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. Author manuscript; available in PMC: 2021 Apr 1.
Published in final edited form as: Neurosurg Clin N Am. 2020 Jan 25;31(2):289–300. doi: 10.1016/j.nec.2019.11.004

Table 1:

Active immunotherapy trials including patients with chordoma

Trial Registration Number Study Title Type/Phase Agent/Intervention
NCT02383498 A Randomized, Double-Blind, Phase 2 Trial of GI-6301 (Yeast-Brachyury Vaccine) Versus Placebo in Combination With Standard of Care Definitive Radiotherapy in Locally Advanced, Unresectable, Chordoma Phase II Biological: GI-6301 Vaccine (Yeast- Brachyury)
Other: GI-6301 Placebo
Radiation: Radiotherapy
NCT03595228 A Phase 2 Trial of BN-Brachyury and Radiation Therapy in Patients With Advanced Chordoma Phase II Biological: BN-Brachyury (brachyury vaccine) plus radiation
NCT03623854 A Signal Finding Phase 2 Study of Nivolumab (Anti-PD-1; BMS-936558; ONO-4538) and Relatlimab (Anti-LAG-3 Monoclonal Antibody; BMS-986016) in Patients With Advanced Chordoma Phase II Biological: Nivolumab (anti-PD-1)
Biological: Relatlimab (anti-LAG-3)
NCT02989636 Phase I Safety Study of Stereotactic Radiosurgery With Concurrent and Adjuvant PD-1 Antibody Nivolumab in Subjects With Recurrent or Advanced Chordoma Phase I Biological: Nivolumab (anti-PD-1)
Radiation: Stereotactic Radiosurgery
NCT02601950 A Phase II, Multicenter Study of the EZH2 Inhibitor Tazemetostat in Adult Subjects With INI1-Negative Tumors or Relapsed/Refractory Synovial Sarcoma Phase II Drug: Tazemetostat (for chordomas with SMARCB1/INI1 loss)
NCT02601937 A Phase 1 Study of the EZH2 Inhibitor Tazemetostat in Pediatric Subjects With Relapsed or Refractory INI1-Negative Tumors or Synovial Sarcoma Phase I Drug: Tazemetostat (for chordomas with INI1 loss)
NCT02193503 An Open Phase I Clinical Study Assessing Safety and Tolerability of MVX-ONCO-1 in Patients With Solid Tumor Who Are Not/Not Any Longer Amenable to Standard Therapy Phase I Other: MVX-ONCO-1 (autologous tumor cell vaccine+ GM-CSF)
NCT03349983 An Open-label Phase 1 Trial to Evaluate the Safety and Tolerability of a Modified Vaccinia Ankara (MVA) Priming Followed by Fowlpox Booster Vaccines Modified to Express Brachyury and T-cell Costimulatory Molecules (MVA-BN-Brachyury/FPV-Brachyury) Phase I Biological: MVA-BN-Brachyury/FPV-Brachyury (Brachyury vaccine)
NCT03886311 The TNT Protocol: A Phase 2 Study Using Talimogene Laherparepvec,Nivolumab and Trabectedin as First, Second/Third Line Therapy for Advanced Sarcoma, Including Desmoid Tumor and Chordoma Phase II Biological: Talimogene Laherparepvec [IMLYGIC] (oncolytic virus)
Biological: Nivolumab (anti-PD-1)
Drug: Trabectedin (chemotherapy inhibiting DNA binding of a family of transcription factors)
NCT03874455 Tazemetostat Expanded Access Program for Adults With Solid Tumors --- Drug: Tazemetostat (for chordomas with SMARCA4/INI1 loss)
NCT03173950 Phase II trial of the immune checkpoint inhibitor nivolumab in patients with select rare CNS cancers Phase II Biological: Nivolumab (anti-PD-1)
NCT02815995 A phase II multi-arm study to test the efficacy of immunotherapeutic agents in multiple sarcoma subtypes Phase II Biological: Durvalumab (anti-PD-1)
Biological: Tremelimumab (anti-CTLA4)