Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2020 Jul 2;12(7):1758.
doi: 10.3390/cancers12071758.

Recent Advances and Challenges in the Treatment of Rhabdomyosarcoma

Affiliations
Review

Recent Advances and Challenges in the Treatment of Rhabdomyosarcoma

Shinji Miwa et al. Cancers (Basel). .

Abstract

Rhabdomyosarcoma, the most common soft tissue sarcoma noted in childhood, requires multimodality treatment, including chemotherapy, surgical resection, and/or radiation therapy. The majority of the patients with localized rhabdomyosarcoma can be cured; however, the long-term outcomes in patients with metastatic rhabdomyosarcoma remain poor. The standard chemotherapy regimen for patients with rhabdomyosarcoma is the combination of vincristine, actinomycin, and cyclophosphamide/ifosfamide. In recent clinical trials, modifications of the standard chemotherapy protocol have shown improvements in the outcomes in patients with rhabdomyosarcoma. In various type of malignancies, new treatments, such as molecular targeted drugs and immunotherapies, have shown superior clinical outcomes compared to those of standard treatments. Therefore, it is necessary to assess the benefits of these treatments in patients with rhabdomyosarcoma. Moreover, recent basic and clinical studies on rhabdomyosarcoma have reported promising therapeutic targets and novel therapeutic approaches. This article reviews the recent challenges and advances in the management of rhabdomyosarcoma.

Keywords: chemotherapy; immunotherapy; molecular targeted drug; rhabdomyosarcoma; therapeutic target.

PubMed Disclaimer

Conflict of interest statement

The authors declare no conflict of interest.

References

    1. Ognjanovic S., Linabery A.M., Charbonneau B., Ross J.A. Trends in childhood rhabdomyosarcoma incidence and survival in the United States, 1975–2005. Cancer. 2009;115:4218–4226. doi: 10.1002/cncr.24465. - DOI - PMC - PubMed
    1. Xia S.J., Rajput P., Strzelecki D.M., Barr F.G. Analysis of genetic events that modulate the oncogenic and growth suppressive activities of the PAX3-FKHR fusion oncoprotein. Lab. Investig. 2007;87:318–325. doi: 10.1038/labinvest.3700521. - DOI - PubMed
    1. Barr F.G. Gene fusions involving PAX and FOX family members in alveolar rhabdomyosarcoma. Oncogene. 2001;20:5736–5746. doi: 10.1038/sj.onc.1204599. - DOI - PubMed
    1. Fletcher C.D.M., Bridge J.A., Hogendoorn P., Mertens F. Who Classification of Tumours of the Soft Tissues and Bone. IARC Press; Lyon, France: 2013.
    1. Barr F.G., Qualman S.J., Macris M.H., Melnyk N., Lawlor E.R., Strzelecki D.M., Triche T.J., Bridge J.A., Sorensen P.H. Genetic heterogeneity in the alveolar rhabdomyosarcoma subset without typical gene fusions. Cancer Res. 2002;62:4704–4710. - PubMed

LinkOut - more resources