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@ARTICLE{Ng:276761,
      author       = {C. H. Ng and D. Obrecht and O. Wells and M. Zapotocky and
                      D. Sumerauer and H. Coltin and D.-A. Khuong-Quang and D. D.
                      Eisenstat and K. M. Kinross and C. L. White and E. M. Algar
                      and A. Luck and H. Witt$^*$ and U. Schüller and M. Mynarek
                      and T. Pietsch and N. U. Gerber and M. Benesch and M.
                      Warmuth-Metz and R. Kortmann and B. Bison and M. D. Taylor
                      and S. Rutkowski and S. M. Pfister$^*$ and D. T. Jones$^*$
                      and N. G. Gottardo and K. von Hoff and K. Pajtler$^*$ and V.
                      Ramaswamy and J. R. Hansford},
      title        = {{A} multi-institutional retrospective pooled outcome
                      analysis of molecularly annotated pediatric supratentorial
                      {ZFTA}-fused ependymoma.},
      journal      = {Neuro-oncology advances},
      volume       = {5},
      number       = {1},
      issn         = {2632-2498},
      address      = {Oxford},
      publisher    = {Oxford University Press},
      reportid     = {DKFZ-2023-01142},
      pages        = {vdad057},
      year         = {2023},
      abstract     = {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.},
      keywords     = {ZFTA-RELA (Other) / central nervous system tumors (Other) /
                      molecular (Other) / pediatric (Other) / supratentorial
                      ependymoma (Other)},
      cin          = {B062 / B360},
      ddc          = {610},
      cid          = {I:(DE-He78)B062-20160331 / I:(DE-He78)B360-20160331},
      pnm          = {312 - Funktionelle und strukturelle Genomforschung
                      (POF4-312)},
      pid          = {G:(DE-HGF)POF4-312},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:37287693},
      pmc          = {pmc:PMC10243832},
      doi          = {10.1093/noajnl/vdad057},
      url          = {https://inrepo02.dkfz.de/record/276761},
}