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Hamartomatous polyposis syndromes.
Surg Clin North Am 2008; 88(4):779-817, viiSC

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.

Authors+Show Affiliations

University of Iowa, Roy J. and Lucille A. Carver College of Medicine, 200 Hawkins Drive, Iowa City, IA 52242-2600, USA.No affiliation info available

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

18672141

Citation

Calva, Daniel, and James R. Howe. "Hamartomatous Polyposis Syndromes." The Surgical Clinics of North America, vol. 88, no. 4, 2008, pp. 779-817, vii.
Calva D, Howe JR. Hamartomatous polyposis syndromes. Surg Clin North Am. 2008;88(4):779-817, vii.
Calva, D., & Howe, J. R. (2008). Hamartomatous polyposis syndromes. The Surgical Clinics of North America, 88(4), pp. 779-817, vii. doi:10.1016/j.suc.2008.05.002.
Calva D, Howe JR. Hamartomatous Polyposis Syndromes. Surg Clin North Am. 2008;88(4):779-817, vii. PubMed PMID: 18672141.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Hamartomatous polyposis syndromes. AU - Calva,Daniel, AU - Howe,James R, PY - 2008/8/2/pubmed PY - 2008/10/1/medline PY - 2008/8/2/entrez SP - 779-817, vii JF - The Surgical clinics of North America JO - Surg. Clin. North Am. VL - 88 IS - 4 N2 - 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. SN - 0039-6109 UR - https://www.unboundmedicine.com/medline/citation/18672141/Hamartomatous_polyposis_syndromes_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0039-6109(08)00069-8 DB - PRIME DP - Unbound Medicine ER -