| A nation-wide study comparing sporadic and familial adenomatous polyposis-related desmoid-type fibromatoses. | |
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MedLine Citation:
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PMID: 20830713 Owner: NLM Status: In-Data-Review |
Abstract/OtherAbstract:
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Desmoid-type fibromatoses are neoplasms of fibroblastic origin, occurring sporadically or associated with familial adenomatous polyposis (FAP) coli. By comparing sporadic and FAP-associated desmoid-type fibromatoses, we tried to identify clinical characteristics, which may indicate FAP. Histopathology data of all Dutch patients with desmoid-type fibromatoses diagnosed between 1999 and 2009 were retrieved from PALGA, the nation-wide network and registry of histopathology in the Netherlands. For calculation of incidence rates, person-years from the general matched population were used. Based on polyp counts in pathological records, the cohort was divided into a FAP group and a non-FAP group. Patient- and tumor characteristics were compared between the two groups. A total number of 519 patients older than 10 years with a confirmed diagnosis of desmoid-type fibromatoses were included. Thirty-nine (7.5%) desmoid patients were documented of having FAP. The incidences of sporadic and FAP-related desmoid-type fibromatoses were 3.42 and 2,784 per million person-years, respectively. The majority of FAP patients developed desmoid-type fibromatoses after the diagnosis of FAP. Having FAP was associated with male gender [odds ratio (OR) 2.0, p = 0.034], desmoid diagnosis at an earlier age (mean 36 vs. 42 years, p = 0.031), and desmoid localization intra-abdominally (OR 18.9, p ≤ 0.001) or in the abdominal wall (OR 4.8, p ≤ 0.001), compared to extra-abdominal desmoid localization. In conclusion, patients with desmoid-type fibromatoses are at risk of underlying FAP. Especially cases with desmoid localization intra-abdominal or in the abdominal wall, and all patients younger than 60 years, have a substantial increased risk and should be referred for colonoscopy. |
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Authors:
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Marry H Nieuwenhuis; Mariel Casparie; Lisbeth M H Mathus-Vliegen; Olaf M Dekkers; Pancras C W Hogendoorn; Hans F A Vasen |
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Publication Detail:
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Type: Journal Article Date: 2010-11-03 |
Journal Detail:
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Title: International journal of cancer. Journal international du cancer Volume: 129 ISSN: 1097-0215 ISO Abbreviation: Int. J. Cancer Publication Date: 2011 Jul |
Date Detail:
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Created Date: 2011-04-26 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 0042124 Medline TA: Int J Cancer Country: United States |
Other Details:
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Languages: eng Pagination: 256-61 Citation Subset: IM |
Copyright Information:
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Copyright © 2010 UICC. |
Affiliation:
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The Netherlands Foundation for the Detection of Hereditary Tumours, Leiden, The Netherlands. |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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