Abstract | BACKGROUND & AIMS:
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:
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Authors | Marry H Nieuwenhuis, Wouter De Vos Tot Nederveen Cappel, Akke Botma, Fokko M Nagengast, Jan H Kleibeuker, Elisabeth M H Mathus-Vliegen, Evelien Dekker, Jan Dees, Juul Wijnen, Hans F A Vasen |
Journal | Clinical gastroenterology and hepatology : the official clinical practice journal of the American Gastroenterological Association
(Clin Gastroenterol Hepatol)
Vol. 6
Issue 2
Pg. 215-9
(Feb 2008)
ISSN: 1542-7714 [Electronic] United States |
PMID | 18237870
(Publication Type: Journal Article)
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Topics |
- Adenomatous Polyposis Coli
(complications)
- Adolescent
- Adult
- Colectomy
- Family Health
- Female
- Fibromatosis, Aggressive
(epidemiology)
- Humans
- Incidence
- Male
- Middle Aged
- Netherlands
- Risk Factors
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