PRIMARY OBJECTIVES:~I. To assess the clinical benefit of the combination of nivolumab and relatlimab in patients with advanced chordomas by objective response rate (ORR).~SECONDARY OBJECTIVES:~I. Ascertain the safety of nivolumab in combination with relatlimab in subjects with metastatic or locally advanced/unresectable chordoma by the frequency of adverse events (AEs).~II. Assess the clinical benefit of the combination of nivolumab and relatlimab in patients with advanced chordomas by progression free survival (PFS).~EXPLORATORY OBJECTIVES:~I. Compare response rate (RR) and clinical benefit rate (CBR) in patients whose tumors are PD-L1+ and PD-L1- at baseline.~II. Compare RR and CBR in patients whose tumors are LAG-3+ and LAG-3- at baseline.~III. In the patients who are PD-L1 positive, compare RR and CBR in patients with 1% and 5% tumor membrane staining.~III. Determine the response to treatment based on the baseline mutation load. IV. Determine the ORR and CBR via Choi criteria.~OUTLINE:~Participants receive nivolumab intravenously (IV) over 60 minutes and relatlimab via infusion over 60 minutes on day 1. Courses repeat every 28 days for up to 2 years in the absence of disease progression or unacceptable toxicity.~After completion of study treatment, participants are followed up within 100 days.
This phase II trial studies how well nivolumab and relatlimab work in treating participants with chordoma that has spread to other places in the body. Monoclonal antibodies, such as nivolumab and relatlimab, may interfere with the ability of tumor cells to grow and spread.