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Sarcoma
Volume 2012 (2012), Article ID 152382, 9 pages
http://dx.doi.org/10.1155/2012/152382
Review Article

At-Risk Populations for Osteosarcoma: The Syndromes and Beyond

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
3Division of Genetic Epidemiology, Department of Biomedical Informatics, University of Utah School of Medicine, Salt Lake City, Utah 84108, USA
4Division of Pediatric Hematology Oncology, Department of Pediatrics, The University of Utah, Salt Lake City, UT 84112, USA

Received 15 October 2011; Accepted 17 November 2011

Academic Editor: Alessandro Gronchi

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

Osteosarcoma is the most common primary malignancy of bone. Most cases are sporadic without a known genetic or environmental cause. Heritable genetic predisposition syndromes are associated with a small percentage of osteosarcomas. Study of these rare disorders has provided insight into the molecular pathogenesis of osteosarcoma. Screening of at-risk families and surveillance of affected individuals for these syndromes may permit earlier diagnosis and more effective treatment of osteosarcoma in these populations. This paper reviews the genetic and clinical features of the known osteosarcoma predisposition syndromes.