
Health Dictionary
Reliable, easy-to-read patient information on common medical conditions β written in plain language, free of jargon, and grounded in current evidence. Browse the most frequent diagnoses, understand what they mean for everyday life, and learn which steps and questions help you have a focused conversation with your doctor.
Hereditary nonpolyposis colorectal cancer classification
Introduction
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 associated with other cancers). Other variants such as Muir-Torre syndrome and Turcot syndrome are considered subtypes of hereditary nonpolyposis colorectal cancer.
Classification
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
MuirβTorre 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.
- 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
References
- β Mintsoulis D, Beecker J (2016). “Muir-Torre syndrome”. CMAJ. 188 (5): E95. doi:10.1503/cmaj.150171. PMCΒ 4786404. PMIDΒ 26527831.
- β John AM, Schwartz RA (2016). “Muir-Torre syndrome (MTS): An update and approach to diagnosis and management”. J Am Acad Dermatol. 74 (3): 558β66. doi:10.1016/j.jaad.2015.09.074. PMIDΒ 26892655.
- β 3.0 3.1 Velter C, Caussade P, Fricker JP, Cribier B (2017). “[Muir-Torre syndrome and Turcot syndrome]”. Ann Dermatol Venereol. 144 (8β9): 525β529. doi:10.1016/j.annder.2017.01.017. PMIDΒ 28256262.
- β 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. PMCΒ 3479943. PMIDΒ 23119205.
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]
