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.
Highlights
Rhabdomyosarcoma (RMS) is a high-grade malignant neoplasm, with a morphologic appearance mimicking that of developing skeletal muscle
The higher 5-year event free survival (EFS) rates for fusion-negative alveolar RMS (aRMS) suggest that this group may unnecessarily receive therapies for high-risk disease, as these outcomes are similar to those of eRMS cases treated with intermediaterisk therapies
Those with “unfavorable risk factors” were randomized to receive differing schedules of window therapy with vincristine and irinotecan (VI) followed by dose-intensive chemotherapy with vincristine/ doxorubicin/cyclophosphamide/ifosfamide/etoposide/irinotecan
Summary
Faculty Reviews are review articles written by the prestigious Members of Faculty Opinions. The articles are commissioned and peer reviewed before publication to ensure that the final, published version is comprehensive and accessible. The reviewers who approved the final version are listed with their names and affiliations. Any comments on the article can be found at the end of the article
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