Elsevier

European Journal of Cancer

Volume 31, Issues 7–8, July–August 1995, Pages 1149-1153
European Journal of Cancer

Upper intestinal surveillance in familial adenomatous polyposis

https://doi.org/10.1016/0959-8049(95)00171-EGet rights and content

Abstract

Our understancling of the natural history of upper gastrointestinal (GI) involvement in familial adenomatous polyposis (FAP) is still evolving, although we know that the main cause of death after colectomy in FAP is upper GI malignancy, affecting 5% of patients. The aim of duodenal surveillance is to target high risk individuals and identify cancers early. We have screened 200 patients prospectively and have observed that duodenal polyposis progresses slowly, but there are some young people who have severe disease who merit close observation. We pay particular attention to endoscopic technique and histological detail, and use a duodenal staging system. Patients are offered randomisation to studies of chemopreventive agents, and those with advanced disease are considered for surgery. Successful management is inhibited by our deficient knowledge of the natural history of upper gastrointestinal polyposis, and by our inability to identify high risk individuals with histological markers rather than because of any technological deficiencies in endoscopic equipment.

References (36)

  • JM Church et al.

    Gastroduodenal polyps in patients with familial adenomatous polyposis

    Dis Colon Rectum

    (1992)
  • S Bulow et al.

    Gastroduodenal polyps in familial polyposis coli

    Dis Colon Rectum

    (1985)
  • HJ Jarvinen et al.

    Gastroduodenal polyps in familial adenomatous and juvenile polyposis

    Endoscopy

    (1986)
  • RG Sarre et al.

    Gastric and duodenal polyps in familial adenomatous polyposis: a prospective study of the nature and prevalence of upper gastrointestinal polyps

    Gut

    (1987)
  • JG Park et al.

    Risk of gastric cancer among Korean familial adenomatous polyposis (FAP) patients (meeting abstract)

  • T Iwama et al.

    The impact of familial adenomatous polyposis on the tumorigenesis and mortality at the several organs: its rational treatment

    Ann Surg

    (1993)
  • J Utsunomiya et al.

    Gastric lesions of familial polyposis coli

    Cancer

    (1974)
  • AD Spigelman et al.

    The upper gastrointestinal tract

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