Oncogenic fusions involving receptor tyrosine kinases (RTK) drive a group of low- and high-grade gliomas (LGG and HGG) in young children. The most common RTK rearrangements involve the oncogenes ALK/ROS1/NTRK/MET and provide an excellent opportunity for therapeutic targeting. These alterations are similar to those observed in multiple unrelated adult and pediatric tumor types, but their roles in gliomagenesis are still largely unknown.
The clinical features and natural history of pediatric RTK-driven gliomas remains uncharacterized. They are more common in infants but also prevalent in older children and adolescents. Interestingly, similar oncofusions drive both benign and malignant gliomas and additional genetic/epigenetic factors are likely to play a role in tumor behavior and explain the clinical heterogeneity.
