Reports

Diffuse intrinsic pontine gliomas (DIPGs) are rare, highly aggressive brainstem tumor primarily affecting children. Over 70 % of DIPGs carry somatic mutations in the H3F3A gene encoding histone H3.3, leading to a p.Lys27Met amino acid substitution (1–3). Tumors positive for the mutation are associated with poorer prognosis and diminished survival. Comprehensive whole genome analyses have shown that the H3.3K27M mutation identifies a distinct subgroup of DIPGs that has significant overlap with p53 mutations and PDGFRA amplification (60 % and 40%, respectively) (4, 5). These genetic studies have paved the way for investigations of the pathogenesis and treatment of this rapidly fatal tumor. However tissue access remains a substantial challenge due to the infiltrative nature and sensitive location of the tumor in the brainstem. Key features o