LAUSR

Simple Summary Recent research has enhanced our understanding of the diverse biological processes that occur in pediatric gliomas; and molecular genetic analysis has become essential to diagnose and treat these conditions. Because targetable molecular aberrations can be detected in pediatric gliomas, identifying these aberrations is very important. This review provides an overview of pediatric gliomas, and describes recent developments made in strategies for their diagnosis and treatment. Additionally, it presents a current picture of pediatric gliomas in light of advances in molecular genetics, and describes the current scientific progress in gliomas’ treatment using information from recently completed and ongoing clinical trials. The era of incorporating molecular genetic analysis into clinical practice is emerging. Abstract Recent research has promoted elucidation of the diverse biological processes that occur in pediatric central nervous system (CNS) tumors. Molecular genetic analysis is essential not only for proper classification, but also for monitoring biological behavior and clinical management of tumors. Ever since the 2016 World Health Organization classification of CNS tumors, molecular profiling has become an indispensable step in the diagnosis, prediction of prognosis, and treatment of pediatric as well as adult CNS tumors. These molecular data are changing diagnosis, leading to new guidelines, and offering novel molecular targeted therapies. The Consortium to Inform Molecular and Practical Approaches to CNS Tumor Taxonomy (cIMPACT-NOW) makes practical recommendations using recent advances in CNS tumor classification, particularly in molecular discernment of these neoplasms as morphology-based classification of tumors is being replaced by molecular-based classification. In this article, we summarize recent knowledge to provide an overview of pediatric gliomas, which are major pediatric CNS tumors, and describe recent developments in strategies employed for their diagnosis and treatment.