Hereditary nonpolyposis colorectal cancer classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Maria Fernanda Villarreal, M.D. [2]

Overview

Hereditary nonpolyposis colorectal cancer may be classified into 2 types: Lynch syndrome I (familial colon cancer) and Lynch syndrome II (hereditary nonpolyposis colorectal cancer associated with other cancers of the gastrointestinal tract or reproductive system).[1] Other variants, such as Muir-Torre syndrome and Turcot syndrome are considered subtypes of hereditary nonpolyposis colorectal cancer.[2]

Classification

Lynch syndrome I (familial colon cancer) and Lynch syndrome II (hereditary nonpolyposis colorectal cancer associated with other cancers of the gastrointestinal tract or reproductive system).[2]

Lynch Syndrome I (Familial Colon Cancer)

  • Site-specific colonic cancer

Lynch Syndrome II

  • Includes extracolonic cancer, particularly carcinoma of the stomach, endometrium
  • The most common cancer that develop among such patients is endometrial carcinoma

Variants

Muir–Torre syndrome[3]

Turcot syndrome[4]

  • Hereditary autosomal recessive syndrome
  • Considered a subtype of HNPCC
  • Individuals are prone to develop intestinal polyposis in addition to CNS tumors such as glioblastoma or medulloblastoma
  • Two-thirds of patients have mutations in the APC gene

References

  1. Hereditary nonpolyposis colorectal cancer. Wikipedia. https://en.wikipedia.org/wiki/Hereditary_nonpolyposis_colorectal_cancer Accessed on December 01, 2015
  2. 2.0 2.1 Lynch HT, Lanspa S, Smyrk T, Boman B, Watson P, Lynch J (1991). "Hereditary nonpolyposis colorectal cancer (Lynch syndromes I & II). Genetics, pathology, natural history, and cancer control, Part I". Cancer Genet. Cytogenet. 53 (2): 143–60. PMID 1648437.
  3. Muir-Torre syndrome. Dr Frank Gaillard et al.http://radiopaedia.org/articles/muir-torre-syndrome Radiopaedia 2015.
  4. Turcot syndrome. Dr Henry Knipe and Dr Frank Gaillard et al.http://radiopaedia.org/articles/turcot-syndrome Radiopaedia 2015.


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