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| 001 | 276761 | ||
| 005 | 20240229155003.0 | ||
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| 100 | 1 | _ | |a Ng, Chia Huan |b 0 |
| 245 | _ | _ | |a A multi-institutional retrospective pooled outcome analysis of molecularly annotated pediatric supratentorial ZFTA-fused ependymoma. |
| 260 | _ | _ | |a Oxford |c 2023 |b Oxford University Press |
| 336 | 7 | _ | |a article |2 DRIVER |
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| 520 | _ | _ | |a 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% 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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| 650 | _ | 7 | |a ZFTA-RELA |2 Other |
| 650 | _ | 7 | |a central nervous system tumors |2 Other |
| 650 | _ | 7 | |a molecular |2 Other |
| 650 | _ | 7 | |a pediatric |2 Other |
| 650 | _ | 7 | |a supratentorial ependymoma |2 Other |
| 700 | 1 | _ | |a Obrecht, Denise |b 1 |
| 700 | 1 | _ | |a Wells, Olivia |b 2 |
| 700 | 1 | _ | |a Zapotocky, Michal |b 3 |
| 700 | 1 | _ | |a Sumerauer, David |b 4 |
| 700 | 1 | _ | |a Coltin, Hallie |b 5 |
| 700 | 1 | _ | |a Khuong-Quang, Dong-Anh |b 6 |
| 700 | 1 | _ | |a Eisenstat, David D |0 0000-0002-5976-0798 |b 7 |
| 700 | 1 | _ | |a Kinross, Kathryn M |b 8 |
| 700 | 1 | _ | |a White, Christine L |b 9 |
| 700 | 1 | _ | |a Algar, Elizabeth M |b 10 |
| 700 | 1 | _ | |a Luck, Amanda |b 11 |
| 700 | 1 | _ | |a Witt, Hendrik |0 P:(DE-He78)046fd145f1008f83f6236580727bbc0f |b 12 |
| 700 | 1 | _ | |a Schüller, Ulrich |0 0000-0002-8731-1121 |b 13 |
| 700 | 1 | _ | |a Mynarek, Martin |0 0000-0003-3302-2719 |b 14 |
| 700 | 1 | _ | |a Pietsch, Torsten |b 15 |
| 700 | 1 | _ | |a Gerber, Nicolas U |b 16 |
| 700 | 1 | _ | |a Benesch, Martin |b 17 |
| 700 | 1 | _ | |a Warmuth-Metz, Monika |0 0000-0002-3544-319X |b 18 |
| 700 | 1 | _ | |a Kortmann, Rolf |b 19 |
| 700 | 1 | _ | |a Bison, Brigitte |b 20 |
| 700 | 1 | _ | |a Taylor, Michael D |b 21 |
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| 700 | 1 | _ | |a Gottardo, Nicholas G |0 0000-0002-1082-6776 |b 25 |
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| 700 | 1 | _ | |a Ramaswamy, Vijay |0 0000-0002-6557-895X |b 28 |
| 700 | 1 | _ | |a Hansford, Jordan R |0 0000-0001-7733-383X |b 29 |
| 773 | _ | _ | |a 10.1093/noajnl/vdad057 |g Vol. 5, no. 1, p. vdad057 |0 PERI:(DE-600)3009682-0 |n 1 |p vdad057 |t Neuro-oncology advances |v 5 |y 2023 |x 2632-2498 |
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