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@ARTICLE{Johann:124447,
author = {P. Johann$^*$ and V. Hovestadt$^*$ and C. Thomas and A.
Jeibmann and K. Heß and S. Bens and F. Oyen and C. Hawkins
and C. R. Pierson and K. Aldape and S.-P. Kim and E. Widing
and D. Sumerauer and P. Hauser and F. van Landeghem and M.
Ryzhova and A. Korshunov$^*$ and D. Capper$^*$ and D.
Jones$^*$ and S. Pfister$^*$ and R. Schneppenheim and R.
Siebert and W. Paulus and M. C. Frühwald and M. Kool$^*$
and M. Hasselblatt},
title = {{C}ribriform neuroepithelial tumor: molecular
characterization of a {SMARCB}1-deficient non-rhabdoid tumor
with favorable long-term outcome.},
journal = {Brain pathology},
volume = {27},
number = {4},
issn = {1015-6305},
address = {Oxford},
publisher = {Wiley-Blackwell},
reportid = {DKFZ-2017-01324},
pages = {411 - 418},
year = {2017},
abstract = {Rhabdoid phenotype and loss of SMARCB1 expression in a
brain tumor are characteristic features of atypical
teratoid/rhabdoid tumors (ATRT). Rare non-rhabdoid brain
tumors showing cribriform growth pattern and SMARCB1 loss
have been designated cribriform neuroepithelial tumor
(CRINET). Small case series suggest that CRINETs may have a
relatively favorable prognosis. However, the long-term
outcome is unclear and it remains uncertain whether CRINET
represents a distinct entity or a variant of ATRT.
Therefore, 10 CRINETs were clinically and molecularly
characterized and compared with 10 ATRTs of each of three
recently described molecular subgroups (i.e. ATRT-TYR,
ATRT-SHH and ATRT-MYC) using Illumina Infinium
HumanMethylation450 arrays, FISH, MLPA, and sequencing.
Furthermore, outcome was compared to a larger cohort of 27
children with ATRT-TYR. Median age of the 6 boys and 4 girls
harboring a CRINET was 20 months. On histopathological
examination, all CRINETs demonstrated a cribriform growth
pattern and distinct tyrosinase staining. On unsupervised
cluster analysis of methylation data, all CRINETs examined
exclusively clustered within the ATRT-TYR molecular
subgroup. As ATRT-TYR, CRINETs mainly showed large
heterozygous 22q deletions (9/10) and SMARCB1 mutations of
the other allele. In two patients, SMARCB1 mutations were
also present in the germline. Estimated mean overall
survival in patients with CRINETs was 125 months $(95\%$
confidence interval 100-151 months) as compared to only 53
(33-74) months in patients with ATRTs of the ATRT-TYR
subgroup (Log-Rank P < 0.05). In conclusion, CRINET
represents a SMARCB1-deficient non-rhabdoid tumor, which
shares molecular similarities with the ATRT-TYR subgroup but
has distinct histopathological features and favorable
long-term outcome.},
cin = {B062 / L101 / B060 / G380},
ddc = {610},
cid = {I:(DE-He78)B062-20160331 / I:(DE-He78)L101-20160331 /
I:(DE-He78)B060-20160331 / I:(DE-He78)G380-20160331},
pnm = {312 - Functional and structural genomics (POF3-312)},
pid = {G:(DE-HGF)POF3-312},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:27380723},
doi = {10.1111/bpa.12413},
url = {https://inrepo02.dkfz.de/record/124447},
}