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@ARTICLE{Karremann:131783,
      author       = {M. Karremann and G. H. Gielen and M. Hoffmann and M. Wiese
                      and N. Colditz and M. Warmuth-Metz and B. Bison and A.
                      Claviez and D. G. van Vuurden and A. O. von Bueren and M.
                      Gessi and I. Kühnle and V. H. Hans and M. Benesch and D.
                      Sturm$^*$ and R.-D. Kortmann and A. Waha and T. Pietsch and
                      C. M. Kramm},
      title        = {{D}iffuse high-grade gliomas with {H}3 {K}27{M} mutations
                      carry a dismal prognosis independent of tumor location.},
      journal      = {Neuro-Oncology},
      volume       = {20},
      number       = {1},
      issn         = {1523-5866},
      address      = {Oxford},
      publisher    = {Oxford Univ. Press},
      reportid     = {DKFZ-2018-00080},
      pages        = {123 - 131},
      year         = {2018},
      abstract     = {The novel entity of 'diffuse midline glioma, H3
                      K27M-mutant' has been defined in the 2016 revision of the
                      World Health Organization (WHO) classification of tumors of
                      the central nervous system (CNS). Tumors of this entity
                      arise in CNS midline structures of predominantly pediatric
                      patients and are associated with an overall dismal
                      prognosis. They are defined by K27M mutations in H3F3A or
                      HIST1H3B/C, encoding for histone 3 variants H3.3 and H3.1,
                      respectively, which are considered hallmark events driving
                      gliomagenesis.Here, we characterized 85 centrally reviewed
                      diffuse gliomas on midline locations enrolled in the
                      nationwide pediatric German HIT-HGG registry regarding tumor
                      site, histone 3 mutational status, WHO grade, age, sex, and
                      extent of tumor resection.We found 56 H3.3 K27M-mutant
                      tumors $(66\%),$ 6 H3.1 K27M-mutant tumors $(7\%),$ and 23
                      H3-wildtype tumors $(27\%).$ H3 K27M-mutant gliomas shared
                      an aggressive clinical course independent of their anatomic
                      location. Multivariate regression analysis confirmed the
                      significant impact of the H3 K27M mutation as the only
                      independent parameter predictive of overall survival (P =
                      0.009). In H3 K27M-mutant tumors, neither anatomic midline
                      location nor histopathological grading nor extent of tumor
                      resection had an influence on survival.These results
                      substantiate the clinical significance of considering
                      diffuse midline glioma, H3 K27M-mutant, as a distinct entity
                      corresponding to WHO grade IV, carrying a universally fatal
                      prognosis.},
      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:29016894},
      pmc          = {pmc:PMC5761525},
      doi          = {10.1093/neuonc/nox149},
      url          = {https://inrepo02.dkfz.de/record/131783},
}