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

  • Chia Huan Ng
  • Denise Obrecht
  • Olivia Wells
  • Michal Zapotocky
  • David Sumerauer
  • Hallie Coltin
  • Dong-Anh Khuong-Quang
  • David D Eisenstat
  • Kathryn M Kinross
  • Christine L White
  • Elizabeth M Algar
  • Amanda Luck
  • Hendrik Witt
  • Ulrich Schüller
  • Martin Mynarek
  • Torsten Pietsch
  • Nicolas U Gerber
  • Martin Benesch
  • Monika Warmuth-Metz
  • Rolf Kortmann
  • Brigitte Bison
  • Michael D Taylor
  • Stefan Rutkowski
  • Stefan M Pfister
  • David Tw Jones
  • Nicholas G Gottardo
  • Katja von Hoff
  • Kristian W Pajtler
  • Vijay Ramaswamy (Geteilte/r Letztautor/in)
  • Jordan R Hansford (Geteilte/r Letztautor/in)

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.

Bibliografische Daten

OriginalspracheEnglisch
ISSN2632-2498
DOIs
StatusVeröffentlicht - 2023

Anmerkungen des Dekanats

© The Author(s) 2023. Published by Oxford University Press, the Society for Neuro-Oncology and the European Association of Neuro-Oncology.

PubMed 37287693