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A multi-institutional retrospective pooled outcome analysis of molecularly annotated pediatric supratentorial ZFTA-fused ependymoma

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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, Vol. 5, No. 1, 2023, p. vdad057.

Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review

Harvard

Ng, CH, Obrecht, D, Wells, O, Zapotocky, M, Sumerauer, D, Coltin, H, Khuong-Quang, D-A, Eisenstat, DD, Kinross, KM, White, CL, Algar, EM, Luck, A, Witt, H, Schüller, U, Mynarek, M, Pietsch, T, Gerber, NU, Benesch, M, Warmuth-Metz, M, Kortmann, R, Bison, B, Taylor, MD, Rutkowski, S, Pfister, SM, Jones, DT, Gottardo, NG, von Hoff, K, Pajtler, KW, Ramaswamy, V & Hansford, JR 2023, 'A multi-institutional retrospective pooled outcome analysis of molecularly annotated pediatric supratentorial ZFTA-fused ependymoma', NEURO-ONCOL ADV, vol. 5, no. 1, pp. vdad057. https://doi.org/10.1093/noajnl/vdad057

APA

Ng, C. H., Obrecht, D., Wells, O., Zapotocky, M., Sumerauer, D., Coltin, H., Khuong-Quang, D-A., Eisenstat, D. D., Kinross, K. M., White, C. L., Algar, E. M., Luck, A., Witt, H., Schüller, U., Mynarek, M., Pietsch, T., Gerber, N. U., Benesch, M., Warmuth-Metz, M., ... Hansford, J. R. (2023). A multi-institutional retrospective pooled outcome analysis of molecularly annotated pediatric supratentorial ZFTA-fused ependymoma. NEURO-ONCOL ADV, 5(1), vdad057. https://doi.org/10.1093/noajnl/vdad057

Vancouver

Ng CH, Obrecht D, Wells O, Zapotocky M, Sumerauer D, Coltin H et al. A multi-institutional retrospective pooled outcome analysis of molecularly annotated pediatric supratentorial ZFTA-fused ependymoma. NEURO-ONCOL ADV. 2023;5(1):vdad057. https://doi.org/10.1093/noajnl/vdad057

Bibtex

@article{24c1d489b98a4348a86f44e60a70bf97,
title = "A multi-institutional retrospective pooled outcome analysis of molecularly annotated pediatric supratentorial ZFTA-fused ependymoma",
abstract = "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.",
author = "Ng, {Chia Huan} and Denise Obrecht and Olivia Wells and Michal Zapotocky and David Sumerauer and Hallie Coltin and Dong-Anh Khuong-Quang and Eisenstat, {David D} and Kinross, {Kathryn M} and White, {Christine L} and Algar, {Elizabeth M} and Amanda Luck and Hendrik Witt and Ulrich Sch{\"u}ller and Martin Mynarek and Torsten Pietsch and Gerber, {Nicolas U} and Martin Benesch and Monika Warmuth-Metz and Rolf Kortmann and Brigitte Bison and Taylor, {Michael D} and Stefan Rutkowski and Pfister, {Stefan M} and Jones, {David Tw} and Gottardo, {Nicholas G} and {von Hoff}, Katja and Pajtler, {Kristian W} and Vijay Ramaswamy and Hansford, {Jordan R}",
note = "{\textcopyright} The Author(s) 2023. Published by Oxford University Press, the Society for Neuro-Oncology and the European Association of Neuro-Oncology.",
year = "2023",
doi = "10.1093/noajnl/vdad057",
language = "English",
volume = "5",
pages = "vdad057",
journal = "NEURO-ONCOL ADV",
issn = "2632-2498",
publisher = "Oxford University Press",
number = "1",

}

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 -