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Gastrointestinal Polyposis Syndromes

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Inherited Cancer Syndromes

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Abstract

The gastrointestinal polyposis syndromes (PS) are characterized by the development of multiple polyps, characteristically colorectal, but occasionally involving the small intestine and (less commonly) the stomach. PS may be characterized by the histologic type of polyps found, being either adenomatous, hamartomatous, or hyperplastic. Additionally, they may be characterized by the mode of inheritance, being either autosomal dominant or autosomal recessive. Changes in both these types of characterization have recently occurred. Until recently, it was thought that all polyposis syndromes were inherited in an autosomal-dominant fashion. However, the discovery of Mut Y homologue-associated polyposis (MAP), which is inherited in an autosomal-recessive fashion, changed this [1]. Additionally, the (relatively) recent addition of hyperplastic polyposis (HPP) was unique as this was the first type of PS involving hyperplastic polyps. The last several years have been a very active time, both with the addition of these new PS and further additions to our knowledge base about the known ones. This chapter will deal with the different PS and the management of each.

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  1. Baptist Hospital, Nashville, TN, USA

    William J. Harb

  2. Cumberland Surgical Associates, PLC, 2011 Church St. Suite 703, Nashville, TN, 37203, USA

    William J. Harb

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    C. Neal Ellis

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Harb, W.J. (2011). Gastrointestinal Polyposis Syndromes. In: Ellis, C. (eds) Inherited Cancer Syndromes. Springer, New York, NY. https://doi.org/10.1007/978-1-4419-6821-0_5

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