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@ARTICLE{Gojo:182533,
author = {J. Gojo$^*$ and M. Kjaersgaard and B. V. Zezschwitz and D.
Capper$^*$ and A. Tietze and M. Kool$^*$ and C. Haberler and
B. Pizer and K. V. Hoff},
title = {{R}are embryonal and sarcomatous central nervous system
tumours: {S}tate-of-the art and future directions.},
journal = {European journal of medical genetics},
volume = {66},
number = {1},
issn = {1729-7212},
address = {New York, NY [u.a.]},
publisher = {Elsevier},
reportid = {DKFZ-2022-02725},
pages = {104660},
year = {2023},
note = {#EA:B062# / 2023 Nov 7;66(1):104660},
abstract = {The introduction of molecular methods into the diagnostics
of central nervous system (CNS) tumours and the subsequent
deciphering of their molecular heterogeneity has resulted in
a significant impact on paediatric neurooncology.
Particularly in the field of rare embryonal and sarcomatous
CNS tumours, novel tumour types have been delineated and
introduced in the recent 5th edition of the WHO
classification of CNS tumours. The rarity and novelty of
these tumour types result in diagnostic and therapeutic
challenges. Apart from distinct histopathological and
molecular features, these tumour types exhibit
characteristic clinical properties and require different
therapeutic approaches for optimal patient management.
However, based on the limited availability of clinical data,
current therapeutic recommendations have to be based on data
from small, predominantly retrospective patient cohorts.
Within this article, we provide guidance for diagnostic
work-up and clinical management of rare embryonal CNS
tumours ('embryonal tumour with multi-layered rosettes',
ETMR; 'CNS neuroblastoma, FOXR2-activated', CNS NB-FOXR2;
'CNS tumour with BCOR-ITD, CNS BCOR-ITD) and rare
sarcomatous CNS tumours ('primary intracranial sarcoma,
DICER1-mutant', CNS DICER1; 'CIC-rearranged sarcoma', CNS
CIC). By emphasizing the significant consequences on patient
management in paediatric CNS tumours, we want to encourage
wide implementation of comprehensive molecular diagnostics
and stress the importance for joint international efforts to
further collect and study these rare tumour types.},
keywords = {CNS tumour (Other) / Clinical management (Other) /
Embryonal (Other) / Molecular diagnostics (Other) /
Paediatric (Other) / Sarcoma (Other)},
cin = {B062 / HD01 / BE01},
ddc = {570},
cid = {I:(DE-He78)B062-20160331 / I:(DE-He78)HD01-20160331 /
I:(DE-He78)BE01-20160331},
pnm = {312 - Funktionelle und strukturelle Genomforschung
(POF4-312)},
pid = {G:(DE-HGF)POF4-312},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:36356895},
doi = {10.1016/j.ejmg.2022.104660},
url = {https://inrepo02.dkfz.de/record/182533},
}