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@ARTICLE{Kirches:170547,
author = {E. Kirches and F. Sahm and A. Korshunov and C. Bluecher and
N. Waldt and S. Kropf and D. Schrimpf and P. Sievers and D.
Stichel and U. Schüller and J. Schittenhelm and M. J.
Riemenschneider and M. A. Karajannis and A. Perry and T.
Pietsch and S. Boekhoff and D. Capper$^*$ and K. Beck$^*$
and N. Paramasivam$^*$ and M. Schlesner$^*$ and P. K.
Brastianos and H. L. Müller and S. M. Pfister$^*$ and C.
Mawrin},
title = {{M}olecular profiling of pediatric meningiomas shows tumor
characteristics distinct from adult meningiomas.},
journal = {Acta neuropathologica},
volume = {142},
number = {5},
issn = {1432-0533},
address = {Heidelberg},
publisher = {Springer},
reportid = {DKFZ-2021-02001},
pages = {873-886},
year = {2021},
note = {2021 Nov;142(5):873-886},
abstract = {In contrast to adults, meningiomas are uncommon tumors in
childhood and adolescence. Whether adult and pediatric
meningiomas differ on a molecular level is unclear. Here we
report detailed genomic analyses of 37 pediatric meningiomas
by sequencing and DNA methylation profiling. Histologically,
the series was dominated by meningioma subtypes with
aggressive behavior, with $70\%$ of patients suffering from
WHO grade II or III meningiomas. The most frequent
cytogenetic aberrations were loss of chromosomes 22 (23/37
$[62\%]),$ 1 (9/37 $[24\%]),$ 18 (7/37 $[19\%]),$ and 14
(5/37 $[14\%]).$ Tumors with NF2 alterations exhibited
overall increased chromosomal instability. Unsupervised
clustering of DNA methylation profiles revealed separation
into three groups: designated group 1 composed of clear cell
and papillary meningiomas, whereas group 2A comprised
predominantly atypical meningiomas and group 2B enriched for
rare high-grade subtypes (rhabdoid, chordoid). Meningiomas
from NF2 patients clustered exclusively within groups 1 and
2A. When compared with a dataset of 105 adult meningiomas,
the pediatric meningiomas largely grouped separately.
Targeted panel DNA sequencing of 34 tumors revealed frequent
NF2 alterations, while other typical alterations found in
adult non-NF2 tumors were absent. These data demonstrate
that pediatric meningiomas are characterized by molecular
features distinct from adult tumors.},
keywords = {Meningioma (Other) / Methylation profile (Other) / NF2
(Other) / Targeted sequencing (Other)},
cin = {BE01 / B240 / B062 / HD01},
ddc = {610},
cid = {I:(DE-He78)BE01-20160331 / I:(DE-He78)B240-20160331 /
I:(DE-He78)B062-20160331 / 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:34495383},
doi = {10.1007/s00401-021-02351-x},
url = {https://inrepo02.dkfz.de/record/170547},
}