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@ARTICLE{Tietze:293311,
      author       = {A. Tietze and B. Bison and J. Engelhardt and T. Fenouil and
                      D. Figarella-Branger and E. Goebell and J. Hakumäki and E.
                      Koscielniak and L. E. Ludlow and D. Meyronet and P. Nyman
                      and I. Øra and J. Pesola and T. Rauramaa and R. E.
                      Reddingius and D. Samuel and A. Sexton-Oates and A.
                      Vasiljevic and A. K. Wefers and J. Zamecnik and D. Jones and
                      M. K. Keck$^*$ and K. von Hoff},
      collaboration = {E. S. f. P. Oncology},
      othercontributors = {S. Avula and C. Faure-Conter and B. Bison and J. Gojo and
                          C. Haberler and J. T. Hayden and P. D. Johann and D.
                          Jones$^*$ and L. S. Korhonen and C. Kramm and M. Kranendonk
                          and M. Lequin and T. Perwein and A. Schouten-van Meeteren
                          and A. Tietze and T. von Kalle and B. von Zezschwitz and P.
                          Wesseling and M. Zapotocky and K. von Hoff},
      title        = {{CNS} {E}mbryonal {T}umor with {PLAGL} {A}mplification, a
                      {N}ew {T}umor {T}ype in {C}hildren and {A}dolescents:
                      {I}nsights from a {C}omprehensive {MRI} {A}nalysis.},
      journal      = {American journal of neuroradiology},
      volume       = {46},
      number       = {3},
      issn         = {0195-6108},
      address      = {Oak Brook, Ill.},
      publisher    = {Soc.},
      reportid     = {DKFZ-2024-01869},
      pages        = {536-543},
      year         = {2025},
      note         = {2025 Mar 4;46(3):536-543},
      abstract     = {CNS embryonal tumor with PLAGL1/PLAGL2 amplification (ET,
                      PLAGL) is a newly identified, highly malignant pediatric
                      tumor. Systematic MRI descriptions of ET, PLAGL are
                      currently lacking.MRI data from 19 treatment-naïve patients
                      with confirmed ET, PLAGL were analyzed. Evaluation focused
                      on anatomical involvement, tumor localization, MRI signal
                      characteristics, DWI behavior, and the presence of necrosis
                      and hemorrhage. Descriptive statistics (median,
                      interquartile range, percentage) were assessed.Ten patients
                      had PLAGL1 and nine PLAGL2 amplifications. The solid
                      components of the tumors were often multinodular with
                      heterogeneous enhancement (mild to intermediate in $47\%$
                      and intermediate to strong in $47\%$ of cases). Non-solid
                      components included cysts in $47\%$ and necrosis in $84\%$
                      of the cases. The tumors showed heterogeneous T2WI hyper-and
                      isointensity $(74\%),$ relatively little diffusion
                      restriction (ADC values < contralateral normal-appearing WM
                      in $36\%$ of cases with available DWI), and tendencies
                      towards hemorrhage/calcification $(42\%).$ No reliable
                      distinction was found between PLAGL1-and PLAGL2-amplified
                      tumors or compared to other embryonal CNS tumors.The study
                      contributes to understanding the imaging characteristics of
                      ET, PLAGL. It underscores the need for collaboration in
                      studying rare pediatric tumors and advocates for the use of
                      harmonized imaging protocols for better
                      characterization.ATRT= atypical teratoid/rhabdoid tumor;
                      ETMR= embryonal tumor with multilayered rosettes; ET, PLAGL=
                      CNS embryonal tumor with PLAGL amplification; EVD= external
                      ventricular drain; IQR: interquartile range; PLAGL1=
                      pleomorphic adenoma gene-like 1; PLAGL2= pleomorphic adenoma
                      gene-like 2; WHO= World Health Organization.},
      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:39271290},
      doi          = {10.3174/ajnr.A8496},
      url          = {https://inrepo02.dkfz.de/record/293311},
}