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Hereditary Colorectal Cancer and Polyposis Syndromes

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Abstract

Colorectal cancer (CRC) is the second leading cause of death in the United States, with approximately 10–30 % of patients having a positive family history. Lynch syndrome is the most common inherited CRC accounting for approximately 5–10 % of all CRC cases. Familial adenomatous polyposis (FAP) is the next common, accounting for approximately 1 % of all CRC cases. Other polyposis syndromes include attenuated FAP (aFAP), MYH-associated polyposis (MAP), Peutz–Jeghers syndrome (PJS), and juvenile polyposis syndrome (JPS). Each of these polyposis syndromes confers with it a higher lifetime risk of development of CRC due to their unique genetic mutations, necessitating early surveillance protocols and possible prophylactic surgical intervention. Extracolonic manifestations and cancer risks are also increased, emphasizing vigilance and early recognition of these anomalies. Preclinical trials have shown clear benefit in NSAIDS and COX inhibitors to exert their effects against all clinical stages of colorectal neoplasia. However, trials for deducing the specific aspects of adenoma reduction are still ongoing, especially in high-risk individuals with FAP and Lynch syndrome.

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Authors and Affiliations

  1. Department of Surgery, Kaleida Health/Buffalo General Med Center, State University of New York at Buffalo, 100 High St., Buffalo, 14203, NY, USA

    Edward Eun Cho M.D., Sc.M.

  2. Department of Surgery, Jersey Shore University Medical Center/Meridian Health, 1945 State Highway 33, 4 floor Ackerman, Neptune, 07753, NJ, USA

    John F. Gibbs M.D.

  3. Department of Surgical Oncology, UT MD Anderson Cancer Center, 1400 Pressler Street, Unit 1484, Houston, 77044, TX, USA

    Miguel Rodriguez-Bigas M.D.

  4. Department of Surgery, Walter Reed National Military Medical Center/National Cancer Institute, 9609 Medical Center Drive, Rm 5E- 228, MSC 9782, Bethesda, 20892-9782, MD, USA

    Luz Maria Rodriguez M.D.

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  1. Edward Eun Cho M.D., Sc.M.
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  2. John F. Gibbs M.D.
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  3. Miguel Rodriguez-Bigas M.D.
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  4. Luz Maria Rodriguez M.D.
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Correspondence to Luz Maria Rodriguez M.D. .

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Editors and Affiliations

  1. Department of Surgery, LSU Health Sciences Center-Shreveport, Shreveport, Louisiana, USA

    Quyen D. Chu

  2. Department of Surgery, Jersey Shore University Medical Center, Neptune, New Jersey, USA

    John F. Gibbs

  3. John C. McDonald Regional Transplant Center, Willis Knighton Health System, Shreveport, Louisiana, USA

    Gazi B. Zibari

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Cho, E.E., Gibbs, J.F., Rodriguez-Bigas, M., Rodriguez, L.M. (2015). Hereditary Colorectal Cancer and Polyposis Syndromes. In: Chu, Q., Gibbs, J., Zibari, G. (eds) Surgical Oncology. Springer, New York, NY. https://doi.org/10.1007/978-1-4939-1423-4_20

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