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@ARTICLE{Bender:278376,
author = {K. Bender and J. Kahn and E. Perez and F. Ehret$^*$ and S.
Roohani and D. Capper$^*$ and S. Schmid and D. Kaul$^*$},
title = {{D}iffuse paediatric-type high-grade glioma, {H}3-wildtype
and {IDH}-wildtype: case series of a new entity.},
journal = {Brain tumor pathology},
volume = {40},
number = {4},
issn = {1433-7398},
address = {Tōkyō},
publisher = {Springer},
reportid = {DKFZ-2023-01626},
pages = {204-214},
year = {2023},
note = {2023 Oct;40(4):204-214},
abstract = {Diffuse paediatric-type high-grade glioma, H3-wildtype and
IDH-wildtype (pHGG) is a rare and aggressive brain tumor
characterized by a specific DNA methylation profile. It was
recently introduced in the 5th World Health Organization
classification of central nervous system tumors of 2021.
Clinical data on this tumor is scarce. This is a case
series, which presents the first clinical experience with
this entity. We compiled a retrospective case series on pHGG
patients treated between 2015 and 2022 at our institution.
Data collected include patients' clinical course, surgical
procedure, histopathology, genome-wide DNA methylation
analysis, imaging and adjuvant therapy. Eight pHGG were
identified, ranging in age from 8 to 71 years. On MRI tumors
presented with an unspecific intensity profile, T1w hypo- to
isointense and T2w hyperintense, with inhomogeneous contrast
enhancement, often with rim enhancement. Three patients died
of the disease, with overall survival of 19, 28 and 30
months. Four patients were alive at the time of the last
follow-up, 4, 5, 6 and 79 months after the initial surgery.
One patient was lost to follow-up. Findings indicate that
pHGG prevalence might be underestimated in the elderly
population.},
keywords = {Case series (Other) / Diffuse paediatric-type high-grade
glioma (Other) / H3-wildtype (Other) / IDH-wildtype (Other)
/ pHGG (Other)},
cin = {BE01},
ddc = {610},
cid = {I:(DE-He78)BE01-20160331},
pnm = {899 - ohne Topic (POF4-899)},
pid = {G:(DE-HGF)POF4-899},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:37561227},
doi = {10.1007/s10014-023-00468-3},
url = {https://inrepo02.dkfz.de/record/278376},
}