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@ARTICLE{Ritzmann:178484,
      author       = {T. A. Ritzmann and R. J. Chapman and J.-P. Kilday and N.
                      Thorp and P. Modena and R. A. Dineen and D. Macarthur and C.
                      Mallucci and T. Jaspan and K. Pajtler$^*$ and M. Giagnacovo
                      and T. S. Jacques and S. M. L. Paine and D. W. Ellison and
                      E. Bouffet and R. G. Grundy},
      collaboration = {B. consortium},
      title        = {{SIOP} {E}pendymoma {I}: {F}inal results, long term
                      follow-up and molecular analysis of the trial cohort: {A}
                      {BIOMECA} {C}onsortium {S}tudy.},
      journal      = {Neuro-Oncology},
      volume       = {24},
      number       = {6},
      issn         = {1522-8517},
      address      = {Oxford},
      publisher    = {Oxford Univ. Press},
      reportid     = {DKFZ-2022-00102},
      pages        = {936-948},
      year         = {2022},
      note         = {2022 Jun 1;24(6):936-948},
      abstract     = {SIOP Ependymoma I was a non-randomised trial assessing
                      event free and overall survival (EFS/OS) of non-metastatic
                      intracranial ependymoma in children aged 3 to 21 years
                      treated with a staged management strategy. A further aim was
                      to assess the response rate (RR) of subtotally resected
                      (STR) ependymoma to vincristine, etoposide and
                      cyclophosphamide (VEC). We report final results with 12-year
                      follow-up and post hoc analyses of recently described
                      biomarkers.74 participants were eligible. Children with
                      gross total resection (GTR) received radiotherapy, whilst
                      those with STR received VEC before radiotherapy. DNA
                      methylation, 1q, hTERT, ReLA, Tenascin-C, H3K27me3 and pAKT
                      status were evaluated.Five- and ten-year EFS was $49.5\%$
                      and $46.7\%,$ OS was $69.3\%$ and $60.5\%.$ GTR was achieved
                      in 33/74 $(44.6\%)$ and associated with improved EFS
                      (p=0.003, HR=2.6, $95\%$ confidence interval (CI) 1.4-5.1).
                      Grade 3 tumours were associated with worse OS (p=0.005,
                      HR=2.8, $95\%CI$ 1.3-5.8). 1q gain and hTERT expression were
                      associated with poorer EFS (p=0.003, HR=2.70, $95\%CI$
                      1.49-6.10 and p=0.014, HR=5.8, $95\%CI$ 1.2-28) and H3K27me3
                      loss with worse OS (p=0.003, HR=4.6, $95\%CI$ 1.5-13.2).
                      Methylation profiles showed expected patterns. 12
                      participants with STR did not receive chemotherapy; a
                      protocol violation. However, best chemotherapy RR was
                      $65.5\%$ (19/29, $95\%CI$ 45.7-82.1), exceeding the
                      prespecified $45\%.Participants$ with totally resected
                      ependymoma had the best outcomes. RR of STR to VEC exceeded
                      the pre-specified efficacy criterion. However, cases of
                      inaccurate stratification highlighted the need for rapid
                      central review. 1q gain, H3K27me3 loss and hTERT expression
                      were all associated with poorer survival outcomes.},
      keywords     = {Chemotherapy (Other) / Ependymoma (Other) / Radiotherapy
                      (Other) / Recurrence (Other) / Resection (Other)},
      cin          = {B062 / HD01},
      ddc          = {610},
      cid          = {I:(DE-He78)B062-20160331 / 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:35018471},
      doi          = {10.1093/neuonc/noac012},
      url          = {https://inrepo02.dkfz.de/record/178484},
}