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@ARTICLE{Benesch:147254,
      author       = {M. Benesch and K. Nemes and P. Neumayer and M. Hasselblatt
                      and B. Timmermann and B. Bison and G. Ebetsberger-Dachs and
                      F. Bourdeaut and C. Dufour and V. Biassoni and A. Morales La
                      Madrid and N. Entz-Werle and V. Laithier and F. Quehenberger
                      and S. Weis and D. Sumerauer and R. Siebert and S. Bens and
                      R. Schneppenheim and M. Kool$^*$ and P. Modena and F.
                      Fouyssac and M. C Frühwald},
      title        = {{S}pinal cord atypical teratoid/rhabdoid tumors in
                      children: {C}linical, genetic, and outcome characteristics
                      in a representative {E}uropean cohort.},
      journal      = {Pediatric blood $\&$ cancer},
      volume       = {67},
      number       = {1},
      issn         = {1545-5017},
      address      = {New York, NY},
      publisher    = {Wiley},
      reportid     = {DKFZ-2019-02377},
      pages        = {e28022},
      year         = {2020},
      note         = {2020 Jan;67(1):e28022},
      abstract     = {Case reports have portrayed spinal cord atypical
                      teratoid/rhabdoid tumor (spATRT) as an aggressive form of
                      ATRT. We conducted a retrospective European survey to
                      collect data on clinical characteristics, molecular biology,
                      treatment, and outcome of children with intramedullary
                      spATRT.Scrutinizing a French national series and the
                      European Rhabdoid Registry database, we identified 13
                      patients (median age 32 months; metastatic disease at
                      diagnosis, n = 6). Systemic postoperative chemotherapy was
                      administered to all patients; three received intrathecal
                      therapy and six were irradiated (craniospinal, n = 3;
                      local, n = 3).Median observation time was 8 (range, 1-93)
                      months. Progression-free and overall survival rates at 1 and
                      (2 years) were $35.2\%$ ± $13.9\%$ $(26.4\%$ ± $12.9\%)$
                      and $38.5\%$ ± $13.5\%$ $(23.1\%$ ± $11.7\%).$ Four
                      patients (ATRT-SHH, n = 2; ATRT-MYC, n = 1; DNA
                      methylation subgroup not available, n = 1) achieved
                      complete remission (CR); two of them are alive in CR 69 and
                      72 months from diagnosis. One patient relapsed after CR and
                      is alive with progressive disease (PD) and one died of the
                      disease. Three patients (ATRT-MYC, n = 2; subgroup not
                      available, n = 1) died after 7 to 22 months due to PD
                      after having achieved a partial remission (n = 1) or
                      stabilization (n = 2). Five patients (ATRT-MYC, n = 2;
                      subgroup not available, n = 3) developed early PD and
                      died. One patient (ATRT-MYC) died of intracerebral
                      hemorrhage prior to response evaluation.Long-term survival
                      is achievable in selected patients with spATRT using
                      aggressive multimodality treatment. Larger case series and
                      detailed molecular analyses are needed to understand
                      differences between spATRT and their inracranial
                      counterparts and the group of extradural malignant rhabdoid
                      tumors.},
      cin          = {B062 / HD01},
      ddc          = {610},
      cid          = {I:(DE-He78)B062-20160331 / I:(DE-He78)HD01-20160331},
      pnm          = {312 - Functional and structural genomics (POF3-312)},
      pid          = {G:(DE-HGF)POF3-312},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:31571386},
      doi          = {10.1002/pbc.28022},
      url          = {https://inrepo02.dkfz.de/record/147254},
}