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000276761 1001_ $$aNg, Chia Huan$$b0
000276761 245__ $$aA multi-institutional retrospective pooled outcome analysis of molecularly annotated pediatric supratentorial ZFTA-fused ependymoma.
000276761 260__ $$aOxford$$bOxford University Press$$c2023
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000276761 520__ $$aZFTA-RELA (formerly known as c11orf-RELA) fused supratentorial ependymoma (ZFTAfus ST-EPN) has been recognized as a novel entity in the 2016 WHO classification of CNS tumors and further defined in the recent 2021 edition. ZFTAfus ST-EPN was reported to portend poorer prognosis when compared to its counterpart, YAP1 ST-EPN in some previously published series. The aim of this study was to determine the treatment outcome of molecularly confirmed and conventionally treated ZFTAfus ST-EPN patients treated in multiple institutions.We conducted a retrospective analysis of all pediatric patients with molecularly confirmed ZFTAfus ST-EPN patients treated in multiple institutions in 5 different countries (Australia, Canada, Germany, Switzerland, and Czechia). Survival outcomes were analyzed and correlated with clinical characteristics and treatment approaches.A total of 108 patients were collated from multiple institutions in 5 different countries across three continents. We found across the entire cohort that the 5- and 10-year PFS were 65% and 63%, respectively. The 5- and 10-year OS of this cohort of patients were 87% and 73%. The rates of gross total resection (GTR) were high with 84 out of 108 (77.8%) patients achieving GTR. The vast majority of patients also received post-operative radiotherapy, 98 out of 108 (90.7%). Chemotherapy did not appear to provide any survival benefit in our patient cohort.This is the largest study to date of contemporaneously treated molecularly confirmed ZFTAfus ST-EPN patients which identified markedly improved survival outcomes compared to previously published series. This study also re-emphasizes the importance of maximal surgical resection in achieving optimal outcomes in pediatric patients with supratentorial ependymoma.
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000276761 650_7 $$2Other$$aZFTA-RELA
000276761 650_7 $$2Other$$acentral nervous system tumors
000276761 650_7 $$2Other$$amolecular 
000276761 650_7 $$2Other$$apediatric
000276761 650_7 $$2Other$$asupratentorial ependymoma
000276761 7001_ $$aObrecht, Denise$$b1
000276761 7001_ $$aWells, Olivia$$b2
000276761 7001_ $$aZapotocky, Michal$$b3
000276761 7001_ $$aSumerauer, David$$b4
000276761 7001_ $$aColtin, Hallie$$b5
000276761 7001_ $$aKhuong-Quang, Dong-Anh$$b6
000276761 7001_ $$00000-0002-5976-0798$$aEisenstat, David D$$b7
000276761 7001_ $$aKinross, Kathryn M$$b8
000276761 7001_ $$aWhite, Christine L$$b9
000276761 7001_ $$aAlgar, Elizabeth M$$b10
000276761 7001_ $$aLuck, Amanda$$b11
000276761 7001_ $$0P:(DE-He78)046fd145f1008f83f6236580727bbc0f$$aWitt, Hendrik$$b12
000276761 7001_ $$00000-0002-8731-1121$$aSchüller, Ulrich$$b13
000276761 7001_ $$00000-0003-3302-2719$$aMynarek, Martin$$b14
000276761 7001_ $$aPietsch, Torsten$$b15
000276761 7001_ $$aGerber, Nicolas U$$b16
000276761 7001_ $$aBenesch, Martin$$b17
000276761 7001_ $$00000-0002-3544-319X$$aWarmuth-Metz, Monika$$b18
000276761 7001_ $$aKortmann, Rolf$$b19
000276761 7001_ $$aBison, Brigitte$$b20
000276761 7001_ $$aTaylor, Michael D$$b21
000276761 7001_ $$aRutkowski, Stefan$$b22
000276761 7001_ $$0P:(DE-He78)f746aa965c4e1af518b016de3aaff5d9$$aPfister, Stefan M$$b23$$udkfz
000276761 7001_ $$0P:(DE-He78)551bb92841f634070997aa168d818492$$aJones, David Tw$$b24$$udkfz
000276761 7001_ $$00000-0002-1082-6776$$aGottardo, Nicholas G$$b25
000276761 7001_ $$00000-0002-5669-8546$$avon Hoff, Katja$$b26
000276761 7001_ $$0P:(DE-He78)a7c1bbac024fa232d9c6b78443328d9d$$aPajtler, Kristian$$b27$$udkfz
000276761 7001_ $$00000-0002-6557-895X$$aRamaswamy, Vijay$$b28
000276761 7001_ $$00000-0001-7733-383X$$aHansford, Jordan R$$b29
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