University of Toronto, Toronto, Ontario, Canada ‡Familial Gastrointestinal Cancer Registry, Mount Sinai Hospital, Toronto, Ontario, Canada §Toronto General Hospital, University of Toronto, Toronto, Ontario, Canada ¶Department of Pathology, Mount Sinai Hospital, University of Toronto, Toronto, Ontario, Canada; and ‖Division of Gastroenterology, St Michael's Hospital, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Pablo E
authors.initials: PE
authors.lastname: Serrano
authors.forename: Robert C
authors.initials: RC
authors.lastname: Grant
authors.forename: Terri C
authors.initials: TC
authors.lastname: Berk
authors.forename: Dowan
authors.initials: D
authors.lastname: Kim
authors.forename: Hassan
authors.initials: H
authors.lastname: Al-Ali
authors.forename: Zane
authors.initials: Z
authors.lastname: Cohen
authors.forename: Aaron
authors.initials: A
authors.lastname: Pollett
authors.forename: Robert
authors.initials: R
authors.lastname: Riddell
authors.forename: Mark S
authors.initials: MS
authors.lastname: Silverberg
authors.forename: Paul
authors.initials: P
authors.lastname: Kortan
authors.forename: Gary R
authors.initials: GR
authors.lastname: May
authors.forename: Steven
authors.initials: S
authors.lastname: Gallinger
mesh_terms: D000236:Adenoma / Q000175:diagnosis* / Q000209:etiology / Q000601:surgery; D011125:Adenomatous Polyposis Coli / Q000150:complications / Q000175:diagnosis*; D000328:Adult; D000368:Aged; D002985:Clinical Protocols; D019468:Disease Management; D018450:Disease Progression; D004379:Duodenal Neoplasms / Q000145:classification / Q000175:diagnosis* / Q000209:etiology / Q000601:surgery; D004384:Duodenoscopy; D055088:Early Detection of Cancer; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging; D011159:Population Surveillance; D011446:Prospective Studies; D012042:Registries; D012307:Risk Factors