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@ARTICLE{Rud:132855,
      author       = {R. Rudà and G. Reifenberger$^*$ and D. Frappaz and S.
                      Pfister$^*$ and A. Laprie and T. Santarius and P. Roth and
                      J. C. Tonn$^*$ and R. Soffietti and M. Weller and E. C.
                      Moyal},
      title        = {{EANO} guidelines for the diagnosis and treatment of
                      ependymal tumors.},
      journal      = {Neuro-Oncology},
      volume       = {20},
      number       = {4},
      issn         = {1523-5866},
      address      = {Oxford},
      publisher    = {Oxford Univ. Press},
      reportid     = {DKFZ-2018-00498},
      pages        = {445 - 456},
      year         = {2018},
      abstract     = {Ependymal tumors are rare CNS tumors and may occur at any
                      age, but their proportion among primary brain tumors is
                      highest in children and young adults. Thus, the level of
                      evidence of diagnostic and therapeutic interventions is
                      higher in the pediatric compared with the adult patient
                      population.The diagnosis and disease staging is performed by
                      craniospinal MRI. Tumor classification is achieved by
                      histological and molecular diagnostic assessment of tissue
                      specimens according to the World Health Organization (WHO)
                      classification 2016. Surgery is the crucial initial
                      treatment in both children and adults. In pediatric patients
                      with intracranial ependymomas of WHO grades II or III,
                      surgery is followed by local radiotherapy regardless of
                      residual tumor volume. In adults, radiotherapy is employed
                      in patients with anaplastic ependymoma WHO grade III, and in
                      case of incomplete resection of WHO grade II ependymoma.
                      Chemotherapy alone is reserved for young children <12 months
                      and for adults with recurrent disease when further surgery
                      and irradiation are no longer feasible. A gross total
                      resection is the mainstay of treatment in spinal
                      ependymomas, and radiotherapy is reserved for incompletely
                      resected tumors. Nine subgroups of ependymal tumors across
                      different anatomical compartments (supratentorial, posterior
                      fossa, spinal) and patient ages have been identified with
                      distinct genetic and epigenetic alterations, and with
                      distinct outcomes. These findings may lead to more precise
                      diagnostic and prognostic assessments, molecular
                      subgroup-adapted therapies, and eventually new
                      recommendations pending validation in prospective studies.},
      subtyp        = {Review Article},
      cin          = {L401 / B062 / L101 / L701},
      ddc          = {610},
      cid          = {I:(DE-He78)L401-20160331 / I:(DE-He78)B062-20160331 /
                      I:(DE-He78)L101-20160331 / I:(DE-He78)L701-20160331},
      pnm          = {312 - Functional and structural genomics (POF3-312)},
      pid          = {G:(DE-HGF)POF3-312},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:29194500},
      pmc          = {pmc:PMC5909649},
      doi          = {10.1093/neuonc/nox166},
      url          = {https://inrepo02.dkfz.de/record/132855},
}