A multi-institutional retrospective pooled outcome analysis of molecularly annotated pediatric supratentorial ZFTA-fused ependymoma
Standard
A multi-institutional retrospective pooled outcome analysis of molecularly annotated pediatric supratentorial ZFTA-fused ependymoma. / Ng, Chia Huan; Obrecht, Denise; Wells, Olivia; Zapotocky, Michal; Sumerauer, David; Coltin, Hallie; Khuong-Quang, Dong-Anh; Eisenstat, David D; Kinross, Kathryn M; White, Christine L; Algar, Elizabeth M; Luck, Amanda; Witt, Hendrik; Schüller, Ulrich; Mynarek, Martin; Pietsch, Torsten; Gerber, Nicolas U; Benesch, Martin; Warmuth-Metz, Monika; Kortmann, Rolf; Bison, Brigitte; Taylor, Michael D; Rutkowski, Stefan; Pfister, Stefan M; Jones, David Tw; Gottardo, Nicholas G; von Hoff, Katja; Pajtler, Kristian W; Ramaswamy, Vijay; Hansford, Jordan R.
in: NEURO-ONCOL ADV, Jahrgang 5, Nr. 1, 2023, S. vdad057.Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung
Harvard
APA
Vancouver
Bibtex
}
RIS
TY - JOUR
T1 - A multi-institutional retrospective pooled outcome analysis of molecularly annotated pediatric supratentorial ZFTA-fused ependymoma
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 W
AU - Ramaswamy, Vijay
AU - Hansford, Jordan R
N1 - © The Author(s) 2023. Published by Oxford University Press, the Society for Neuro-Oncology and the European Association of Neuro-Oncology.
PY - 2023
Y1 - 2023
N2 - BACKGROUND: 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.METHODS: 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.RESULTS: 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.CONCLUSION: 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.
AB - BACKGROUND: 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.METHODS: 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.RESULTS: 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.CONCLUSION: 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.
U2 - 10.1093/noajnl/vdad057
DO - 10.1093/noajnl/vdad057
M3 - SCORING: Journal article
C2 - 37287693
VL - 5
SP - vdad057
JO - NEURO-ONCOL ADV
JF - NEURO-ONCOL ADV
SN - 2632-2498
IS - 1
ER -