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]Ali Akram, M.B.B.S.[3]
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). Other variants such as Muir-Torre syndrome and Turcot syndrome are considered subtypes of hereditary nonpolyposis colorectal cancer.
Classification
Lynch Syndrome I
- Site-specific colonic cancer.
Lynch Syndrome II
- Includes extracolonic cancer.
- The most common cancer that develops among such patients is endometrial carcinoma.
Variants
- Hereditary autosomal dominant syndrome.
- Considered a subtype of hereditary nonpolyposis colorectal cancer.
- Affected individuals are prone to develop cancers of the colon, breast, and genitourinary tract along with skin lesions such as keratoacanthomas and sebaceous tumors.
- Genes affected are MLH1, MSH2, and MSH6, which are involved in the DNA mismatch repair.
- Hereditary autosomal recessive syndrome.
- Considered a sub-type of hereditary nonpolyposis colorectal cancer.
- Individuals are prone to develop intestinal polyposis in addition to CNS tumors such as glioblastoma or medulloblastoma.
References
- ↑ Mintsoulis D, Beecker J (2016). "Muir-Torre syndrome". CMAJ. 188 (5): E95. doi:10.1503/cmaj.150171. PMC 4786404. PMID 26527831.
- ↑ John AM, Schwartz RA (2016). "Muir-Torre syndrome (MTS): An update and approach to diagnosis and management". J Am Acad Dermatol. 74 (3): 558–66. doi:10.1016/j.jaad.2015.09.074. PMID 26892655.
- ↑ 3.0 3.1 Velter C, Caussade P, Fricker JP, Cribier B (2017). "[Muir-Torre syndrome and Turcot syndrome]". Ann Dermatol Venereol. 144 (8–9): 525–529. doi:10.1016/j.annder.2017.01.017. PMID 28256262.
- ↑ Dipro S, Al-Otaibi F, Alzahrani A, Ulhaq A, Al Shail E (2012). "Turcot syndrome: a synchronous clinical presentation of glioblastoma multiforme and adenocarcinoma of the colon". Case Rep Oncol Med. 2012: 720273. doi:10.1155/2012/720273. PMC 3479943. PMID 23119205.