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
. 2022 Apr;69(4):e29511.
doi: 10.1002/pbc.29511. Epub 2022 Feb 7.

An update on rhabdomyosarcoma risk stratification and the rationale for current and future Children's Oncology Group clinical trials

Josephine H Haduong  1 , Christine M Heske  2 , Wendy Allen-Rhoades  3 , Wei Xue  4 , Lisa A Teot  5 , David A Rodeberg  6 , Sarah S Donaldson  7 , Aaron Weiss  8 , Douglas S Hawkins  9 , Rajkumar Venkatramani  10
Affiliations
Review

An update on rhabdomyosarcoma risk stratification and the rationale for current and future Children's Oncology Group clinical trials

Josephine H Haduong et al. Pediatr Blood Cancer. 2022 Apr.

Abstract

Children and adolescents with rhabdomyosarcoma (RMS) comprise a heterogeneous population with variable overall survival rates ranging between approximately 6% and 100% depending on defined risk factors. Although the risk stratification of patients has been refined across five decades of collaborative group studies, molecular prognostic biomarkers beyond FOXO1 fusion status have yet to be incorporated prospectively in upfront risk-based therapy assignments. This review describes the evolution of risk-based therapy and the current risk stratification, defines a new risk stratification incorporating novel biomarkers, and provides the rationale for the current and upcoming Children's Oncology Group RMS studies.

Keywords: MYOD1; TP53; rhabdomyosarcoma.

PubMed Disclaimer

Conflict of interest statement

CONFLICT OF INTEREST STATEMENT

The authors have no conflicts of interest to disclose.

Figures

Figure 1:
Figure 1:
Outcomes of 2157 patients enrolled on IRS V (D9602, D9803, and D9802) and COG ARST (ARST0331, ARST0531, ARST0431 and ARST08P1) studies based on clinical factors. 1A: EFS and OS by age at diagnosis; 1B: EFS and OS by tumor site; 1C: EFS and OS by tumor size
Figure 2:
Figure 2:
Outcomes of 2157 patients enrolled on IRS V (D9602, D9803 and D9602) and COG ARST (ARST0331, ARST0531, ARST0431 and ARST08P1) studies based on clinical factors. 2A: EFS and OS by nodal status; 2B: EFS and OS by Clinical Group; 2C: EFS and OS by histology
See this image and copyright information in PMC

References

    1. Howlader N NA KM, Miller D, Bishop K, Kosary CL, Yu M, Ruhl J, Tatlovich Z, Mariotto A, Lewis DR, Chen HS, Feuer EJ, Cronin KA (eds). SEER Cancer Statistics Review, 1975–2014. In: Institute NC, editor. 2016 ed. https://seer.cancer.gov/csr/1975_2014/2016.
    1. Shern JF, Yohe ME, Khan J. Pediatric Rhabdomyosarcoma. Crit Rev Oncog. 2015;20(3–4):227–43. doi:10.1615/critrevoncog.2015013800 - DOI - PMC - PubMed
    1. Skapek SX, Ferrari A, Gupta AA, et al. Rhabdomyosarcoma. Nat Rev Dis Primers. Jan 7 2019;5(1):1. doi:10.1038/s41572-018-0051-2 - DOI - PMC - PubMed
    1. Raney RB, Maurer HM, Anderson JR, et al. The Intergroup Rhabdomyosarcoma Study Group (IRSG): Major Lessons From the IRS-I Through IRS-IV Studies as Background for the Current IRS-V Treatment Protocols. Sarcoma. 2001;5(1):9–15. doi:10.1080/13577140120048890 - DOI - PMC - PubMed
    1. Rudzinski ER, Kelsey A, Vokuhl C, et al. Pathology of childhood rhabdomyosarcoma: A consensus opinion document from the Children’s Oncology Group, European Paediatric Soft Tissue Sarcoma Study Group, and the Cooperative Weichteilsarkom Studiengruppe. Pediatr Blood Cancer. Mar 2021;68(3):e28798. doi:10.1002/pbc.28798 - DOI - PubMed

Publication types