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@ARTICLE{Sturm:125372,
author = {D. Sturm$^*$ and S. Pfister$^*$ and D. Jones$^*$},
title = {{P}ediatric {G}liomas: {C}urrent {C}oncepts on {D}iagnosis,
{B}iology, and {C}linical {M}anagement.},
journal = {Journal of clinical oncology},
volume = {35},
number = {21},
issn = {1527-7755},
address = {Alexandria, Va.},
publisher = {American Society of Clinical Oncology},
reportid = {DKFZ-2017-01505},
pages = {2370 - 2377},
year = {2017},
abstract = {Gliomas are the most common CNS tumors in children and
adolescents, and they show an extremely broad range of
clinical behavior. The majority of pediatric gliomas present
as benign, slow-growing lesions classified as grade I or II
by the WHO classification of CNS tumors. These pediatric
low-grade gliomas (LGGs) are fundamentally different from
IDH-mutant LGGs occurring in adults, because they rarely
undergo malignant transformation and show excellent overall
survival under current treatment strategies. However, a
significant fraction of gliomas develop over a short period
of time and progress rapidly and are therefore classified as
WHO grade III or IV high-grade gliomas (HGGs). Despite all
therapeutic efforts, they remain largely incurable, with the
most aggressive forms being lethal within months. Thus, the
intentions of neurosurgeons, pediatric oncologists, and
radiotherapists to improve care for pediatric patients with
glioma range from increasing quality of life and preventing
long-term sequelae in what is often a chronic, but rarely
life-threatening disease (LGG), to uncovering effective
treatment options to prolong patient survival in an almost
universally fatal setting (HGG). The last decade has seen
unprecedented progress in understanding the molecular
biology underlying pediatric gliomas, fueling hopes to
achieve both goals. Large-scale collaborative studies around
the globe have cataloged genomic and epigenomic alterations
in gliomas across ages, grades, and histologies. These
studies have revealed biologic subgroups characterized by
distinct molecular, pathologic, and clinical features, with
clear relevance for patient management. In this review, we
summarize hallmark discoveries that have expanded our
knowledge in pediatric LGGs and HGGs, explain their role in
tumor biology, and convey our current concepts on how these
findings may be translated into novel therapeutic
approaches.},
subtyp = {Review Article},
cin = {B062 / L101},
ddc = {050},
cid = {I:(DE-He78)B062-20160331 / I:(DE-He78)L101-20160331},
pnm = {312 - Functional and structural genomics (POF3-312)},
pid = {G:(DE-HGF)POF3-312},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:28640698},
doi = {10.1200/JCO.2017.73.0242},
url = {https://inrepo02.dkfz.de/record/125372},
}