Lurbinectedin in FET-Fused Tumors

Inclusion criteria

• . Age ≥ 10 years.
• . Phase 1: Histological confirmed diagnosis of recurrent or relapsed solid tumor failing primary therapy. Patients must have a known FET fusion (fusion that contains EWSR1, FUS, or TAF15) as documented by next generation sequencing, polymerase chain reaction (PCR) or Fluorescence in situ hybridization (FISH). Patients with a histological diagnosis of Ewing sarcoma with EWS-FLI1 are eligible for dose escalation but not for the exploratory cohort. Please note patients with Ewing sarcoma and alternative FET-ETS fusions (including but not limited to EWS-ERG, EWS-ETV1, EWS-ETV4, EWS-FEV, FUS-ERG, FUS-FEV) are eligible for the exploratory cohort.
• . Phase 2: Histologically confirmed diagnosis of recurrent or relapsed Ewing sarcoma failing primary therapy with confirmation of EWS-FLI1 fusion and breakpoint by Next generation sequencing or PCR or EWSR1 rearrangement confirmed by FISH and available tissue for central confirmation of EWS-FLI1 fusion and breakpoint.
• . Eastern Cooperative Oncology Group (ECOG) Performance Status of 0 or 1 (age \>16 years) or Lansky of at least 70 (age \<16 years).
• . Disease status (baseline imaging must be performed within 28 days of Day 1 of study treatment):
• . Phase 1: At least one site of measurable disease on CT or MRI as defined by RECIST 1.1 OR evaluable disease with at least one site of disease that has not been previously radiated
• . Phase 2: At least one site of measurable disease on CT or MRI as defined by RECIST 1.1
• . Meets organ function requirements as outlined below: