Gastrointestinal Polyposis Syndromes

Authors:
  • WHW. Arnout van Hattem
    W. Arnout van Hattem
  • MJMarnix Jansen
    Marnix Jansen
  • WLWendy W.J. de Leng
    Wendy W.J. de Leng
  • FGFrancis M. Giardiello
    Francis M. Giardiello
  • GOG. Johan A. Offerhaus
    G. Johan A. Offerhaus
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Abstract
Colorectal cancer is one of the leading causes of cancer-related death in the Western society, and the incidence is rising. Rare hereditary gastrointestinal polyposis syndromes that predispose to colorectal cancer have provided a model for the investigation of cancer initiation and progression in the general population. Many insights in the molecular genetic basis of cancer have emerged from the study of these syndromes. This review discusses the genetics and clinical manifestations of the three most common syndromes with gastrointestinal polyposis and an increased risk of colorectal cancer: familial adenomatous polyposis (FAP), juvenile polyposis (JP) and Peutz- Jeghers syndrome (PJS).