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).
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).[1]
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[2]
- Hereditary autosomal dominant syndrome
- Considered a subtype of hereditary nonpolyposis colorectal cancer
- 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[3]
- Hereditary autosomal recessive syndrome
- Considered a subtype of hereditary nonpolyposis colorectal cancer
- 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
- ↑ 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.