This article is part of the Research Topic The Basic Biology of Pediatric Cancer


Front. Oncol., 17 July 2013 |

Human rhabdomyosarcoma cell lines for rhabdomyosarcoma research: utility and pitfalls

  • 1Department of Pediatrics, Duke University Medical Center, Durham, NC, USA
  • 2Duke University School of Medicine, Durham, NC, USA
  • 3Department of Pediatrics, University of Virginia School of Medicine, Charlottesville, VA, USA
  • 4Laboratory of Pathology, National Cancer Institute, Bethesda, MD, USA
  • 5Department of Pharmacology and Cancer Biology, Duke University Medical Center, Durham, NC, USA

Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma of childhood and adolescence. Despite intergroup clinical trials conducted in Europe and North America, outcomes for high risk patients with this disease have not significantly improved in the last several decades, and survival of metastatic or relapsed disease remains extremely poor. Accrual into new clinical trials is slow and difficult, so in vitro cell-line research and in vivo xenograft models present an attractive alternative for preclinical research for this cancer type. Currently, 30 commonly used human RMS cell lines exist, with differing origins, karyotypes, histologies, and methods of validation. Selecting an appropriate cell line for RMS research has important implications for outcomes. There are also potential pitfalls in using certain cell lines including contamination with murine stromal cells, cross-contamination between cell lines, discordance between the cell line and its associated original tumor, imposter cell lines, and nomenclature errors that result in the circulation of two or more presumed unique cell lines that are actually from the same origin. These pitfalls can be avoided by testing for species-specific isoenzymes, microarray analysis, assays for subtype-specific fusion products, and short tandem repeat analysis.

Keywords: alveolar, embryonal, human cell line, rhabdomyosarcoma, xenograft

Citation: Hinson ARP, Jones R, Crose LES, Belyea BC, Barr FG and Linardic CM (2013) Human rhabdomyosarcoma cell lines for rhabdomyosarcoma research: utility and pitfalls. Front. Oncol. 3:183. doi: 10.3389/fonc.2013.00183

Received: 31 March 2013; Paper pending published: 21 April 2013;
Accepted: 27 June 2013; Published online: 17 July 2013.

Edited by:

Stephen Lessnick, University of Utah, USA

Reviewed by:

Rimas J. Orentas, National Institutes of Health, USA
Peter J. Houghton, Nationwide Children’s Hospital, USA

Copyright: © 2013 Hinson, Jones, Crose, Belyea, Barr and Linardic. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in other forums, provided the original authors and source are credited and subject to any copyright notices concerning any third-party graphics etc.

*Correspondence: Corinne M. Linardic, Departments of Pediatrics, Pharmacology and Cancer Biology, Duke University Medical Center, Box 102382, Durham, NC 27710, USA e-mail: