title: Surgical management for advanced duodenal adenomatosis and duodenal cancer in Dutch patients with familial adenomatous polyposis: a nationwide retrospective cohort study.
abstract: BACKGROUND
  Duodenal cancer is a major cause of mortality in patients with familial adenomatous polyposis (FAP). The clinical challenge is to perform duodenectomy before cancer develops; however, procedures are associated with complications. Our aim was to gain insight into the pros and cons of prophylactic duodenectomy.
  
  
  METHODS
  Patients with FAP from the nationwide Dutch polyposis registry who underwent prophylactic duodenectomy or were diagnosed with duodenal cancer were identified and classified as having benign disease or cancer at preoperative endoscopy. Surveillance, clinical presentation, surgical management, outcome, survival, and recurrence were compared.
  
  
  RESULTS
  Of 1,066 patients with FAP in the registry, 52 (5%; 25 males) were included: 36 with benign adenomatosis (median: 48 years old; including two (6%) cancer cases diagnosed after operation), and 16 with cancer (median: 53 years old). Cancer cases had been diagnosed with colorectal cancer more often (6% vs 44%; P < .01). Forty-three patients underwent duodenectomy (35 benign/eight cancer): 30-day mortality was 4.7% (n = 2), and in-hospital morbidity occurred in 21 patients (49%), without differences between patients with benign adenomatosis and cancer. Adenomas recurred in reconstructed proximal small bowel in 14 of 28 patients (50%, median time to recurrence: 75 months), and one patient developed cancer. Median survival of all 18 cancer cases in the registry (1.7%; 12 ampullary/six duodenal) was 11 months.
  
  
  CONCLUSIONS
  Prognosis of duodenal cancer in patients with FAP is poor, which justifies an aggressive approach to advanced benign adenomatosis. Strict adherence to recommended surveillance intervals is essential for a well-timed intervention. Given the substantial morbidity and mortality of duodenectomy, patients' individual characteristics are to be critically evaluated preoperatively. As adenomas recur, postoperative endoscopic surveillance is mandatory.
authors.affiliation: Department of Gastroenterology and Hepatology, Radboud University Nijmegen Medical Centre, Nijmegen, the Netherlands. b.vanheumen@mdl.umcn.nl
authors.forename: Bjorn W H
authors.initials: BW
authors.lastname: van Heumen
authors.forename: Marry H
authors.initials: MH
authors.lastname: Nieuwenhuis
authors.forename: Harry
authors.initials: H
authors.lastname: van Goor
authors.forename: Lisbeth E M H
authors.initials: LE
authors.lastname: Mathus-Vliegen
authors.forename: Evelien
authors.initials: E
authors.lastname: Dekker
authors.forename: Dirk J
authors.initials: DJ
authors.lastname: Gouma
authors.forename: Jan
authors.initials: J
authors.lastname: Dees
authors.forename: Casper H J
authors.initials: CH
authors.lastname: van Eijck
authors.forename: Hans F A
authors.initials: HF
authors.lastname: Vasen
authors.forename: Fokko M
authors.initials: FM
authors.lastname: Nagengast
mesh_terms: D011125:Adenomatous Polyposis Coli / Q000150:complications* / Q000401:mortality; D000293:Adolescent; D000328:Adult; D002648:Child; D015331:Cohort Studies; D004379:Duodenal Neoplasms / Q000209:etiology / Q000401:mortality / Q000517:prevention & control / Q000601:surgery*; D004386:Duodenum / Q000601:surgery*; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local; D009426:Netherlands; D011183:Postoperative Complications; D011379:Prognosis; D012042:Registries; D012189:Retrospective Studies; D015996:Survival Rate; D016896:Treatment Outcome; D055815:Young Adult