title: Gastrointestinal polyposis syndromes.
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).
authors.affiliation: Department of Pathology, University Medical Center, Heidelberglaan 100, 3584 CX Utrecht, The Netherlands. l.a.brosens@amc.uva.nl
authors.forename: Lodewijk A A
authors.initials: LA
authors.lastname: Brosens
authors.forename: W Arnout
authors.initials: WA
authors.lastname: van Hattem
authors.forename: Marnix
authors.initials: M
authors.lastname: Jansen
authors.forename: Wendy W J
authors.initials: WW
authors.lastname: de Leng
authors.forename: Francis M
authors.initials: FM
authors.lastname: Giardiello
authors.forename: G Johan A
authors.initials: GJ
authors.lastname: Offerhaus
mesh_terms: D011125:Adenomatous Polyposis Coli / Q000175:diagnosis / Q000235:genetics / Q000473:pathology* / Q000628:therapy; D025601:Adenomatous Polyposis Coli Protein / Q000235:genetics / Q000378:metabolism; D000818:Animals; D005770:Gastrointestinal Neoplasms / Q000175:diagnosis / Q000235:genetics / Q000473:pathology* / Q000628:therapy; D006801:Humans; D010580:Peutz-Jeghers Syndrome / Q000175:diagnosis / Q000235:genetics / Q000473:pathology / Q000628:therapy; D015398:Signal Transduction; D016212:Transforming Growth Factor beta / Q000378:metabolism
chemical_list: D025601:Adenomatous Polyposis Coli Protein; D016212:Transforming Growth Factor beta