Hereditary nonpolyposis colorectal cancer classification

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

Muir–Torre Syndrome^[1]^[2]^[3]

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.

Turcot Syndrome^[3]^[4]

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.

Template:WikiDoc Sources

[pmid26527831-1] Mintsoulis D, Beecker J (2016). “Muir-Torre syndrome”. CMAJ. 188 (5): E95. doi:10.1503/cmaj.150171. PMC 4786404. PMID 26527831.

[pmid26892655-2] 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.

[pmid28256262-3] 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.

[pmid23119205-4] 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.

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