Gliomas represent the most frequently diagnosed tumors within the central nervous system (CNS) in children and adolescents, exhibiting a wide spectrum of clinical behaviors. A significant proportion of gliomas in pediatric patients are characterized as benign and slow-growing lesions, typically classified as grade I or II according to the World Health Organization (WHO) classification of CNS tumors. These pediatric low-grade gliomas (LGGs) are distinct from the IDH-mutant LGGs observed in adults, primarily because they rarely undergo malignant transformation and demonstrate high overall survival rates with current treatment strategies.
However, a considerable number of gliomas in children are aggressive, developing and progressing rapidly, leading to their classification as WHO grade III or IV high-grade gliomas (HGGs). Despite intensive therapeutic interventions, these HGGs remain largely incurable, with the most aggressive forms proving fatal within a few months. Consequently, the objectives of neurosurgeons, pediatric oncologists, and radiotherapists caring for young glioma patients range from enhancing the quality of life and preventing long-term complications in the context of LGGs, which are often chronic but seldom life-threatening, to discovering effective treatment modalities to extend patient survival in the almost invariably fatal setting of HGGs.
The past decade has witnessed remarkable advancements in our understanding of the molecular biology underpinning pediatric gliomas. These breakthroughs offer hope for achieving both improved quality of life for LGG patients and effective treatments for HGG patients. Extensive collaborative research efforts worldwide have cataloged genomic and epigenomic alterations in gliomas across different ages, grades, and histological subtypes. These studies have identified distinct biologic subgroups characterized by unique molecular, pathological, and clinical features, which have significant implications for patient management and Pediatric Diagnosis. These molecular insights are increasingly crucial in refining pediatric diagnosis and tailoring treatment strategies.
This review summarizes key discoveries that have significantly expanded our knowledge of both pediatric LGGs and HGGs. It elucidates their roles in tumor biology and conveys current concepts on how these findings can be translated into innovative therapeutic approaches, ultimately improving pediatric diagnosis accuracy and treatment efficacy.
