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Review
. 2020 Jul 8:9:F1000 Faculty Rev-685.
doi: 10.12688/f1000research.22451.1. eCollection 2020.

Recent advances in understanding and managing pediatric rhabdomyosarcoma

Jessica Gartrell  1 , Alberto Pappo  1
Affiliations
Review

Recent advances in understanding and managing pediatric rhabdomyosarcoma

Jessica Gartrell et al. F1000Res. .

Abstract

Rhabdomyosarcoma (RMS) is a high-grade malignant neoplasm, with a morphologic appearance mimicking that of developing skeletal muscle. Over the last 30 years, patient outcomes have improved with the incorporation of multimodal therapies, including chemotherapy, radiation therapy, and surgery. The overall cure rates exceed 70%, with patients who have low-, intermediate-, and high-risk disease experiencing long-term survival rates of >90%, 70%, and <30%, respectively. Historically, RMS was classified according to histology; however, recent advances have revealed new molecular subgroups that allow us to more accurately identify high-, intermediate-, and low-risk disease. In this review, we discuss recent advances made in understanding RMS tumor biology and propose how this understanding can drive a new classification system that can guide clinical approaches for treatment de-escalation in patients with expected favorable outcomes and escalation for those with expected poor outcomes.

Keywords: pediatric; rhabdomyosarcoma; sarcoma.

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Conflict of interest statement

No competing interests were disclosed.No competing interests were disclosed.Competing interests: LM is a member of soft tissue sarcoma committee of the Children’s Oncology Group. No competing interests were disclosed.

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Grants and funding

The author(s) declared that no grants were involved in supporting this work.

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