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@ARTICLE{Goschzik:126609,
author = {T. Goschzik and A. Zur Mühlen and G. Kristiansen and C.
Haberler and H. Stefanits and C. Friedrich and K. von Hoff
and S. Rutkowski and S. Pfister$^*$ and T. Pietsch},
title = {{M}olecular stratification of medulloblastoma: comparison
of histological and genetic methods to detect {W}nt
activated tumours.},
journal = {Neuropathology $\&$ applied neurobiology},
volume = {41},
number = {2},
issn = {0305-1846},
address = {Oxford [u.a.]},
publisher = {Wiley-Blackwell},
reportid = {DKFZ-2017-02637},
pages = {135 - 144},
year = {2015},
abstract = {Wnt activation in medulloblastomas is associated with good
outcome. Upfront testing and risk-adapted stratification of
patients will be done in future clinical studies. In a
cohort of 186 paediatric medulloblastomas our aim was to
identify the optimal methods in standard clinical practice
to detect this subgroup.Nuclear accumulation of β-catenin
was analysed by immunohistochemistry (IHC). DNA of FFPE
tissue was amplified by PCR for single-strand conformation
polymorphism analysis and direct sequencing of CTNNB1 exon
3. Copy number of chromosome 6 was analysed by multiplex
ligation-dependent probe amplification and molecular
inversion profiling.Different automated immunostaining
systems showed similar results. Twenty-one of 186 samples
had nuclear accumulation in $≥5\%$ of cells, 17 samples
showed $<5\%$ β-catenin positive nuclei. None of these 17
cases had CTNNB1 mutations, but 18 of 21 cases with $≥5\%$
accumulation did, identifying these 18 cases as Wnt-subgroup
medulloblastomas. Fifteen of 18 mutated cases showed
monosomy 6, 3 had balanced chromosome 6. On the contrary,
none of the CTNNB1 wild-type tumours had monosomy 6.Standard
neuropathological evaluation of medulloblastoma samples
should include IHC of β-catenin because tumours with high
nuclear accumulation of β-catenin most probably belong to
the Wnt subgroup of medulloblastomas. Still, IHC alone may
be insufficient to detect all Wnt cases. Similarly,
chromosome 6 aberrations were not present in all
CTNNB1-mutated cases. Therefore, we conclude that sequencing
analysis of CTNNB1 exon 3 in combination with β-catenin IHC
(possibly as pre-screening method) is a feasible and
cost-efficient way for the determination of Wnt
medulloblastomas.},
keywords = {CTNNB1 protein, human (NLM Chemicals) / beta Catenin (NLM
Chemicals)},
cin = {B062},
ddc = {610},
cid = {I:(DE-He78)B062-20160331},
pnm = {312 - Functional and structural genomics (POF3-312)},
pid = {G:(DE-HGF)POF3-312},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:24894640},
doi = {10.1111/nan.12161},
url = {https://inrepo02.dkfz.de/record/126609},
}