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@ARTICLE{Hoffman:136865,
      author       = {L. M. Hoffman and S. E. M. Veldhuijzen van Zanten and N.
                      Colditz and J. Baugh and B. Chaney and M. Hoffmann and A.
                      Lane and C. Fuller and L. Miles and C. Hawkins and U.
                      Bartels and E. Bouffet and S. Goldman and S. Leary and N. K.
                      Foreman and R. Packer and K. E. Warren and A. Broniscer and
                      M. W. Kieran and J. Minturn and M. Comito and E. Broxson and
                      C.-S. Shih and S. Khatua and M. Chintagumpala and A. S.
                      Carret and N. Y. Escorza and T. Hassall and D. S. Ziegler
                      and N. Gottardo and H. Dholaria and R. Doughman and M.
                      Benesch and R. Drissi and J. Nazarian and N. Jabado and N.
                      Boddaert and P. Varlet and G. Giraud and D. Castel and S.
                      Puget and C. Jones and E. Hulleman and P. Modena and M.
                      Giagnacovo and M. Antonelli and T. Pietsch and G. H. Gielen
                      and D. Jones$^*$ and D. Sturm$^*$ and S. Pfister$^*$ and N.
                      U. Gerber and M. A. Grotzer and E. Pfaff$^*$ and A. O. von
                      Bueren and D. Hargrave and G. A. Solanki and F. Jadrijevic
                      Cvrlje and G. J. L. Kaspers and W. P. Vandertop and J. Grill
                      and S. Bailey and V. Biassoni and M. Massimino and R. Calmon
                      and E. Sanchez and B. Bison and M. Warmuth-Metz and J. Leach
                      and B. Jones and D. G. van Vuurden and C. M. Kramm and M.
                      Fouladi},
      title        = {{C}linical, {R}adiologic, {P}athologic, and {M}olecular
                      {C}haracteristics of {L}ong-{T}erm {S}urvivors of {D}iffuse
                      {I}ntrinsic {P}ontine {G}lioma ({DIPG}): {A} {C}ollaborative
                      {R}eport {F}rom the {I}nternational and {E}uropean {S}ociety
                      for {P}ediatric {O}ncology {DIPG} {R}egistries.},
      journal      = {Journal of clinical oncology},
      volume       = {36},
      number       = {19},
      issn         = {1527-7755},
      address      = {Alexandria, Va.},
      publisher    = {American Society of Clinical Oncology},
      reportid     = {DKFZ-2018-01303},
      pages        = {1963 - 1972},
      year         = {2018},
      abstract     = {Purpose Diffuse intrinsic pontine glioma (DIPG) is a
                      brainstem malignancy with a median survival of < 1 year. The
                      International and European Society for Pediatric Oncology
                      DIPG Registries collaborated to compare clinical,
                      radiologic, and histomolecular characteristics between
                      short-term survivors (STSs) and long-term survivors (LTSs).
                      Materials and Methods Data abstracted from registry
                      databases included patients from North America, Australia,
                      Germany, Austria, Switzerland, the Netherlands, Italy,
                      France, the United Kingdom, and Croatia. Results Among 1,130
                      pediatric and young adults with radiographically confirmed
                      DIPG, 122 $(11\%)$ were excluded. Of the 1,008 remaining
                      patients, 101 $(10\%)$ were LTSs (survival ≥ 2 years).
                      Median survival time was 11 months (interquartile range, 7.5
                      to 16 months), and 1-, 2-, 3-, 4-, and 5-year survival rates
                      were $42.3\%$ $(95\%$ CI, $38.1\%$ to $44.1\%),$ $9.6\%$
                      $(95\%$ CI, $7.8\%$ to $11.3\%),$ $4.3\%$ $(95\%$ CI,
                      $3.2\%$ to $5.8\%),$ $3.2\%$ $(95\%$ CI, $2.4\%$ to
                      $4.6\%),$ and $2.2\%$ $(95\%$ CI, $1.4\%$ to $3.4\%),$
                      respectively. LTSs, compared with STSs, more commonly
                      presented at age < 3 or > 10 years $(11\%$ v $3\%$ and
                      $33\%$ v $23\%,$ respectively; P < .001) and with longer
                      symptom duration ( P < .001). STSs, compared with LTSs, more
                      commonly presented with cranial nerve palsy $(83\%$ v
                      $73\%,$ respectively; P = .008), ring enhancement $(38\%$ v
                      $23\%,$ respectively; P = .007), necrosis $(42\%$ v $26\%,$
                      respectively; P = .009), and extrapontine extension $(92\%$
                      v $86\%,$ respectively; P = .04). LTSs more commonly
                      received systemic therapy at diagnosis $(88\%$ v $75\%$ for
                      STSs; P = .005). Biopsies and autopsies were performed in
                      299 patients $(30\%)$ and 77 patients $(10\%),$
                      respectively; 181 tumors $(48\%)$ were molecularly
                      characterized. LTSs were more likely to harbor a HIST1H3B
                      mutation (odds ratio, 1.28; $95\%$ CI, 1.1 to 1.5; P =
                      .002). Conclusion We report clinical, radiologic, and
                      molecular factors that correlate with survival in children
                      and young adults with DIPG, which are important for risk
                      stratification in future clinical trials.},
      cin          = {B062 / L101},
      ddc          = {050},
      cid          = {I:(DE-He78)B062-20160331 / 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:29746225},
      pmc          = {pmc:PMC6075859},
      doi          = {10.1200/JCO.2017.75.9308},
      url          = {https://inrepo02.dkfz.de/record/136865},
}