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@ARTICLE{Korshunov:127673,
      author       = {A. Korshunov$^*$ and D. Sturm$^*$ and M. Ryzhova and V.
                      Hovestadt$^*$ and M. Gessi and D. Jones$^*$ and M. Remke and
                      P. Northcott$^*$ and A. Perry and D. Picard and M. Rosenblum
                      and M. Antonelli and E. Aronica and U. Schüller and M.
                      Hasselblatt and A. Woehrer and O. Zheludkova and E. Kumirova
                      and S. Puget and M. D. Taylor and F. Giangaspero and V.
                      Peter Collins and A. von Deimling$^*$ and P. Lichter$^*$ and
                      A. Huang and T. Pietsch and S. Pfister$^*$ and M. Kool$^*$},
      title        = {{E}mbryonal tumor with abundant neuropil and true rosettes
                      ({ETANTR}), ependymoblastoma, and medulloepithelioma share
                      molecular similarity and comprise a single
                      clinicopathological entity.},
      journal      = {Acta neuropathologica},
      volume       = {128},
      number       = {2},
      issn         = {1432-0533},
      address      = {Berlin},
      publisher    = {Springer},
      reportid     = {DKFZ-2017-03696},
      pages        = {279 - 289},
      year         = {2014},
      abstract     = {Three histological variants are known within the family of
                      embryonal rosette-forming neuroepithelial brain tumors.
                      These include embryonal tumor with abundant neuropil and
                      true rosettes (ETANTR), ependymoblastoma (EBL), and
                      medulloepithelioma (MEPL). In this study, we performed a
                      comprehensive clinical, pathological, and molecular analysis
                      of 97 cases of these rare brain neoplasms, including
                      genome-wide DNA methylation and copy number profiling of 41
                      tumors. We identified uniform molecular signatures in all
                      tumors irrespective of histological patterns, indicating
                      that ETANTR, EBL, and MEPL comprise a single biological
                      entity. As such, future WHO classification schemes should
                      consider lumping these variants into a single diagnostic
                      category, such as embryonal tumor with multilayered rosettes
                      (ETMR). We recommend combined LIN28A immunohistochemistry
                      and FISH analysis of the 19q13.42 locus for molecular
                      diagnosis of this tumor category. Recognition of this
                      distinct pediatric brain tumor entity based on the fact that
                      the three histological variants are molecularly and
                      clinically uniform will help to distinguish ETMR from other
                      embryonal CNS tumors and to better understand the biology of
                      these highly aggressive and therapy-resistant pediatric CNS
                      malignancies, possibly leading to alternate treatment
                      strategies.},
      cin          = {G380 / B062 / B060},
      ddc          = {610},
      cid          = {I:(DE-He78)G380-20160331 / I:(DE-He78)B062-20160331 /
                      I:(DE-He78)B060-20160331},
      pnm          = {312 - Functional and structural genomics (POF3-312)},
      pid          = {G:(DE-HGF)POF3-312},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:24337497},
      pmc          = {pmc:PMC4102829},
      doi          = {10.1007/s00401-013-1228-0},
      url          = {https://inrepo02.dkfz.de/record/127673},
}