title: Desmoid tumors in a dutch cohort of patients with familial adenomatous polyposis.
abstract: OBJECTIVE
  Desmoid tumors are a severe extracolonic manifestation in familial adenomatous polyposis (FAP). Identification of risk factors might be helpful in the management of FAP patients with such tumors. The aim of this study was to assess potential risk factors for the development of desmoids in a cohort of Dutch FAP patients.
  
  
  METHODS
  The medical records of 735 FAP patients were analyzed for the occurrence of desmoids. Relative risks and survival times were calculated to assess the influence of potential risk factors (female sex, family history, mutation site, abdominal surgery, and pregnancy) on desmoid development.
  
  
  RESULTS
  Desmoid tumors were identified in 66 of the 735 patients (9%). The cumulative risk of developing desmoids was 14%. No correlation was found between specific adenomatous polyposis coli mutation sites and desmoid development. Patients with a positive family history for desmoids had a significant increased risk to develop this tumor (30% vs 6.7%, P < .001). No association was found between female sex or pregnancy and desmoid development. Most desmoid patients (95%) had undergone previous abdominal surgery. In a substantial proportion of patients with an ileorectal anastomosis, it was impossible to convert the ileorectal anastomosis to an ileal pouch-anal anastomosis as a result of desmoid development.
  
  
  CONCLUSIONS
  A positive family history of desmoids is an evident risk factor for developing desmoids. Most desmoids develop after colectomy. No correlation was found between desmoids and the adenomatous polyposis coli gene mutation site, female sex, and pregnancy. Ileal pouch-anal anastomosis is the appropriate type of surgery in FAP patients with a positive family history for desmoids.
authors.affiliation: The Netherlands Foundation for the Detection of Hereditary Tumours, Leiden, The Netherlands.
authors.forename: Marry H
authors.initials: MH
authors.lastname: Nieuwenhuis
authors.forename: Wouter
authors.initials: W
authors.lastname: De Vos Tot Nederveen Cappel
authors.forename: Akke
authors.initials: A
authors.lastname: Botma
authors.forename: Fokko M
authors.initials: FM
authors.lastname: Nagengast
authors.forename: Jan H
authors.initials: JH
authors.lastname: Kleibeuker
authors.forename: Elisabeth M H
authors.initials: EM
authors.lastname: Mathus-Vliegen
authors.forename: Evelien
authors.initials: E
authors.lastname: Dekker
authors.forename: Jan
authors.initials: J
authors.lastname: Dees
authors.forename: Juul
authors.initials: J
authors.lastname: Wijnen
authors.forename: Hans F A
authors.initials: HF
authors.lastname: Vasen
mesh_terms: D011125:Adenomatous Polyposis Coli / Q000150:complications*; D000293:Adolescent; D000328:Adult; D003082:Colectomy; D005192:Family Health; D005260:Female; D018222:Desmoid Tumors / Q000453:epidemiology*; D006801:Humans; D015994:Incidence; D008297:Male; D008875:Middle Aged; D009426:Netherlands; D012307:Risk Factors