A phase II trial (NCT04416568) is currently recruiting patients to investigate the combination of nivolumab (anti-PD1) with ipilimumab (anti-CTLA4) in children and young adults with SMARCB1-deficient tumours, including RTs, epithelioid sarcomas, and chordomas [58]. Here, CTLA4 is linked to epithelioid sarcoma.