EZH2 and central nervous system cancer: EZH2 is currently thought to play a pro-tumorigenic role in pediatric gliomas, specifically in diffuse midline gliomas (DMG), where a mutated histone H3K27M is present in 80% of cases, and pharmacological inhibition of EZH2 activity by EZH2 inhibitors results in the inhibition of proliferation in DMG cells [54,55].