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  • Between Bedside and Bench
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Muscling in: Uncovering the origins of rhabdomyosarcoma

Nature Medicine volume 16, pages 171–173 (2010)Cite this article

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Rhabdomyosarcoma, an aggressive childhood cancer with a poor prognosis, is the most common soft-tissue sarcoma in children and adolescents1. Rhabdomyosarcomas are generally thought of as skeletal muscle tumors, in large part because they typically arise in or near muscle beds and show features of myogenic differentiation. These muscle-like features may vary from small round cells to large, oblong cells that have a strap-like appearance and, occasionally, cross-striations and multinucleation1. Yet, rhabdomyosarcomas also can arise at body sites that normally lack skeletal muscle, such as the biliary and the genitourinary tract, and recent clinical observations highlight important questions about the origins of these tumors2,3. Clinical reports from Lisboa et al.2 and Shinkoda et al.3 describe three atypical cases of alveolar rhabdomyosarcoma, one of the two major rhabdomyosarcoma subtypes, in which the individuals presented with widespread tumor infiltration in the bone marrow, reminiscent of acute leukemia, and an absence of any detectable primary tumor.

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Figure 1: Model of skeletal myogenesis, with possible cellular origins of rhabdomyosarcoma as suggested experimentally.

Katie Vicari

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Authors and Affiliations

  1. Department of Stem Cell and Regenerative Biology, Simone Hettmer and Amy J. Wagers are at the Howard Hughes Medical Institute, Harvard University, Harvard Stem Cell Institute, and Joslin Diabetes Center, Cambridge, Massachusetts, USA.,

    Simone Hettmer & Amy J Wagers

  2. Simone Hettmer is also in the Department of Pediatric Oncology, and the Division of Pediatric Hematology/Oncology, Dana Farber Cancer Institute, Children's Hospital, Boston, Massachusetts, USA.,

    Simone Hettmer

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  1. Simone Hettmer
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  2. Amy J Wagers
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Correspondence to Amy J Wagers.

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Hettmer, S., Wagers, A. Muscling in: Uncovering the origins of rhabdomyosarcoma. Nat Med 16, 171–173 (2010). https://doi.org/10.1038/nm0210-171

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