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@ARTICLE{Fangusaro:285301,
author = {J. Fangusaro and D. T. Jones$^*$ and R. J. Packer and D. H.
Gutmann and T. Milde$^*$ and O. Witt$^*$ and S. Mueller and
M. J Fisher and J. R. Hansford and U. Tabori and D. Hargrave
and P. Bandopadhayay},
title = {{P}ediatric low-grade glioma: {S}tate-of-the-art and
ongoing challenges.},
journal = {Neuro-Oncology},
volume = {26},
number = {1},
issn = {1522-8517},
address = {Oxford},
publisher = {Oxford Univ. Press},
reportid = {DKFZ-2023-02322},
pages = {25-37},
year = {2024},
note = {2024 Jan 5;26(1):25-37},
abstract = {The most common childhood central nervous system (CNS)
tumor is pediatric low-grade glioma (pLGG), representing
$30\%-40\%$ of all CNS tumors in children. Although there is
high associated morbidity, tumor-related mortality is
relatively rare. pLGG is now conceptualized as a chronic
disease, underscoring the importance of functional outcomes
and quality-of-life measures. A wealth of data has emerged
about these tumors, including a better understanding of
their natural history and their molecular drivers, paving
the way for the use of targeted inhibitors. While these
treatments have heralded tremendous promise, challenges
remain about how to best optimize their use, and the
long-term toxicities associated with these inhibitors remain
unknown. The International Pediatric Low-Grade Glioma
Coalition (iPLGGc) is a global group of physicians and
scientists with expertise in pLGG focused on addressing key
pLGG issues. Here, the iPLGGc provides an overview of the
current state-of-the-art in pLGG, including epidemiology,
histology, molecular landscape, treatment paradigms,
survival outcomes, functional outcomes, imaging response,
and ongoing challenges. This paper also serves as an
introduction to 3 other pLGG manuscripts on (1) pLGG
preclinical models, (2) consensus framework for conducting
early-phase clinical trials in pLGG, and (3) pLGG
resistance, rebound, and recurrence.},
keywords = {MAPK/ERK pathway (Other) / overview (Other) / pediatric
low-grade glioma (Other) / targeted therapy (Other)},
cin = {B360 / B310 / HD01},
ddc = {610},
cid = {I:(DE-He78)B360-20160331 / I:(DE-He78)B310-20160331 /
I:(DE-He78)HD01-20160331},
pnm = {312 - Funktionelle und strukturelle Genomforschung
(POF4-312)},
pid = {G:(DE-HGF)POF4-312},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:37944912},
doi = {10.1093/neuonc/noad195},
url = {https://inrepo02.dkfz.de/record/285301},
}