BET inhibitors (BETi), such as JQ1, have demonstrated antitumor activity in several fusion-driven sarcomas, including rhabdomyosarcoma (RMS) and Ewing sarcoma (ES), by disrupting BRD4-dependent transcriptional activity, reducing oncogene expression (e.g., MYC), and impairing super-enhancer function [[18], [19], [20], [21]]. This evidence concerns the gene MYC and Ewing sarcoma.