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@ARTICLE{Koelsche:143358,
author = {C. Koelsche and M. Kriegsmann and F. K. F. Kommoss and D.
Stichel$^*$ and K. Kriegsmann and C. Vokuhl and T. G. P.
Grünewald$^*$ and L. Romero-Pérez and T. Kirchner$^*$ and
E. de Alava and J. Diaz-Martin and W. Hartmann and D.
Baumhoer and C. R. Antonescu and K. Szuhai and U. Flucke and
U. Dirksen and S. Pfister$^*$ and D. Jones$^*$ and G.
Mechtersheimer and A. von Deimling$^*$},
title = {{DNA} methylation profiling distinguishes {E}wing-like
sarcoma with {EWSR}1-{NFAT}c2 fusion from {E}wing sarcoma.},
journal = {Journal of cancer research and clinical oncology},
volume = {145},
number = {5},
issn = {0171-5216},
address = {Berlin},
publisher = {Springer42162},
reportid = {DKFZ-2019-00946},
pages = {1273-1281},
year = {2019},
note = {145(5):1273-1281},
abstract = {Recent studies revealed divergent gene expression patterns
in Ewing sarcoma (EwS) with canonical EWSR1-ETS gene fusions
and undifferentiated round cell sarcomas (URCS) with EWSR1
rearrangements fused to the non-ETS gene NFATc2. Thus, the
question arises whether the latter tumors really belong to
EwS.We collected five cases matching the group of URCS with
EWSR1-NFATc2 fusion and performed DNA methylation and copy
number profiling. Results were compared to methylation data
of 30 EwS with various EWSR1-ETS fusions and one EwS with
FUS-ERG fusion, 16 URCS with CIC rearrangement and 10 URCS
with BCOR alteration and a total of 81 EWSR1-associated soft
tissue sarcomas including 7 angiomatoid fibrous
histiocytomas, 7 clear cell sarcomas of the soft tissue, 28
desmoplastic small round cell tumors, 10 extraskeletal
myxoid chondrosarcomas and 29 myxoid
liposarcomas.Unsupervised hierarchical clustering and
t-distributed stochastic neighbor embedding analysis of DNA
methylation data revealed a homogeneous methylation cluster
for URCS with EWSR1-NFATc2 fusion, which clearly segregated
from EwS and the other subtypes. Copy number profiles of
EWSR1-NFATc2 cases showed recurrent losses on chromosome 9q
and segmental gains on 20q13 and 22q12 involving the EWSR1
and NFATc2 loci, respectively.In summary, URCS with
EWSR1-NFATc2 fusion share a distinct DNA methylation
signature and carry characteristic copy number alterations,
which emphasizes that these sarcomas should be considered
separately from EwS.},
cin = {B300 / E131 / B062 / B360 / L101 / L701},
ddc = {610},
cid = {I:(DE-He78)B300-20160331 / I:(DE-He78)E131-20160331 /
I:(DE-He78)B062-20160331 / I:(DE-He78)B360-20160331 /
I:(DE-He78)L101-20160331 / I:(DE-He78)L701-20160331},
pnm = {312 - Functional and structural genomics (POF3-312)},
pid = {G:(DE-HGF)POF3-312},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:30895378},
doi = {10.1007/s00432-019-02895-2},
url = {https://inrepo02.dkfz.de/record/143358},
}