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@ARTICLE{Pierce:148651,
      author       = {A. M. Pierce and D. A. Witt and A. M. Donson and A. Gilani
                      and B. Sanford and M. Sill$^*$ and B. Van Court and A.
                      Oweida and E. W. Prince and J. Steiner and E. Danis and K.
                      Dorris and T. Hankinson and M. H. Handler and K. L. Jones
                      and S. D. Karam and N. J. Serkova and R. Vibhakar and N. K.
                      Foreman and A. M. Griesinger},
      title        = {{E}stablishment of patient-derived orthotopic xenograft
                      model of 1q+ posterior fossa group {A} ependymoma.},
      journal      = {Neuro-Oncology},
      volume       = {21},
      number       = {12},
      issn         = {1523-5866},
      address      = {Oxford},
      publisher    = {Oxford Univ. Press},
      reportid     = {DKFZ-2019-03189},
      pages        = {1540 - 1551},
      year         = {2019},
      abstract     = {Treatment for pediatric posterior fossa group A (PFA)
                      ependymoma with gain of chromosome 1q (1q+) has not improved
                      over the past decade owing partially to lack of clinically
                      relevant models. We described the first 2 1q+ PFA cell
                      lines, which have significantly enhanced our understanding
                      of PFA tumor biology and provided a tool to identify
                      specific 1q+ PFA therapies. However, cell lines do not
                      accurately replicate the tumor microenvironment. Our present
                      goal is to establish patient-derived xenograft (PDX) mouse
                      models.Disaggregated tumors from 2 1q+ PFA patients were
                      injected into the flanks of NSG mice. Flank tumors were then
                      transplanted into the fourth ventricle or lateral ventricle
                      of NSG mice. Characterization of intracranial tumors was
                      performed using imaging, histology, and
                      $bioinformatics.MAF-811_XC$ and $MAF-928_XC$ established
                      intracranially within the fourth ventricle and retained
                      histological, methylomic, and transcriptomic features of
                      primary patient tumors. We tested the feasibility of
                      treating PDX mice with fractionated radiation or
                      chemotherapy. Mice tolerated radiation despite significant
                      tumor burden, and follow-up imaging confirmed radiation can
                      reduce tumor size. Treatment with fluorouracil reduced tumor
                      size but did not appear to prolong $survival.MAF-811_XC$ and
                      $MAF-928_XC$ are novel, authentic, and reliable models for
                      studying 1q+ PFA in vivo. Given the successful response to
                      radiation, these models will be advantageous for testing
                      clinically relevant combination therapies to develop future
                      clinical trials for this high-risk subgroup of pediatric
                      ependymoma.},
      cin          = {B062},
      ddc          = {610},
      cid          = {I:(DE-He78)B062-20160331},
      pnm          = {312 - Functional and structural genomics (POF3-312)},
      pid          = {G:(DE-HGF)POF3-312},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:31276586},
      doi          = {10.1093/neuonc/noz116},
      url          = {https://inrepo02.dkfz.de/record/148651},
}