title: Duodenal surveillance improves the prognosis after duodenal cancer in familial adenomatous polyposis.
abstract: OBJECTIVE
  Duodenal adenomatosis in familial adenomatous polyposis results in a cancer risk that increases with age. Endoscopic surveillance has been recommended, but the effect has not yet been documented. The aim of this study was to present the results of long-term duodenal surveillance and to evaluate the risk of cancer development.
  
  
  METHODS
  Follow up of patients in a previous study with gastroduodenoscopy in 1990-2010. Statistical analysis included the χ(2) test, actuarial method and Kaplan-Meier analysis.
  
  
  RESULTS
  Among 304 patients, 261 (86%) had more than one endoscopy. The median follow up was 14 (interquartile range, 9-17) years. The cumulative lifetime risk of duodenal adenomatosis was 88% (95% CI, 84-93), and of Spigelman stage IV was 35% (95% CI, 25-45). The Spigelman stage improved in 32 (12%) patients, remained unchanged in 88 (34%) and worsened in 116 (44%). Twenty (7%) patients had duodenal cancer at a median age of 56 (range, 44-82) years. The cumulative cancer incidence was 18% at 75 years of age (95% CI, 8-28) and increased with increasing Spigelman stage at the index endoscopy to 33% in Spigelman stage IV (P < 0.0001). The median overall survival was 6.4 years (95% CI, 1.7 to not estimated): 8 years after a screen-detected cancer vs 0.8 years (95% CI, 0.03-1.7) after a symptomatic cancer (P < 0.0001). The location of the mutation in the APC gene did not influence the risk of developing Spigelman stage IV (P = 0.46) or duodenal cancer (P = 0.83).
  
  
  CONCLUSIONS
  The risk of duodenal cancer in familial adenomatous polyposis is considerable, and regular surveillance and cancer prophylactic surgery result in a significantly improved prognosis.
authors.affiliation: The Danish Polyposis Register, Hvidovre University Hospital, Copenhagen, Denmark. sbulow@dadlnet.dk
authors.forename: S
authors.initials: S
authors.lastname: Bülow
authors.forename: I J
authors.initials: IJ
authors.lastname: Christensen
authors.forename: H
authors.initials: H
authors.lastname: Højen
authors.forename: J
authors.initials: J
authors.lastname: Björk
authors.forename: M
authors.initials: M
authors.lastname: Elmberg
authors.forename: H
authors.initials: H
authors.lastname: Järvinen
authors.forename: A
authors.initials: A
authors.lastname: Lepistö
authors.forename: M
authors.initials: M
authors.lastname: Nieuwenhuis
authors.forename: H
authors.initials: H
authors.lastname: Vasen
mesh_terms: D011125:Adenomatous Polyposis Coli / Q000453:epidemiology / Q000473:pathology* / Q000601:surgery; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D003718:Denmark / Q000453:epidemiology; D004379:Duodenal Neoplasms / Q000453:epidemiology / Q000473:pathology* / Q000601:surgery; D004384:Duodenoscopy; D005260:Female; D005387:Finland / Q000453:epidemiology; D005500:Follow-Up Studies; D005773:Gastroscopy; D006801:Humans; D015994:Incidence; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging; D009426:Netherlands / Q000453:epidemiology; D009664:Norway / Q000453:epidemiology; D011159:Population Surveillance; D011379:Prognosis; D012306:Risk; D013548:Sweden / Q000453:epidemiology