Abstract
In this review, we discuss the molecular characteristics, development, evolution, and therapeutic perspectives for pediatric high-grade glioma (pHGG) arising in cerebral hemispheres. Recently, the understanding of biology of pHGG experienced a revolution with discoveries arising from genomic and epigenomic high-throughput profiling techniques. These findings led to identification of prevalent molecular alterations in pHGG and revealed a strong connection between epigenetic dysregulation and pHGG development. Although we are only beginning to unravel the molecular biology underlying pHGG, there is a desperate need to develop therapies that would improve the outcome of pHGG patients, as current therapies do not elicit significant improvement in median survival for this patient population. We explore the molecular and cell biology and clinical state-of-the-art of pediatric high-grade gliomas (pHGGs) arising in cerebral hemispheres. We discuss the role of driving mutations, with a special consideration of the role of epigenetic-disrupting mutations. We will also discuss the possibilities of targeting unique molecular vulnerabilities of hemispherical pHGG to design innovative tailored therapies.
Highlights
Pediatric high-grade gliomas are highly invasive brain tumors accounting for approximately 15% of all central nervous system tumors in children and adolescents [1]
Significant efforts have been directed toward identifying the epigenetic mechanisms underlying H3K27M mutations and their roled in gliomagenesis and Pediatric high-grade gliomas (pHGG) development in the context of midline pHGG, which led to great discoveries over the last few years
Our knowledge of cortical pHGG has drastically increased over the last decade
Summary
Pediatric high-grade gliomas (pHGG) are highly invasive brain tumors accounting for approximately 15% of all central nervous system tumors in children and adolescents [1]. Patients with pHGG exhibit an array of symptoms consistent with CNS malignancies, such as focal neurological deficits and cranial nerve palsies, with individual presentation largely dependent on the patients’ age and the location of the tumor [5]. Due to their proliferative nature, high-grade gliomas have shorter durations between symptom onset and diagnosis compared to tumors of lower grade, precluding the clinical advantages of early detection [6,7]. There are no effective treatment options and pediatric high-grade glioma has become the leading cause of cancer-related death in children and adolescents under 19 [3,4]
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