Abstract
Gliomas are the most common primary central nervous system (CNS) neoplasms in children
and adolescents and are thought to arise from their glial progenitors or stem cells.
Although the exact cells of origin for most pediatric gliomas remain to be identified,
our current understanding is that specific cell populations during CNS development
are susceptible to particular oncogenic events during certain time windows and thus
give rise to pediatric gliomas with distinct histological, molecular, and clinical
features. These may be roughly segregated into low-grade gliomas (WHO grades I or
II; including most mixed glial–neuronal tumors) and high-grade gliomas (WHO grades
III or IV) according to their clinical course when untreated, even though this is
not yet entirely clear for some of the recently emerging groups. The genetic and epigenetic
characterization of pediatric gliomas across ages and histologies has facilitated
the delineation of biologically relevant subgroups and have revealed potentially targetable
alterations in some of them. This review outlines diagnostic features and molecular
alterations in pediatric low- and high-grade gliomas and how the latter might be exploited
with future targeted therapeutic strategies.
Keywords
CNS - brain tumor - pediatric glioma - astrocytoma - genetics - epigenetics - targeted
therapy
