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@ARTICLE{Mayr:166660,
      author       = {L. Mayr and J. Gojo and A. Peyrl and A. A. Azizi and N. M.
                      Stepien and T. Pletschko and T. Czech and C. Dorfer and S.
                      Lambo$^*$ and K. Dieckmann and C. Haberler and M. Kool$^*$
                      and I. Slavc},
      title        = {{P}otential {I}mportance of {E}arly {F}ocal {R}adiotherapy
                      {F}ollowing {G}ross {T}otal {R}esection for {L}ong-{T}erm
                      {S}urvival in {C}hildren {W}ith {E}mbryonal {T}umors {W}ith
                      {M}ultilayered {R}osettes.},
      journal      = {Frontiers in oncology},
      volume       = {10},
      issn         = {2234-943X},
      address      = {Lausanne},
      publisher    = {Frontiers Media},
      reportid     = {DKFZ-2021-00027},
      pages        = {584681},
      year         = {2020},
      abstract     = {Embryonal tumor with multilayered rosettes (ETMR) is a
                      rare, aggressive embryonal central nervous system tumor
                      characterized by LIN28A expression and alterations in the
                      C19MC locus. ETMRs predominantly occur in young children,
                      have a dismal prognosis, and no definitive treatment
                      guidelines have been established. We report on nine
                      consecutive patients and review the role of
                      initiation/timing of radiotherapy on survival. Between 2006
                      and 2018, nine patients were diagnosed with ETMR. Diagnosis
                      was confirmed histopathologically, immunohistochemically and
                      molecularly. Median age was 25 months (5-38). Location was
                      supratentorial in five, pineal in three, and brainstem in
                      one. Seven patients had a gross total resection, one a
                      partial resection and one a biopsy at initial diagnosis.
                      Chemotherapy augmented with intrathecal therapy started a
                      median of 10 days (7-20) after surgery. Only two patients
                      who after gross total resection received radiotherapy very
                      early on (six weeks after diagnosis) are alive and in
                      complete remission 56 and 50 months after diagnosis. All
                      remaining patients for whom radiotherapy was deferred until
                      the end of chemotherapy recurred, albeit none with
                      leptomeningeal disease. A literature research identified 228
                      patients with ETMR. Including our patients only 26 $(11\%)$
                      of 237 patients survived >36 months with no evidence of
                      disease at last follow-up. All but two long-term (>36
                      months) survivors received radiotherapy, ten of whom early
                      on following gross total resection (GTR). GTR followed by
                      early focal radiotherapy and intrathecal therapy to prevent
                      leptomeningeal disease are potentially important to improve
                      survival of ETMR in the absence of effective targeted
                      therapies.},
      keywords     = {embryonal brain tumors (Other) / embryonal tumor with
                      abundant neuropil and true rosette (Other) / embryonal tumor
                      with multilayered rosette (Other) / focal radiotherapy
                      (Other) / intrathecal therapy (Other) / radiotherapy
                      (Other)},
      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:33392079},
      pmc          = {pmc:PMC7773839},
      doi          = {10.3389/fonc.2020.584681.},
      url          = {https://inrepo02.dkfz.de/record/166660},
}