Abstract
Pediatric low-grade gliomas (PLGGs) are the most common brain tumors in children.
Though histologically benign and associated with excellent outcome, patients with
unresectable lesions—mostly young children with midline tumors—experience multiple
progressions and are at increased risk for long-term neurological sequelae. PLGGs
in children with underlying genetic predisposition syndromes—especially neurofibromatosis
type 1 and tuberous sclerosis—have a distinct natural history and biology with important
treatment implications. Given the complexity of medical issues, optimal management
requires a large network of health care providers; treatment decisions must address
both tumor control and potential side effects of the therapy. Current treatment strategies
often fail to induce sustained tumor regression and many children require several
lines of therapy, highlighting the need for novel therapies. Here, we review the current
management of PLGG and discuss how new molecular targets—in particular alterations
of the Ras/MAPK pathway—are rapidly changing our approach to PLGG.
Keywords
low-grade glioma - children - Ras/MAPK - NF1 - tuberous sclerosis
