DMG-H3K27a was newly recognized in the 2016 WHO classification of the CNS tumors as "diffuse midline glioma, H3 K27M-mutant.” In WHO CNS5, this tumor is defined as a WHO grade 4 neoplasm characterized by infiltrative and midline-located glioma with loss of H3 p.K28me3, harboring either H3 K27M mutation or H3 K27M-wild type with either of EZHIP overexpression, EGFR amplification, EGFR missense mutation, or EGFR small in-frame insertion [6, 24]. Here, EGFR is linked to central nervous system cancer.