Clinical trials of tazemetostat in combination with nivolumab and ipilimumab in children with INI1-negative tumours, nivolumab and ipilimumab in children and young adults with INI1-negative tumours, and a combination of atezolizumab with the anti-TIGIT tiragolumab in INI1-deficient tumours, are currently ongoing and include patients with ES (Table 1). The gene discussed is SMARCB1; the disease is epithelioid sarcoma.