Hereditary nonpolyposis colorectal cancer classification: Difference between revisions
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==Overview== | ==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 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]]).<ref name="wiki"> Hereditary nonpolyposis colorectal cancer. Wikipedia. https://en.wikipedia.org/wiki/Hereditary_nonpolyposis_colorectal_cancer Accessed on December 01, 2015 </ref> Other variants, such as Muir-Torre syndrome and Turcot syndrome are considered subtypes of hereditary nonpolyposis colorectal cancer.<ref name="pmid1648437">{{cite journal |vauthors=Lynch HT, Lanspa S, Smyrk T, Boman B, Watson P, Lynch J |title=Hereditary nonpolyposis colorectal cancer (Lynch syndromes I & II). Genetics, pathology, natural history, and cancer control, Part I |journal=Cancer Genet. Cytogenet. |volume=53 |issue=2 |pages=143–60 |year=1991 |pmid=1648437 |doi= |url=}}</ref> | ||
==Classification== | ==Classification== | ||
Lynch syndrome | 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).<ref name="pmid1648437">{{cite journal |vauthors=Lynch HT, Lanspa S, Smyrk T, Boman B, Watson P, Lynch J |title=Hereditary nonpolyposis colorectal cancer (Lynch syndromes I & II). Genetics, pathology, natural history, and cancer control, Part I |journal=Cancer Genet. Cytogenet. |volume=53 |issue=2 |pages=143–60 |year=1991 |pmid=1648437 |doi= |url=}}</ref> | ||
===Lynch Syndrome I (Familial Colon Cancer)=== | ===Lynch Syndrome I (Familial Colon Cancer)=== | ||
* Site-specific colonic cancer | * Site-specific colonic cancer | ||
===Lynch Syndrome II=== | ===Lynch Syndrome II=== | ||
* Includes extracolonic cancer, particularly carcinoma of the [[stomach]] and [[endometrium]] | |||
* Includes extracolonic cancer, particularly carcinoma of the stomach | * The most common cancer that develop among such patients is endometrial carcinoma | ||
* The most common is endometrial carcinoma | |||
===Variants=== | ===Variants=== | ||
'''Muir–Torre syndrome'''<ref name=radiopedia1> Muir-Torre syndrome. Dr Frank Gaillard et al.http://radiopaedia.org/articles/muir-torre-syndrome Radiopaedia 2015.</ref> | '''Muir–Torre syndrome'''<ref name=radiopedia1> Muir-Torre syndrome. Dr Frank Gaillard et al.http://radiopaedia.org/articles/muir-torre-syndrome Radiopaedia 2015.</ref> | ||
::* | ::*Hereditary [[autosomal dominant]] syndrome | ||
::*Considered a subtype of [[HNPCC]] | ::*Considered a subtype of [[HNPCC]] | ||
::*Individuals are prone to develop cancers of the colon, breast, and genitourinary tract, and skin lesions, such as [[keratoacanthoma]]s and sebaceous tumors | ::*Individuals are prone to develop cancers of the colon, breast, and genitourinary tract, and skin lesions, such as [[keratoacanthoma]]s and sebaceous tumors | ||
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'''Turcot syndrome'''<ref name=radiopedia> Turcot syndrome. Dr Henry Knipe and Dr Frank Gaillard et al.http://radiopaedia.org/articles/turcot-syndrome Radiopaedia 2015. </ref> | '''Turcot syndrome'''<ref name=radiopedia> Turcot syndrome. Dr Henry Knipe and Dr Frank Gaillard et al.http://radiopaedia.org/articles/turcot-syndrome Radiopaedia 2015. </ref> | ||
::* | ::*Hereditary [[autosomal recessive]] syndrome | ||
::*Considered a subtype of [[HNPCC]] | ::*Considered a subtype of [[HNPCC]] | ||
::*Individuals are prone to develop intestinal polyposis | ::*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 | ::*Two-thirds of patients have mutations in the APC gene | ||
Revision as of 19:31, 6 February 2016
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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 and endometrium
- The most common cancer that develop among such patients is endometrial carcinoma
Variants
Muir–Torre syndrome[3]
- Hereditary autosomal dominant syndrome
- Considered a subtype of HNPCC
- Individuals are prone to develop cancers of the colon, breast, and genitourinary tract, and skin lesions, such as keratoacanthomas and sebaceous tumors
- Genes affected are MLH1, MSH2, and more recently, MSH6, and are involved in DNA mismatch repair
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
- ↑ Hereditary nonpolyposis colorectal cancer. Wikipedia. https://en.wikipedia.org/wiki/Hereditary_nonpolyposis_colorectal_cancer Accessed on December 01, 2015
- ↑ 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.
- ↑ Muir-Torre syndrome. Dr Frank Gaillard et al.http://radiopaedia.org/articles/muir-torre-syndrome Radiopaedia 2015.
- ↑ Turcot syndrome. Dr Henry Knipe and Dr Frank Gaillard et al.http://radiopaedia.org/articles/turcot-syndrome Radiopaedia 2015.