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Review
. 2021 Mar;68(3):e28798.
doi: 10.1002/pbc.28798. Epub 2020 Dec 11.

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

Affiliations
Review

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

Erin R Rudzinski et al. Pediatr Blood Cancer. 2021 Mar.

Abstract

The diagnosis and classification of rhabdomyosarcoma (RMS) has undergone several shifts over the last 30 years. While the main diagnostic categories remained the same, changes in the histologic criteria necessary for diagnosis, as well as varied reliance on immunohistochemical and molecular data over time, have created confusion, particularly regarding how these shifts impacted risk stratification and enrollment onto clinical trials. The goal of this report is to review the evolution and current status of RMS diagnosis, focusing on diagnostic criteria in the Children's Oncology Group (COG), the European Paediatric Soft Tissue Sarcoma Group (EpSSG), and the Cooperative Weichteilsarkom Studiengruppe (CWS). In addition, we emphasize research tools used to classify RMS and address biological questions within current clinical trials run by each group. The INternational Soft Tissue SaRcoma ConsorTium (INSTRuCT) initiative will maximize potential to optimize risk stratification by recognizing and accounting for differences in historical data and current practices.

Keywords: pathology; pediatric; rhabdomyosarcoma.

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References

REFERENCES

    1. Hibbitts E, Chi YY, Hawkins DS, et al. Refinement of risk stratification for childhood rhabdomyosarcoma using FOXO1 fusion status in addition to established clinical outcome predictors: a report from the Children's Oncology Group. Cancer Med. 2019;8:6437-6448.
    1. Skapek SX, Ferrari A, Gupta AA, et al. Rhabdomyosarcoma. Nat Rev Dis Primers. 2019;5. https://doi.org/10.1038/s41572-018-0051-2
    1. Parham DM. Pathologic classification of rhabdomyosarcomas and correlations with molecular studies. Mod Pathol. 2001;14:506-514.
    1. Rudzinski ER, Anderson JR, Hawkins DS, Skapek SX, Parham DM, Teot LA. The World Health Organization classification of skeletal muscle tumors in pediatric rhabdomyosarcoma: a report from the Children's Oncology Group. Arch Pathol Lab Med. 2015;139:1281-1287.
    1. Newton WA Jr, Gehan EA, Webber BL, et al. Classification of rhabdomyosarcomas and related sarcomas. Pathologic aspects and proposal for a new classification-an Intergroup Rhabdomyosarcoma Study. Cancer. 1995;76:1073-1085.

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