Alisertib (MLN8237), an Aurora K inhibitor, has shown early evidence of remarkable activity in the treatment of ATRT patients [32], while CDK, MEK, and EZH2 inhibitors have been shown to be effective in restricting tumor cell growth in rhabdoid tumor cell line and xenograft-based models [33-36]. This evidence concerns the gene EZH2 and atypical teratoid rhabdoid tumor.