Recent advances in understanding and managing pediatric rhabdomyosarcoma

F1000Research
Jessica Gartrell, Alberto Pappo

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.

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Citations

May 8, 2021·Cell Death & Disease·Tatiana TiagoSerena Carra
Jul 31, 2021·Frontiers in Cell and Developmental Biology·Virginie Barbet, Laura Broutier
Aug 1, 2021·Cancer Chemotherapy and Pharmacology·Marielle HuotNoël J M Raynal

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Methods Mentioned

BETA
X-ray
xenograft

Clinical Trials Mentioned

NCT02567435
NCT00075582
NCT00354835
NCT00003958
NCT01055314
NCT01222715
NCT02095132

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