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@ARTICLE{Reisinger:143633,
author = {D. Reisinger and J. Gojo and G. Kasprian and C. Haberler
and A. Peyrl and A. A. Azizi and L. Mayr and M. Chocholous
and M. Kool$^*$ and T. Czech and I. Slavc},
title = {{P}redisposition of {W}ingless {S}ubgroup {M}edulloblastoma
for {P}rimary {T}umor {H}emorrhage.},
journal = {Neurosurgery},
volume = {86},
number = {4},
issn = {0148-396X},
address = {Oxford},
publisher = {Oxford University Press},
reportid = {DKFZ-2019-01210},
pages = {478-484},
year = {2020},
note = {2020 Apr 1;86(4):478-484},
abstract = {Primary intratumoral hemorrhage as a presenting sign is
rare in children with medulloblastomas but may result in
severe complications. Given the distinct properties of
molecular medulloblastoma subgroups, the impact on
neurosurgical practice has still to be defined.To
investigate both clinical and radiological presentation of
intratumoral hemorrhage in medulloblastoma patients in the
context of molecular subgroups.Data of all consecutive
medulloblastoma patients treated at our institution between
1993 and 2018 (n = 104) were retrospectively reviewed in
respect of clinical and radiological presentation as well as
molecular subgroups. For cases with available tumor tissue
(n = 86), subgroups were assigned by either 450 K
methylation array or immunohistochemistry and CTNNB1
sequencing. Available imaging at diagnosis (n = 62) was
reviewed by an experienced neuroradiologist.Within the
entire cohort, 4 patients $(4\%)$ presented with massive
spontaneous hemorrhage. Although no patient died as a direct
consequence of hemorrhage, all suffered from serious
sequelae. Moreover, 3 additional patients displayed
radiological evidence of significant hemorrhage.
Interestingly, all 7 cases belonged to the wingless (WNT)
subgroup (n = 13), resulting in intratumoral hemorrhage in
$54\%$ (7/13) of pediatric WNT medulloblastomas. In
contrast, significant hemorrhage was absent in all other
molecular subgroups.Our results suggest that a substantial
proportion of pediatric WNT medulloblastomas display
significant intratumoral hemorrhage at the time of
diagnosis. Consequently, the presence of significant
hemorrhage in fourth ventricle childhood tumors is
suggestive of WNT medulloblastoma and should lead to a less
aggressive attempt for total resection in this
prognostically favorable tumor type.},
cin = {B062 / HD01},
ddc = {610},
cid = {I:(DE-He78)B062-20160331 / I:(DE-He78)HD01-20160331},
pnm = {312 - Functional and structural genomics (POF3-312)},
pid = {G:(DE-HGF)POF3-312},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:31065705},
doi = {10.1093/neuros/nyz148},
url = {https://inrepo02.dkfz.de/record/143633},
}