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@ARTICLE{Flaadt:299483,
      author       = {T. Flaadt and L. Lemelle and M. Abele and C. Virgone and T.
                      Ben-Ami and D. Kachanov and A. Pourtsidis and A. Ferrari and
                      G. Bisogno and E. Bien and N. J. Dos Reis Farinha and J.
                      Godzinski and Y. Reguerre and J. Roganovic and L. D.
                      Kloker$^*$ and U. M. Lauer$^*$ and D. T. Schneider and I. B.
                      Brecht and D. Orbach},
      title        = {{NUT} carcinoma in children and adolescents: {A}n analysis
                      of the {E}uropean {C}ooperative {S}tudy {G}roup on pediatric
                      rare tumors ({EXP}e{RT}).},
      journal      = {Lung cancer},
      volume       = {201},
      issn         = {0169-5002},
      address      = {Amsterdam [u.a.]},
      publisher    = {Elsevier},
      reportid     = {DKFZ-2025-00443},
      pages        = {108449},
      year         = {2025},
      abstract     = {NUT carcinoma (NC) is a sporadic, highly aggressive tumor
                      that primarily affects children, adolescents, and young
                      adults and is characterized by the presence of somatic NUTM1
                      rearrangements. This analysis by the European Cooperative
                      Study Group for Pediatric Rare Tumors (EXPeRT) aims to fill
                      the knowledge gap regarding the clinical characteristics of
                      children with NC.A retrospective case series of NC-patients
                      aged 0-18 years treated between 2011 and 2023 was conducted
                      using the EXPeRT database. Relevant clinical
                      characteristics, including treatment and outcome were
                      recorded.Twenty-seven patients with a median age of 13 years
                      (range 7-18) were analyzed. Thirteen patients were initially
                      misdiagnosed. Sixteen patients had thoracic and 11
                      extra-thoracic tumors, including three in the nasal/sinus
                      region and two in the submandibular glands. Despite intense
                      multimodal treatment, median event-free and overall
                      survivals were 1.5 and 6.5 months, respectively.Early
                      diagnosis of NC by examination of the NUTM1 rearrangement in
                      undifferentiated or poorly differentiated carcinomas is
                      crucial in order to initiate specific and intensive therapy
                      as quickly as possible. Similar to adult patients, only a
                      minority of pediatric patients achieved prolonged survival.
                      Therefore, the development of novel therapeutic strategies
                      in future joint clinical trials is essential.},
      keywords     = {Children (Other) / EXPeRT group (Other) / NUT carcinoma
                      (Other) / Rare tumors (Other) / Targeted therapy (Other)},
      cin          = {TU01},
      ddc          = {610},
      cid          = {I:(DE-He78)TU01-20160331},
      pnm          = {899 - ohne Topic (POF4-899)},
      pid          = {G:(DE-HGF)POF4-899},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:39999637},
      doi          = {10.1016/j.lungcan.2025.108449},
      url          = {https://inrepo02.dkfz.de/record/299483},
}