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Hamartomatous polyposis syndromes.

Abstract

Hamartomatous polyposis syndromes are a diverse group of inherited conditions grouped together because they exhibit hamartomatous rather than epithelial polyp histology. Each syndrome exhibits characteristic polyp histology, gastrointestinal polyp distribution, gastrointestinal cancer risks, extra-intestinal benign findings and often extra-intestinal cancer risks. Identifying individuals at risk for these syndromes and accurately defining the precise diagnosis are necessary for planning surveillance and management in order to prevent the benign and malignant complications. Characteristic syndrome features including gastrointestinal findings, pathology, genetics, and management options for the three most common hamartomatous polyposis syndromes, Peutz-Jeghers syndrome, PTEN hamartoma tumour syndrome, and juvenile polyposis will be presented in this review.

Authors+Show Affiliations

Huntsman Cancer Institute, 2000 Circle of Hope, Salt Lake City, UT 84112-5550, USA. amanda.gammon@hci.utah.eduNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't
Review

Language

eng

PubMed ID

19414148

Citation

Gammon, Amanda, et al. "Hamartomatous Polyposis Syndromes." Best Practice & Research. Clinical Gastroenterology, vol. 23, no. 2, 2009, pp. 219-31.
Gammon A, Jasperson K, Kohlmann W, et al. Hamartomatous polyposis syndromes. Best Pract Res Clin Gastroenterol. 2009;23(2):219-31.
Gammon, A., Jasperson, K., Kohlmann, W., & Burt, R. W. (2009). Hamartomatous polyposis syndromes. Best Practice & Research. Clinical Gastroenterology, 23(2), pp. 219-31. doi:10.1016/j.bpg.2009.02.007.
Gammon A, et al. Hamartomatous Polyposis Syndromes. Best Pract Res Clin Gastroenterol. 2009;23(2):219-31. PubMed PMID: 19414148.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Hamartomatous polyposis syndromes. AU - Gammon,Amanda, AU - Jasperson,Kory, AU - Kohlmann,Wendy, AU - Burt,Randall W, PY - 2009/5/6/entrez PY - 2009/5/6/pubmed PY - 2009/7/17/medline SP - 219 EP - 31 JF - Best practice & research. Clinical gastroenterology JO - Best Pract Res Clin Gastroenterol VL - 23 IS - 2 N2 - Hamartomatous polyposis syndromes are a diverse group of inherited conditions grouped together because they exhibit hamartomatous rather than epithelial polyp histology. Each syndrome exhibits characteristic polyp histology, gastrointestinal polyp distribution, gastrointestinal cancer risks, extra-intestinal benign findings and often extra-intestinal cancer risks. Identifying individuals at risk for these syndromes and accurately defining the precise diagnosis are necessary for planning surveillance and management in order to prevent the benign and malignant complications. Characteristic syndrome features including gastrointestinal findings, pathology, genetics, and management options for the three most common hamartomatous polyposis syndromes, Peutz-Jeghers syndrome, PTEN hamartoma tumour syndrome, and juvenile polyposis will be presented in this review. SN - 1532-1916 UR - https://www.unboundmedicine.com/medline/citation/19414148/Hamartomatous_polyposis_syndromes_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S1521-6918(09)00030-4 DB - PRIME DP - Unbound Medicine ER -