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Adenomatous Polyposis Syndromes: Familial Adenomatous Polyposis and MutYH-Associated Polyposis

  • Genetic Syndromes, Screening, and Surveillance in Colorectal Cancer (N Kubiliun, Section Editor)
  • Published:
Current Colorectal Cancer Reports

Abstract

The purpose of this review is to provide an overview of the etiology, diagnosis, and clinical management of the two most common polyposis; change adenomatous to polyposis hereditary colorectal cancer syndromes, familial adenomatous polyposis (FAP) and MUTYH-associated polyposis (MAP). The syndromes are caused by inherited genetic variants in the APC and MutYH genes, respectively. Both syndromes carry significant increased risk of colorectal and extracolonic cancers. Intense surveillance is required to reduce this risk, and colectomy or proctocolectomy is frequently the ultimate form of colorectal cancer risk reduction. The colorectal phenotype and extracolonic manifestations continue to evolve as more is learned about the natural history of the diseases, particularly MAP.

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Authors and Affiliations

  1. Department of Colorectal Surgery, Digestive Disease and Surgery Institute, Desk A30, 9500 Euclid Avenue, Cleveland, OH, 44195, USA

    Jean H. Ashburn & Matthew F. Kalady

  2. Sanford R. Weiss, MD, Center for Hereditary Colorectal Neoplasia, Digestive Disease and Surgery Institute, Cleveland Clinic Foundation, 9500 Euclid Ave, Cleveland, OH, 44195, USA

    Jean H. Ashburn & Matthew F. Kalady

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  1. Jean H. Ashburn
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Correspondence to Matthew F. Kalady.

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Jean H. Ashburn and Matthew F. Kalady declare they have no conflict of interest.

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This article is part of the Topical Collection on Genetic Syndromes, Screening, and Surveillance in Colorectal Cancer

Prepared for Current Colorectal Cancer Reports: Genetic Syndromes, Screening and Surveillance in Colorectal Cancer

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Ashburn, J.H., Kalady, M.F. Adenomatous Polyposis Syndromes: Familial Adenomatous Polyposis and MutYH-Associated Polyposis. Curr Colorectal Cancer Rep 13, 302–309 (2017). https://doi.org/10.1007/s11888-017-0379-0

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  • DOI: https://doi.org/10.1007/s11888-017-0379-0

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