| Hamartomatous polyposis syndromes. | |
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MedLine Citation:
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PMID: 18672141 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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. |
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Authors:
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Daniel Calva; James R Howe |
Publication Detail:
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Type: Journal Article; Review |
Journal Detail:
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Title: The Surgical clinics of North America Volume: 88 ISSN: 0039-6109 ISO Abbreviation: Surg. Clin. North Am. Publication Date: 2008 Aug |
Date Detail:
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Created Date: 2008-08-01 Completed Date: 2008-09-30 Revised Date: 2011-09-26 |
Medline Journal Info:
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Nlm Unique ID: 0074243 Medline TA: Surg Clin North Am Country: United States |
Other Details:
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Languages: eng Pagination: 779-817, vii Citation Subset: AIM; IM |
Affiliation:
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University of Iowa, Roy J. and Lucille A. Carver College of Medicine, 200 Hawkins Drive, Iowa City, IA 52242-2600, USA. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Diagnosis, Differential Digestive System Surgical Procedures / methods* Endoscopy, Gastrointestinal Genetic Predisposition to Disease* Humans Peutz-Jeghers Syndrome* / diagnosis, genetics, surgery Prognosis |
| Grant Support | |
ID/Acronym/Agency:
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R01 CA098193-01A1/CA/NCI NIH HHS |
| Comments/Corrections | |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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