Journal article
Hamartomatous polyposis syndromes
The Surgical clinics of North America, Vol.88(4), pp.779-817
08/2008
DOI: 10.1016/j.suc.2008.05.002
PMCID: PMC2659506
PMID: 18672141
Abstract
Since the histologic description of the hamartomatous polyp in 1957 by Horrilleno and colleagues, descriptions have appeared of several different syndromes with the propensity to develop these polyps in the upper and lower gastrointestinal tracts. These syndromes include juvenile polyposis, Peutz-Jeghers syndrome, hereditary mixed polyposis syndrome, and the phosphatase and tensin homolog gene (PTEN) hamartoma tumor syndromes (Cowden and Bannayan-Riley-Ruvalcaba syndromes), which are autosomal-dominantly inherited, and Cronkhite-Canada syndrome, which is acquired. This article reviews the clinical aspects, the molecular pathogenesis, the affected organ systems, the risks of cancer, and the management of these hamartomatous polyposis syndromes. Although the incidence of these syndromes is low, it is important for clinicians to recognize these disorders to prevent morbidity and mortality in these patients, and to perform presymptomatic testing in patients at risk.
Details
- Title: Subtitle
- Hamartomatous polyposis syndromes
- Creators
- Daniel Calva - University of Iowa, Roy J. and Lucille A. Carver College of Medicine, 200 Hawkins Drive, Iowa City, IA 52242-2600, USAJames R Howe
- Resource Type
- Journal article
- Publication Details
- The Surgical clinics of North America, Vol.88(4), pp.779-817
- DOI
- 10.1016/j.suc.2008.05.002
- PMID
- 18672141
- PMCID
- PMC2659506
- NLM abbreviation
- Surg Clin North Am
- ISSN
- 0039-6109
- eISSN
- 1558-3171
- Publisher
- United States
- Grant note
- R01 CA098193-01A1 / NCI NIH HHS R01 CA098193 / NCI NIH HHS
- Language
- English
- Date published
- 08/2008
- Academic Unit
- Surgery
- Record Identifier
- 9984051793402771
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