Reimagining pilocytic astrocytomas in the context of pediatric low-grade gliomas.
Pediatric low-grade gliomas (pLGGs) are the most common brain tumor in children and are associated with lifelong clinical morbidity. Relative to their high-grade adult counterparts or other malignant childhood brain tumors, there is a paucity of authenticated preclinical models for these pLGGs and an incomplete understanding of their molecular and cellular pathogenesis. While large-scale genomic profiling efforts have identified the majority of pathogenic driver mutations, which converge on the MAPK/ERK signaling pathway, it is now appreciated that these events may not be sufficient by themselves for gliomagenesis and clinical progression. In light of the recent World Health Organization reclassification of pLGGs, and pilocytic astrocytoma (PA), in particular, we review our current understanding of these pediatric brain tumors, provide a conceptual framework for future mechanistic studies, and outline the challenges and pressing needs for the pLGG clinical and research communities.
['Milde|Till|T|0000-0002-7267-1052', 'Rodriguez|Fausto J|FJ|0000-0001-9783-4797', 'Barnholtz-Sloan|Jill S|JS|0000-0001-6190-9304', 'Patil|Nirav|N|', 'Eberhart|Charles G|CG|0000-0002-2910-9427', 'Gutmann|David H|DH|0000-0002-3127-5045']
D001254:Astrocytoma D001932:Brain Neoplasms D002648:Child D005910:Glioma D006801:Humans D020935:MAP Kinase Signaling System D015398:Signal Transduction
pilocytic astrocytoma low-grade glioma pediatric brain tumor BRAF MEK cellular senescence neurofibromatosis type 1 tumor microenvironment