In a non-mesothelioma setting, a patient with SMARCB1-deleted, metastatic, poorly differentiated chordoma was treated with Tazemetostat (EZH2 inhibitor), and had a significant increase in intratumoral and stromal infiltration by immune cells expressing checkpoint regulators PD-1 and LAG-3 [167]. The gene discussed is LAG3; the disease is mesothelioma.