This Article Full Text Full Text (PDF) eLetters: Submit a response to this article Purchase Article View Shopping Cart Alert me when this article is cited Alert me when eLetters are posted Alert me if a correction is posted Citation Map Services E-mail this article link to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Reprints/Permissions Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Dagher, R. Articles by Helman, L. Search for Related Content PubMed PubMed Citation Articles by Dagher, R. Articles by Helman, L.

Rhabdomyosarcoma: An Overview

Pediatric Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, Maryland, USA

Correspondence: Lee Helman, M.D., Pediatric Oncology Branch, National Cancer Institute, National Institutes of Health, Building 10, Room 13N240, 10 Center Drive, Bethesda, Maryland 20892, USA; Telephone: 301-496-4257; Fax: 301-402-0575; e-mail: helmanl{at}exchange.nih.gov\|[emsp ]\|\|[emsp ]\|

Rhabdomyosarcoma (RMS) is a malignant tumor of mesenchymal origin thought to arise from cells committed to a skeletal muscle lineage. With approximately 250 cases diagnosed yearly in the United States, it is the third most common extracranial solid tumor of childhood after Wilms' tumor and neuroblastoma. Important epidemiologic, biologic, and therapeutic differences have been elucidated within the RMS family. Common sites of primary disease include the head and neck region, genitourinary tract, and extremities. A site-based tumor-nodes-metastasis staging system is being incorporated into use for assessing prognosis and assigning therapy in conjunction with the traditional surgicopathologic clinical grouping system. The development of intensive multimodality treatment protocols tested in large-scale international trials has resulted in significant improvements in outcome, especially for patients with local or locally extensive disease for whom a 60%-70% disease-free survival can be expected. Despite aggressive approaches incorporating surgery, dose-intensive combination chemotherapy, and radiation therapy, the outcome for patients with metastatic disease remains poor. Future challenges include the development of less toxic therapy for patients with localized disease and new approaches for patients with metastatic disease.

Key Words. Rhabdomyosarcoma • Radiation therapy • Adjuvant chemotherapy • Surgery

This article has been cited by other articles:

				M. S. Merchant and C. L. Mackall Current Approach to Pediatric Soft Tissue Sarcomas Oncologist, November 1, 2009; 14(11): 1139 - 1153. [Abstract] [Full Text] [PDF]

				A. D. Miller, M. Steffey, A. Alcaraz, and B. Cooper Embryonal Rhabdomyosarcoma in a Young Maine Coon Cat J. Am. Anim. Hosp. Assoc., January 1, 2009; 45(1): 43 - 47. [Abstract] [Full Text] [PDF]

				J. Gerhart, C. Neely, J. Elder, J. Pfautz, J. Perlman, L. Narciso, K. K. Linask, K. Knudsen, and M. George-Weinstein Cells that express MyoD mRNA in the epiblast are stably committed to the skeletal muscle lineage J. Cell Biol., August 9, 2007; 178(4): 649 - 660. [Abstract] [Full Text] [PDF]

				Atif Ali Ahmed and M. Tsokos Sinonasal Rhabdomyosarcoma in Children and Young Adults International Journal of Surgical Pathology, April 1, 2007; 15(2): 160 - 165. [Abstract] [PDF]

				E. F. Petricoin III, V. Espina, R. P. Araujo, B. Midura, C. Yeung, X. Wan, G. S. Eichler, D. J. Johann Jr., S. Qualman, M. Tsokos, et al. Phosphoprotein Pathway Mapping: Akt/Mammalian Target of Rapamycin Activation Is Negatively Associated with Childhood Rhabdomyosarcoma Survival Cancer Res., April 1, 2007; 67(7): 3431 - 3440. [Abstract] [Full Text] [PDF]

				G. Vassal, D. Couanet, E. Stockdale, A. Geoffray, B. Geoerger, D. Orbach, F. Pichon, J. C. Gentet, S. Picton, C. Bergeron, et al. Phase II Trial of Irinotecan in Children With Relapsed or Refractory Rhabdomyosarcoma: A Joint Study of the French Society of Pediatric Oncology and the United Kingdom Children's Cancer Study Group J. Clin. Oncol., February 1, 2007; 25(4): 356 - 361. [Abstract] [Full Text] [PDF]

				J. Gerhart, J. Elder, C. Neely, J. Schure, T. Kvist, K. Knudsen, and M. George-Weinstein MyoD-positive epiblast cells regulate skeletal muscle differentiation in the embryo J. Cell Biol., October 23, 2006; 175(2): 283 - 292. [Abstract] [Full Text] [PDF]

				R. Saab, J. L. Bills, A. P. Miceli, C. M. Anderson, J. D. Khoury, D. W. Fry, F. Navid, P. J. Houghton, and S. X. Skapek Pharmacologic inhibition of cyclin-dependent kinase 4/6 activity arrests proliferation in myoblasts and rhabdomyosarcoma-derived cells Mol. Cancer Ther., May 1, 2006; 5(5): 1299 - 1308. [Abstract] [Full Text] [PDF]

				L. H. Baker Medical and Pediatric Oncology, Not Adult and Pediatric Oncology J. Clin. Oncol., June 20, 2005; 23(18): 4003 - 4005. [Full Text] [PDF]

				B. L. Dake, M. Boes, L. A. Bach, and R. S. Bar Effect of an Insulin-Like Growth Factor Binding Protein Fusion Protein on Thymidine Incorporation in Neuroblastoma and Rhabdomyosarcoma Cell Lines Endocrinology, July 1, 2004; 145(7): 3369 - 3374. [Abstract] [Full Text] [PDF]

				J. Gerhart, B. Bast, C. Neely, S. Iem, P. Amegbe, R. Niewenhuis, S. Miklasz, P. F. Cheng, and M. George-Weinstein MyoD-positive myoblasts are present in mature fetal organs lacking skeletal muscle J. Cell Biol., October 29, 2001; 155(3): 381 - 392. [Abstract] [Full Text] [PDF]

				I. Petak, L. Douglas, D. M. Tillman, R. Vernes, and J. A. Houghton Pediatric Rhabdomyosarcoma Cell Lines Are Resistant to Fas-induced Apoptosis and Highly Sensitive to TRAIL-induced Apoptosis Clin. Cancer Res., October 1, 2000; 6(10): 4119 - 4127. [Abstract] [Full Text]

				H. Hahn, L. Wojnowski, K. Specht, R. Kappler, J. Calzada-Wack, D. Potter, A. Zimmer, U. Muller, E. Samson, L. Quintanilla-Martinez, et al. Patched Target Igf2 Is Indispensable for the Formation of Medulloblastoma and Rhabdomyosarcoma J. Biol. Chem., September 8, 2000; 275(37): 28341 - 28344. [Abstract] [Full Text] [PDF]