TY  - JOUR
AU  - Ng, Chia Huan
AU  - Obrecht, Denise
AU  - Wells, Olivia
AU  - Zapotocky, Michal
AU  - Sumerauer, David
AU  - Coltin, Hallie
AU  - Khuong-Quang, Dong-Anh
AU  - Eisenstat, David D
AU  - Kinross, Kathryn M
AU  - White, Christine L
AU  - Algar, Elizabeth M
AU  - Luck, Amanda
AU  - Witt, Hendrik
AU  - Schüller, Ulrich
AU  - Mynarek, Martin
AU  - Pietsch, Torsten
AU  - Gerber, Nicolas U
AU  - Benesch, Martin
AU  - Warmuth-Metz, Monika
AU  - Kortmann, Rolf
AU  - Bison, Brigitte
AU  - Taylor, Michael D
AU  - Rutkowski, Stefan
AU  - Pfister, Stefan M
AU  - Jones, David Tw
AU  - Gottardo, Nicholas G
AU  - von Hoff, Katja
AU  - Pajtler, Kristian
AU  - Ramaswamy, Vijay
AU  - Hansford, Jordan R
TI  - A multi-institutional retrospective pooled outcome analysis of molecularly annotated pediatric supratentorial ZFTA-fused ependymoma.
JO  - Neuro-oncology advances
VL  - 5
IS  - 1
SN  - 2632-2498
CY  - Oxford
PB  - Oxford University Press
M1  - DKFZ-2023-01142
SP  - vdad057
PY  - 2023
AB  - ZFTA-RELA (formerly known as c11orf-RELA) fused supratentorial ependymoma (ZFTAfus ST-EPN) has been recognized as a novel entity in the 2016 WHO classification of CNS tumors and further defined in the recent 2021 edition. ZFTAfus ST-EPN was reported to portend poorer prognosis when compared to its counterpart, YAP1 ST-EPN in some previously published series. The aim of this study was to determine the treatment outcome of molecularly confirmed and conventionally treated ZFTAfus ST-EPN patients treated in multiple institutions.We conducted a retrospective analysis of all pediatric patients with molecularly confirmed ZFTAfus ST-EPN patients treated in multiple institutions in 5 different countries (Australia, Canada, Germany, Switzerland, and Czechia). Survival outcomes were analyzed and correlated with clinical characteristics and treatment approaches.A total of 108 patients were collated from multiple institutions in 5 different countries across three continents. We found across the entire cohort that the 5- and 10-year PFS were 65
KW  - ZFTA-RELA (Other)
KW  - central nervous system tumors (Other)
KW  - molecular  (Other)
KW  - pediatric (Other)
KW  - supratentorial ependymoma (Other)
LB  - PUB:(DE-HGF)16
C6  - pmid:37287693
C2  - pmc:PMC10243832
DO  - DOI:10.1093/noajnl/vdad057
UR  - https://inrepo02.dkfz.de/record/276761
ER  -