EpSSG RMS 2005.

Headline: As predicted, the first real progress in three decades has been made in the treatment of high-risk pediatric rhabdomyosarcoma. In this prospective trial, 371 patients were randomized to standard therapy +/- maintenance vinorelbine/cyclophosphamide x 6. Before you google the most recent protocol, here’s a refresher on the current standard therapy: ifosfamide/vincristine/actinomycin D +/- doxorubicin x 9 → surgery +/- radiation. Those receiving additional maintenance therapy had improved rates at 3 years of both event-free (72 → 78%) and overall survival (77 → 87%). Bottom line: You probably won’t use this today, but additional maintenance therapy for pediatric patients with high-risk rhabdo improves overall survival, creating a new standard of care. | Bisogno, ASCO 2018


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