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@ARTICLE{ObrechtSturm:292426,
      author       = {D. Obrecht-Sturm and M. Schoof and A. Eckhardt and M.
                      Mynarek and M. R. Gilbert and K. Aldape and T. S. Armstrong
                      and V. Ramaswamy and M. Bockmayr and K. von Hoff and G.
                      Fleischhack and J. E. Adolph and S. Tippelt and S.
                      Pfister$^*$ and K. Pajtler$^*$ and D. Sturm$^*$ and R.
                      Drexler and F. L. Ricklefs and N. Stepien and J. Gojo and T.
                      Pietsch and M. Warmuth-Metz and R. Kortmann and B.
                      Timmermann and C. Haberler and S. Rutkowski and U.
                      Schüller},
      title        = {{D}istinct relapse pattern across molecular ependymoma
                      types.},
      journal      = {Neuro-Oncology},
      volume       = {27},
      number       = {1},
      issn         = {1522-8517},
      address      = {Oxford},
      publisher    = {Oxford Univ. Press},
      reportid     = {DKFZ-2024-01709},
      pages        = {267–276},
      year         = {2025},
      note         = {Volume 27, Issue 1, January 2025, Pages 267–276},
      abstract     = {Ependymoma (EPN) is not a uniform disease but represents
                      different disease types with biological and clinical
                      heterogeneity. However, the pattern of when and where
                      different types of EPN relapse is not yet comprehensively
                      described.We assembled 269 relapsed intracranial EPN from
                      pediatric (n=233) and adult (n=36) patients from European
                      and Northern American cohorts and correlated DNA methylation
                      patterns and copy-number alterations with clinical
                      information.The cohort comprised the following molecular EPN
                      types: PF-EPN-A (n=177), ST-EPN-ZFTA (n=45), PF-EPN-B
                      (n=31), PF-EPN-SE (n=12), and ST-EPN-YAP (n=4). First
                      relapses of PF-EPN-B (PF: posterior-fossa) and PF-EPN-SE
                      (SE: subependymoma) occurred later than of PF-EPN-A,
                      ST-EPN-YAP (ST: supratentorial), or ST-EPN-ZFTA (median time
                      to relapse: 4.3 and 6.0 years vs. 1.9/1.0/2.4 years;
                      p<0.01). Metastatic or combined recurrences in PF-EPN-B and
                      -A more often involved the spinal cord than in ST-EPN-ZFTA
                      $(72.7\%$ and 40.0 vs. $12.5\%;$ p<0.01). No distant
                      relapses were observed in ST-EPN-YAP (n=4) or PF-EPN-SE
                      (n=12). Post-relapse survival (PRS) was poor for PF-EPN-A
                      and ST-EPN-ZFTA (5-year PRS: $44.5±4.4/47.8±9.1\%),$
                      whereas PF-EPN-B and PF-EPN-SE displayed a 5-year PRS of
                      $89.5±7.1/90.0±9.5\%$ (p=0.03). However, 10-year PRS for
                      PF-EPN-B dropped to $45.8±17.3\%.$ Neither between
                      radiation field and relapse pattern nor between radiation
                      field and spinal involvement at relapse an impact was
                      identified. Notably, all patients with relapsed ST-EPN-YAP
                      did not receive upfront radiotherapy, but were successfully
                      salvaged using irradiation at relapse.Relapse patterns of
                      specific EPN types are different. Future clinical trials,
                      treatment adaptions, duration of surveillance and
                      diagnostics should be planned incorporating entity-specific
                      relapse information.},
      keywords     = {ependymoma (Other) / metastatic (Other) / recurrence
                      (Other) / relapse (Other)},
      cin          = {B360 / HD01},
      ddc          = {610},
      cid          = {I:(DE-He78)B360-20160331 / I:(DE-He78)HD01-20160331},
      pnm          = {312 - Funktionelle und strukturelle Genomforschung
                      (POF4-312)},
      pid          = {G:(DE-HGF)POF4-312},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:39171767},
      doi          = {10.1093/neuonc/noae166},
      url          = {https://inrepo02.dkfz.de/record/292426},
}