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@ARTICLE{Ramaswamy:130383,
      author       = {V. Ramaswamy and M. Remke$^*$ and E. Bouffet and S. Bailey
                      and S. C. Clifford and F. Doz and M. Kool$^*$ and C. Dufour
                      and G. Vassal and T. Milde$^*$ and O. Witt$^*$ and K. von
                      Hoff and T. Pietsch and P. A. Northcott and A. Gajjar and G.
                      W. Robinson and L. Padovani and N. André and M. Massimino
                      and B. Pizer and R. Packer and S. Rutkowski and S.
                      Pfister$^*$ and M. D. Taylor and S. L. Pomeroy},
      title        = {{R}isk stratification of childhood medulloblastoma in the
                      molecular era: the current consensus.},
      journal      = {Acta neuropathologica},
      volume       = {131},
      number       = {6},
      issn         = {1432-0533},
      address      = {Berlin},
      publisher    = {Springer},
      reportid     = {DKFZ-2017-05462},
      pages        = {821 - 831},
      year         = {2016},
      abstract     = {Historical risk stratification criteria for medulloblastoma
                      rely primarily on clinicopathological variables pertaining
                      to age, presence of metastases, extent of resection,
                      histological subtypes and in some instances individual
                      genetic aberrations such as MYC and MYCN amplification. In
                      2010, an international panel of experts established
                      consensus defining four main subgroups of medulloblastoma
                      (WNT, SHH, Group 3 and Group 4) delineated by
                      transcriptional profiling. This has led to the current
                      generation of biomarker-driven clinical trials assigning WNT
                      tumors to a favorable prognosis group in addition to
                      clinicopathological criteria including MYC and MYCN gene
                      amplifications. However, outcome prediction of non-WNT
                      subgroups is a challenge due to inconsistent survival
                      reports. In 2015, a consensus conference was convened in
                      Heidelberg with the objective to further refine the risk
                      stratification in the context of subgroups and agree on a
                      definition of risk groups of non-infant, childhood
                      medulloblastoma (ages 3-17). Published and unpublished data
                      over the past 5 years were reviewed, and a consensus was
                      reached regarding the level of evidence for currently
                      available biomarkers. The following risk groups were defined
                      based on current survival rates: low risk $(>90 \%$
                      survival), average (standard) risk $(75-90 \%$ survival),
                      high risk $(50-75 \%$ survival) and very high risk
                      $(<50 \%$ survival) disease. The WNT subgroup and
                      non-metastatic Group 4 tumors with whole chromosome 11 loss
                      or whole chromosome 17 gain were recognized as low-risk
                      tumors that may qualify for reduced therapy. High-risk
                      strata were defined as patients with metastatic SHH or Group
                      4 tumors, or MYCN-amplified SHH medulloblastomas. Very
                      high-risk patients are Group 3 with metastases or SHH with
                      TP53 mutation. In addition, a number of consensus points
                      were reached that should be standardized across future
                      clinical trials. Although we anticipate new data will emerge
                      from currently ongoing and recently completed clinical
                      trials, this consensus can serve as an outline for
                      prioritization of certain molecular subsets of tumors to
                      define and validate risk groups as a basis for future
                      clinical trials.},
      keywords     = {Biomarkers, Tumor (NLM Chemicals)},
      cin          = {B062 / G340 / L401 / L101},
      ddc          = {610},
      cid          = {I:(DE-He78)B062-20160331 / I:(DE-He78)G340-20160331 /
                      I:(DE-He78)L401-20160331 / I:(DE-He78)L101-20160331},
      pnm          = {312 - Functional and structural genomics (POF3-312)},
      pid          = {G:(DE-HGF)POF3-312},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:27040285},
      pmc          = {pmc:PMC4867119},
      doi          = {10.1007/s00401-016-1569-6},
      url          = {https://inrepo02.dkfz.de/record/130383},
}