Pediatric-Type Diffuse Low Grade Glioma.
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Pediatric-type diffuse low grade glioma are a novel subgrouping of pediatric glioma defined in the updated WHO 2021 classification of central nervous system tumors. The newly recognized pediatric-type diffuse low grade glioma family is comprised of four distinct entities, including diffuse astrocytoma MYB or MYBL1-altered, angiocentric glioma, polymorphous low-grade neuroepithelial tumor of the young, and diffuse low grade glioma MAPK-altered. Due to significant overlap in histopathology and molecular alterations between pediatric-type diffuse low grade glioma, accurate diagnosis of these tumor subtypes requires integration of both histology and molecular findings. Herein, we describe the epidemiologic, imaging, and molecular features of these pediatric diffuse glioma. In addition, we review current knowledge regarding management approach and treatment outcomes, including potential therapeutic implications of prevalent molecular alterations within this family of tumors.