This makes EZH2 a prime therapeutic target, and several compounds have been developed since EZH2 inhibitor Tazemetostat was FDA-approved for advanced epithelioid sarcoma as well as relapsed or refractory follicular lymphoma, with several ongoing phase 1 and 2 clinical trials investigating similar drugs, such as SHR2554 and CPI-1205 (or lirametostat) in small intestine neuroendocrine tumors and relapsed or refractory B-cell/T-cell and Hodgkin’s lymphomas, respectively [78,79,80]. Here, EZH2 is linked to epithelioid sarcoma.