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@ARTICLE{Adolph:166506,
      author       = {J. E. Adolph and G. Fleischhack and R. Mikasch and J.
                      Zeller and M. Warmuth-Metz and B. Bison and M. Mynarek and
                      S. Rutkowski and U. Schüller and K. von Hoff and D. Obrecht
                      and T. Pietsch and S. M. Pfister$^*$ and K. W. Pajtler$^*$
                      and O. Witt$^*$ and H. Witt$^*$ and R.-D. Kortmann and B.
                      Timmermann and J. Krauß and M. C. Frühwald and A. Faldum
                      and R. Kwiecien and U. Bode and S. Tippelt},
      collaboration = {G. G. HIT-Network},
      title        = {{L}ocal and {S}ystemic {T}herapy of {R}ecurrent
                      {E}pendymoma in {C}hildren and {A}dolescents: {S}hort- and
                      {L}ong-term {R}esults of the {E}-{HIT}-{REZ} 2005 {S}tudy.},
      journal      = {Neuro-Oncology},
      volume       = {23},
      number       = {6},
      issn         = {1523-5866},
      address      = {Oxford},
      publisher    = {Oxford Univ. Press},
      reportid     = {DKFZ-2020-02949},
      pages        = {1012-1023},
      year         = {2021},
      note         = {2021 Jun 1;23(6):1012-1023},
      abstract     = {Survival in recurrent ependymomas in children and
                      adolescents mainly depends on the extent of resection.
                      Studies on repeated radiotherapy and chemotherapy at relapse
                      have shown conflicting results.Using data from the German
                      multi-center E-HIT-REZ-2005 study, we examined the role of
                      local therapy and the efficacy of chemotherapy with
                      blockwise temozolomide (TMZ) in children and adolescents
                      with recurrent ependymomas.53 patients with a median age of
                      6.9 years (1.25-25.4) at 1 st recurrence and a median
                      follow-up time of 36 months (2-115) were recruited. Gross
                      and near total resection (GTR/NTR) were achieved in 34
                      $(64.2\%)$ patients and associated with a markedly improved
                      5-year overall survival (OS) of $48.7\%$ vs. $5.3\%$ in less
                      than GTR/NTR. Radiotherapy showed no improvement in OS
                      following complete resection (OS: 70 (CI: 19.9-120.1) vs. 95
                      (CI: 20.7-169.4) months), but an advantage was found in less
                      than GTR/NTR (OS: 22 (CI: 12.7-31.3) vs. 7 (CI: 0-15.8)
                      months). Following application of TMZ, disease progression
                      was observed in most evaluable cases (18/21). Subsequent
                      change to oral etoposide and trofosfamide showed no improved
                      response. PF-A EPN were most abundant in relapses (n = 27).
                      RELA-positive EPN (n=5) had a 5-year-OS of $0\%.The$ extent
                      of resection is the most important predictor of survival at
                      relapse. Focal re-irradiation is a useful approach if
                      complete resection cannot be achieved, but no additional
                      benefit was seen after GTR/NTR. Longer-term disease
                      stabilization (>6 months) mediated by TMZ occurred in a
                      small number of cases $(14.3\%).$},
      keywords     = {chemotherapy (Other) / ependymoma (Other) / radiotherapy
                      (Other) / relapse (Other) / tumor resection (Other)},
      cin          = {B062 / B310},
      ddc          = {610},
      cid          = {I:(DE-He78)B062-20160331 / I:(DE-He78)B310-20160331},
      pnm          = {312 - Funktionelle und strukturelle Genomforschung
                      (POF4-312)},
      pid          = {G:(DE-HGF)POF4-312},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:33331885},
      doi          = {10.1093/neuonc/noaa276},
      url          = {https://inrepo02.dkfz.de/record/166506},
}