Hereditary Colorectal Cancer Syndromes

Surg Clin North Am. 2017 Jun;97(3):605-625. doi: 10.1016/j.suc.2017.01.009.

Abstract

Awareness of hereditary colorectal cancer syndromes is important to facilitate their identification because affected patients are at increased risk for early onset, synchronous, and metachronous colorectal malignancies, and certain extracolonic malignancies depending on the syndrome. Identification of an affected individual allows for screening and early interventions for patients and their at-risk kindred. Genetic counseling and testing is important to the care of these patients. As knowledge of the genetic basis of these syndromes grows, unique genotype-phenotype profiles allow clinicians to tailor surveillance and treatment strategies based on individual risk.

Keywords: Familial adenomatous polyposis; Hereditary nonpolyposis colorectal cancer; Inherited colon cancer; Lynch syndrome; MUTYH-associated polyposis; Serrated polyposis syndrome.

Publication types

  • Review

MeSH terms

  • Adenomatous Polyposis Coli / genetics
  • Colonic Polyps / genetics*
  • Colonic Polyps / surgery
  • Colorectal Neoplasms / genetics*
  • Colorectal Neoplasms / prevention & control
  • Colorectal Neoplasms / surgery
  • Colorectal Neoplasms, Hereditary Nonpolyposis / genetics
  • DNA Glycosylases / genetics
  • Genetic Testing
  • Humans
  • Mutation
  • Neoplastic Syndromes, Hereditary / genetics*

Substances

  • DNA Glycosylases
  • mutY adenine glycosylase