title: Worldwide survey among polyposis registries of surgical management of severe duodenal adenomatosis in familial adenomatous polyposis.
abstract: BACKGROUND
  The lifetime risk of developing duodenal cancer in familial adenomatous polyposis (FAP) is about 5 per cent. When and to what extent surgical intervention should be undertaken to prevent death from invasive carcinoma is controversial. The aim of this study was to determine the effectiveness of various surgical treatments for cancer and severe duodenal adenomatosis.
  
  
  METHODS
  A questionnaire was mailed to the members of the Leeds Castle Polyposis Group to obtain data on patients with FAP, treated for duodenal cancer or severe duodenal adenomatosis after 1990.
  
  
  RESULTS
  Sixty-nine patients were included. The indication for surgery was invasive cancer in 13 patients, of whom six died from metastatic disease. Fifty-six patients were initially treated for severe duodenal adenomatosis, five (9 per cent) of whom died from metastatic disease (P = 0.002). In surviving patients, adenomas recurred after ampullectomy (six of eight, at mean follow-up of 11 months), after duodenotomy with polypectomy (17 of 21, at mean 29 months) and after pancreatoduodenectomy (six of 25, at mean 47 months). None of six patients who underwent a pancreas-sparing duodenectomy had recurrence of adenoma (mean follow-up 11 months).
  
  
  CONCLUSIONS
  Surgery for duodenal adenomatosis should take place before endoscopic biopsy reveals invasive cancer. Even after extensive surgical procedures, small bowel adenomas may occur, emphasizing the need for chemoprevention.
authors.affiliation: The Netherlands Foundation for the Detection of Hereditary Tumours and Department of Gastroenterology, Leiden University Medical Center, Leiden, The Netherlands.
authors.forename: W H
authors.initials: WH
authors.lastname: de Vos tot Nederveen Cappel
authors.forename: H J
authors.initials: HJ
authors.lastname: Järvinen
authors.forename: J
authors.initials: J
authors.lastname: Björk
authors.forename: T
authors.initials: T
authors.lastname: Berk
authors.forename: G
authors.initials: G
authors.lastname: Griffioen
authors.forename: H F A
authors.initials: HF
authors.lastname: Vasen
mesh_terms: D000236:Adenoma / Q000209:etiology / Q000601:surgery*; D011125:Adenomatous Polyposis Coli / Q000150:complications*; D000328:Adult; D000368:Aged; D003082:Colectomy / Q000379:methods; D004379:Duodenal Neoplasms / Q000209:etiology / Q000601:surgery*; D014943:Global Health; D006801:Humans; D008875:Middle Aged; D009361:Neoplasm Invasiveness; D011795:Surveys and Questionnaires