Molecular genetic features of pediatric gliomas

Abstract

Gliomas are the most common central nervous system tumors demonstrating an extremely broad range of clinical behavior. Over last few decades the understanding of molecular genetic mechanisms of tumor initiation and progression increased significantly. Furthermore, the identification of prognostic and predictive biomarkers aids the development of personalized and risk-adapted therapeutic approaches. In this review, we summarize the molecular findings in pediatric gliomas, both low and high grade (LGG and HGG), focusing on recurrent somatic mutations. There are nucleotide substitutions inBRAF, H3F3A, Hist1H3B/С, IDH1/2genes,BRAFandNTRK1/2/3fusions, andCDKN2A/Bcopy-number aberrations, known to be clinically relevant in the prognosis defining or predicting the efficacy of targeted therapy. We also describe how these findings could pave the way towards the novel genetic classification and risk-group stratification for pediatric patients with glial tumors.