Clinicopathology of diffuse intrinsic pontine glioma and its redefined genomic and epigenomic landscape

Diffuse intrinsic pontine glioma (DIPG) is one of the most lethal pediatric central nervous system (CNS) cancers. Recently, a surge in molecular studies of DIPG has occurred, in large part due to the increased availability of tumor tissue through donation of post-mortem specimens. These new discoveries have established DIPGs as biologically distinct from adult gliomas, harboring unique genomic aberrations. Mutations in histone encoding genes are shown to be associated with >70% of DIPG cases. However, the exact molecular mechanisms of the tumorigenicity of these mutations remain elusive. Understanding the driving mutations and genomic landscape of DIPGs can now guide the development of targeted therapies for this incurable childhood cancer.