Identification of distinct genetic and epigenetic profiles in various
neuroepithelial tumors has improved the classification and uncovered novel diagnostic, prognostic, and predictive molecular
biomarkers for improved prediction of treatment response and outcome. Especially, in pediatric high-grade
brain tumors, such as diffuse midline
glioma, H3K27M-altered and posterior fossa group A-
ependymoma, epigenetic changes predominate, along with changes in expression of known oncogenes and tumor suppressor genes induced by histone modifications and DNA methylation. The precise role of epigenetic abnormalities is important for understanding
tumorigenesis and the establishment of
brain tumor treatment strategies. Using powerful epigenetic-based
therapies for
cancer cells, the aberrantly regulated epigenome can be restored to a more normal state through epigenetic reprogramming. Combinations of agents targeting DNA methylation and/or other epigenetic modifications may be a promising
cancer treatment. Therefore, the integration of multi-omics data including epigenomics is now important for classifying
primary brain tumors and predicting their
biological behavior. Recent advances in molecular genetics and epigenetic integrated diagnostics of
brain tumors influence new strategies for targeted
therapy.