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Volume 2012, Article ID 406239, 13 pages
Review Article

Embryonic Signaling Pathways and Rhabdomyosarcoma: Contributions to Cancer Development and Opportunities for Therapeutic Targeting

1Department of Pediatrics, Duke University Medical Center, Durham, NC 27710, USA
2Department of Pediatrics, University of Virginia, Charlottesville, VA 22908, USA
3Department of Pharmacology & Cancer Biology, Duke University Medical Center, Durham, NC 27710, USA
4Department of Radiation Oncology, Duke University Medical Center, Durham, NC 27710, USA

Received 15 October 2011; Accepted 17 January 2012

Academic Editor: Ivo Leuschner

Copyright © 2012 Brian Belyea 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.


Rhabdomyosarcoma is the most common soft tissue sarcoma of childhood and adolescence, accounting for approximately 7% of childhood cancers. Current therapies include nonspecific cytotoxic chemotherapy regimens, radiation therapy, and surgery; however, these multimodality strategies are unsuccessful in the majority of patients with high-risk disease. It is generally believed that these tumors represent arrested or aberrant skeletal muscle development, and, accordingly, developmental signaling pathways critical to myogenesis such as Notch, WNT, and Hedgehog may represent new therapeutic targets. In this paper, we summarize the current preclinical studies linking these embryonic pathways to rhabdomyosarcoma tumorigenesis and provide support for the investigation of targeted therapies in this embryonic cancer.