Background: Thephase II BERNIE study evaluated the role of bevacizumab as part of the multi-modality treatment of children and adolescents with metastatic RMS/NRSTS. Methods: Eligible patients aged ≥6 months and <18 years were randomized to receive 18 months of induction chemotherapy (4x 21-day cycles of IVADo [ifosfamide, vincristine, actinomycin-D, doxorubicin] + 5x cycles of IVA; ± bevacizumab 7.5mg/kg q3w) with local therapy, followed by maintenance chemotherapy (12x 28-day cycles of cyclophosphamide + vinorelbine; ± bevacizumab 5.0mg/kg q2w). The primary objective was independent review committee (IRC)-assessed event-free survival (EFS). Secondary objectives included: pharmacokinetics; safety; overall response rate (ORR); and overall survival (OS). Results: 154 patients were randomized to receive chemotherapy (n=80) or bevacizumab plus chemotherapy (n=74). Baseline characteristics were balanced. The cut-off for the results shown below was May 31, 2015, 19 months after the last patient was enrolled. Conclusions: The addition of bevacizumab to the chemotherapy backbone appeared tolerable and enhanced ORR in children and adolescents with metastatic RMS/NRSTS, but the primary and secondary efficacy endpoints were not met. Long-term observation continues. Clinical trial information: NCT00643565