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Sarcoma
Volume 2012, Article ID 726537, 11 pages
http://dx.doi.org/10.1155/2012/726537
Review Article

Extra-Abdominal Desmoid Tumors Associated with Familial Adenomatous Polyposis

1Department of Orthopaedics and Huntsman Cancer Institute, The University of Utah, Salt Lake City, UT 84112, USA
2Sarcoma Services, Center for Children, Huntsman Cancer Institute, The University of Utah, Salt Lake City, UT 84112, USA
3Department of Medicine and Huntsman Cancer Institute, The University of Utah, Salt Lake City, UT 84112, USA

Received 2 February 2012; Accepted 30 March 2012

Academic Editor: Leslie G. Dodd

Copyright © 2012 George T. Calvert et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Extra-abdominal desmoid tumors are a significant cause of morbidity in patients with familial adenomatous polyposis syndrome. Understanding of the basic biology and natural history of these tumors has increased substantially over the past decade. Accordingly, medical and surgical management of desmoid tumors has also evolved. This paper analyzes recent evidence pertaining to the epidemiology, molecular biology, histopathology, screening, and treatment of extra-abdominal desmoid tumors associated with familial adenomatous polyposis syndrome.