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Principles of Clinical Cancer Genetics pp 63–76Cite as

Hereditary Colon Cancer: Colonic Polyposis Syndromes

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Abstract

In the U.S., the lifetime risk of colorectal cancer among both men and women is approximately 6% [1]. Although as many as 25% of colorectal cancers are associated with a family history of the disease, approximately 3–5% of cases arise in the setting of well-described familial genetic syndromes [2]. The most prevalent familial colorectal cancer syndrome is Lynch syndrome or hereditary nonpolyposis colorectal cancer (HNPCC). Patients with Lynch do not present clinically with an unusually heavy burden of adenomatous polyps, the precursor to the vast majority of colorectal cancers. On the other hand, the remaining 1–2% of hereditary colorectal cancer syndromes are characterized by significant polyposis. These syndromes can be broadly classified as adenomatous polyposis syndromes, hamartomatous polyposis syndromes, and the hyperplastic polyposis syndrome (Table 5.1). In this chapter, we review the salient clinical and genetic features of each of these polyposis syndromes.

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Authors and Affiliations

  1. GI Cancer Genetics Program, Gastrointestinal Unit and Cancer Center, Boston, MA, USA

    Andrew T. Chan & Daniel C. Chung

  2. Harvard Medical School, Boston, MA, USA

    Andrew T. Chan & Daniel C. Chung

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  1. Andrew T. Chan
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  2. Daniel C. Chung
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Correspondence to Andrew T. Chan or Daniel C. Chung .

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Editors and Affiliations

  1. Cancer Center and Gastrointestinal Unit, Harvard Medical School, Massachusetts General Hospital, Boston, 02114, USA

    Daniel C. Chung

  2. Cancer Center, Harvard Medical School and, Massachusetts General Hospital, Boston, 02114, USA

    Daniel A. Haber

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Chan, A.T., Chung, D.C. (2010). Hereditary Colon Cancer: Colonic Polyposis Syndromes. In: Chung, D., Haber, D. (eds) Principles of Clinical Cancer Genetics. Springer, Boston, MA. https://doi.org/10.1007/978-0-387-93846-2_5

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