Hereditary nonpolyposis colorectal cancer classification: Difference between revisions
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* Includes extracolonic cancer, particularly carcinoma of the stomach, endometrium | * Includes extracolonic cancer, particularly carcinoma of the stomach, endometrium | ||
* Associated condition, Muir-Torre syndrome | * Associated condition, Muir-Torre syndrome | ||
* The most common of which is endometrial carcinoma. | * The most common of which is endometrial carcinoma | ||
===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> | |||
::*Is a rare hereditary, [[autosomal dominant]] [[cancer]] syndrome | |||
::*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 | |||
::*Genes affected are [[MLH1]], [[MSH2]], and more recently, MSH6, and are involved in [[DNA mismatch repair]] | |||
'''Turcot syndrome'''<ref name=radiopedia> Turcot syndrome. Dr Henry Knipe and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/turcot-syndrome</ref> | |||
::*Is a very rare hereditary [[autosomal recessive]] syndrome | |||
::*Considered a subtype of [[HNPCC]] | |||
::*Individuals are prone to develop intestinal polyposis | |||
::*Related to CNS tumors: most commonly glioblastoma or medulloblastoma | |||
::*Two-thirds of patients have mutations in the APC gene | |||
==References== | ==References== |
Revision as of 14:36, 2 December 2015
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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 according to MSI-H into 3 subtypes: right-sided poorly differentiated cancers, right-sided mucinous cancers, and adenocarcinomas in any location showing any measurable level of intraepithelial lymphocyte (TIL). In addition, HNPCC can be divided into Lynch syndrome I (familial colon cancer) and Lynch syndrome II (HNPCC associated with other cancers of the gastrointestinal tract or reproductive system).[1]
Classification
Lynch syndrome may be classified into 2 subtypes: Type I and Type 2[2]
Lynch syndrome I (familial colon cancer)
- Site-specific colonic cancer
Lynch syndrome II
- Extracolonic cancer
- Includes extracolonic cancer, particularly carcinoma of the stomach, endometrium
- Associated condition, Muir-Torre syndrome
- The most common of which is endometrial carcinoma
Variants
Muir–Torre syndrome[3]
- Is a rare hereditary, autosomal dominant cancer 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]
- Is a very rare hereditary autosomal recessive syndrome
- Considered a subtype of HNPCC
- Individuals are prone to develop intestinal polyposis
- Related to CNS tumors: most commonly 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
- ↑ 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. Radiopaedia 2015. http://radiopaedia.org/articles/turcot-syndrome