Previous studies of malignant rhabdoid tumors (MRTs) with SMARCB1/SNF5 loss had shown that those tumors upregulate expression the PRC2 complex, containing the essential subunit EZH2, and develop a significant sensitivity EZH2 inhibitors such as EPZ‐6438 (tazemetostat).14, 15, 16 Both RMC cell lines demonstrated similar increases in expression of PRC2 complex subunits including EZH2 but demonstrated a variable response to treatment with single agent EZH2 inhibitors. Here, EZH2 is linked to rhabdoid tumor.