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@ARTICLE{Johann:132820,
author = {P. Johann$^*$ and S. Bens and F. Oyen and R. Wagener and C.
Giannini and A. Perry and J. M. Raisanen and G. F. Reis and
S. Nobusawa and K. Arita and J. Felsberg and G.
Reifenberger$^*$ and A. Agaimy and R. Buslei and D. Capper
and S. Pfister$^*$ and R. Schneppenheim and R. Siebert and
M. C. Frühwald and W. Paulus and M. Kool$^*$ and M.
Hasselblatt},
title = {{S}ellar {R}egion {A}typical {T}eratoid/{R}habdoid {T}umors
({ATRT}) in {A}dults {D}isplay {DNA} {M}ethylation
{P}rofiles of the {ATRT}-{MYC} {S}ubgroup.},
journal = {The American journal of surgical pathology},
volume = {42},
number = {4},
issn = {0147-5185},
address = {Philadelphia, Pa.},
publisher = {Lippincott Williams $\&$ Wilkins},
reportid = {DKFZ-2018-00464},
pages = {506 - 511},
year = {2018},
abstract = {Atypical teratoid/rhabdoid tumor (ATRT) is a highly
malignant brain tumor predominantly encountered in infants.
Mutations of the SMARCB1 gene are the characteristic genetic
lesion. A small group of ATRT stands out clinically, because
these tumors are located in the sellar region of adults. To
investigate if sellar region ATRT in adults represents a
molecular distinct entity, we characterized molecular
alterations in 7 sellar region ATRTs in adults as compared
with 150 pediatric ATRTs and 47 pituitary adenomas using
SMARCB1 sequencing, multiplex ligation-dependent probe
amplification and fluorescence in situ hybridization as well
as DNA methylation profiling. The median age of the 6 female
and 1 male patients was 56 years. On histopathologic
examination, all tumors were malignant rhabdoid tumors
showing loss of SMARCB1/INI1 protein expression. Two cases
displayed compound heterozygous SMARCB1 point mutations, 3
cases showed heterozygous SMARCB1 deletions with point
mutations of the other allele and 1 case a homozygous
SMARCB1 deletion; in 1 case, underlying SMARCB1 alterations
could not be identified. On unsupervised hierarchical
cluster analysis of DNA methylation profiles, sellar region
ATRTs did not form a distinct group, but clustered with
ATRT-MYC, 1 of 3 recently described molecular subgroups of
ATRT. On analysis of DNA methylation array intensity data,
only 1 sellar region ATRT showed characteristic features of
pediatric ATRT-MYC, that is, major copy number losses
affecting the SMARCB1 region. In conclusion, these results
suggest that sellar region ATRTs in adults form a clinically
distinct entity with a different mutational spectrum, but
epigenetic similarities with pediatric ATRTs of the ATRT-MYC
subgroup.},
cin = {B062 / L101 / L401},
ddc = {610},
cid = {I:(DE-He78)B062-20160331 / I:(DE-He78)L101-20160331 /
I:(DE-He78)L401-20160331},
pnm = {312 - Functional and structural genomics (POF3-312)},
pid = {G:(DE-HGF)POF3-312},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:29324471},
doi = {10.1097/PAS.0000000000001023},
url = {https://inrepo02.dkfz.de/record/132820},
}