Pediatric adenomatous polyposis syndromes: an update
- PMID: 17511923
- DOI: 10.1007/s11894-007-0025-0
Pediatric adenomatous polyposis syndromes: an update
Abstract
Juvenile polyps are a common finding in the pediatric population. In contrast, colon adenomas, which are viewed as dysplastic precancerous lesions, are found sporadically in late adulthood. Adenomas in children and young adults are highly unusual and suggest one of several forms of inherited colorectal cancer. These disorders show a predilection to early adenoma formation and can present in childhood. Familial adenomatous polyposis and Lynch syndrome are autosomal dominant, often with involvement of multiple family members, or can be seen in an individual arising from a de novo mutation. The most recently described adenomatous polyposis syndrome, MutYH-associated polyposis, is autosomal recessive, requiring an inherited mutation from each parent. All three adenomatous polyposis disorders can display tremendous variation in expression, even within the same family, and can have a common overlapping phenotype. These disorders require regular medical care to minimize cancer risk in the digestive tract and in other organ systems.
Similar articles
-
Hereditary Colorectal Cancer Syndromes.Surg Clin North Am. 2017 Jun;97(3):605-625. doi: 10.1016/j.suc.2017.01.009. Surg Clin North Am. 2017. PMID: 28501250 Review.
-
ACMG technical standards and guidelines for genetic testing for inherited colorectal cancer (Lynch syndrome, familial adenomatous polyposis, and MYH-associated polyposis).Genet Med. 2014 Jan;16(1):101-16. doi: 10.1038/gim.2013.166. Epub 2013 Dec 5. Genet Med. 2014. PMID: 24310308
-
Clinical characterization and mutation spectrum in Hispanic families with adenomatous polyposis syndromes.Fam Cancer. 2013 Sep;12(3):555-62. doi: 10.1007/s10689-013-9617-z. Fam Cancer. 2013. PMID: 23460355 Free PMC article.
-
Clinical utility gene card for: MUTYH-associated polyposis (MAP), autosomal recessive colorectal adenomatous polyposis, multiple colorectal adenomas, multiple adenomatous polyps (MAP) - update 2012.Eur J Hum Genet. 2013 Jan;21(1). doi: 10.1038/ejhg.2012.163. Epub 2012 Aug 8. Eur J Hum Genet. 2013. PMID: 22872101 Free PMC article. No abstract available.
-
[Hereditary colorectal cancer; familial adenomatous polyposis, MUTYH associated polyposis and Lynch syndrome].Nihon Rinsho. 2011 Apr;69 Suppl 3:59-64. Nihon Rinsho. 2011. PMID: 22213932 Review. Japanese. No abstract available.
Cited by
-
Pediatric manifestations of Lynch Syndrome: A single center experience.J Pediatr Surg Case Rep. 2022 Nov;86:102431. doi: 10.1016/j.epsc.2022.102431. Epub 2022 Aug 26. J Pediatr Surg Case Rep. 2022. PMID: 36313796 Free PMC article.
References
Publication types
MeSH terms
Substances
LinkOut - more resources
Research Materials