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@ARTICLE{Keck:300291,
      author       = {M.-K. Keck$^*$ and A. Tietze and B. Bison and S. Avula and
                      J. Engelhardt and C. Faure-Conter and T. Fenouil and D.
                      Figarella-Branger and E. Goebell and J. Gojo and C. Haberler
                      and J. Hakumäki and J. T. Hayden and L. S. Korhonen and E.
                      Koscielniak and C. M. Kramm and M. E. G. Kranendonk and M.
                      Lequin and L. E. Ludlow and D. Meyronet and P. Nyman and I.
                      Øra and T. Perwein and J. Pesola and T. Rauramaa and R.
                      Reddingius and D. Samuel and A. Y. N. Schouten-van Meeteren
                      and A. Sexton-Oates and A. Vasiljevic and T. von Kalle and
                      A. K. Wefers and P. Wesseling and J. Zamecnik and M.
                      Zapotocky and K. von Hoff and D. Jones$^*$},
      title        = {{C}omparative {C}linical and {I}maging-{B}ased {E}valuation
                      of {T}herapeutic {M}odalities in {CNS} {E}mbryonal {T}umours
                      {W}ith {PLAGL} {A}mplification.},
      journal      = {Neuropathology $\&$ applied neurobiology},
      volume       = {51},
      number       = {2},
      issn         = {0305-1846},
      address      = {Oxford [u.a.]},
      publisher    = {Wiley-Blackwell},
      reportid     = {DKFZ-2025-00744},
      pages        = {e70015},
      year         = {2025},
      note         = {#EA:B360#LA:B360#},
      abstract     = {Embryonal tumours with PLAGL1 or PLAGL2 amplification (ET,
                      PLAGL) show substantial heterogeneity regarding their
                      clinical characteristics and have been treated
                      inconsistently, resulting in diverse outcomes. In this
                      study, we aimed to evaluate the clinical behaviour of ET,
                      PLAGL and elucidate their response pattern across the
                      different applied treatment regimens.We conducted an
                      in-depth retrospective analysis of clinical and serial
                      imaging data of 18 patients with ET, PLAGL (nine each of
                      PLAGL1 and PLAGL2 amplified).Patients with PLAGL1-amplified
                      tumours (ET, PLAGL1) had fewer relapses (3/9), while
                      PLAGL2-amplified tumours (ET, PLAGL2) were prone to early
                      relapse or progression (8/9) and to distant, leptomeningeal
                      and intraventricular relapses. Progression-free survival
                      differed significantly between the subtypes (log-rank test,
                      p = 0.0055). Postoperative treatment included chemotherapy
                      (n = 17, various protocols), alone (n = 8) or combined with
                      radiotherapy (n = 9). Responses to chemotherapy were
                      observed in both subtypes, and incomplete resection was not
                      associated with inferior survival. All three survivors with
                      ET, PLAGL2 were treated with induction and high-dose
                      chemotherapy with (n = 1-low-dose CSI and boost) or without
                      (n = 2) radiotherapy, whereas five patients with less
                      intensive chemotherapy relapsed. All six survivors with ET,
                      PLAGL1 were treated with conventional chemotherapy regimens,
                      with (n = 4-local radiotherapy n = 3; CSI and boost n = 1)
                      or without (n = 2) radiotherapy. Two patients with ET,
                      PLAGL1 relapsed after 8 years.Adjuvant therapy should be
                      considered for all ET, PLAGL patients: Patients with ET,
                      PLAGL2 might benefit from intensified chemotherapy regimens.
                      In contrast, patients with ET, PLAGL1 showed superior
                      outcomes without high-dose chemotherapy or craniospinal
                      irradiation.},
      keywords     = {Humans / Male / Female / Adult / Adolescent / Child /
                      Child, Preschool / Gene Amplification / Middle Aged /
                      DNA-Binding Proteins: genetics / Brain Neoplasms: genetics /
                      Brain Neoplasms: therapy / PLAGL1 (Other) / PLAGL2 (Other) /
                      ET, PLAGL (Other) / embryonal CNS tumour (Other) / treatment
                      (Other) / DNA-Binding Proteins (NLM Chemicals)},
      cin          = {B360},
      ddc          = {610},
      cid          = {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:40196918},
      doi          = {DOI:10.1111/nan.70015},
      url          = {https://inrepo02.dkfz.de/record/300291},
}