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@ARTICLE{CreusBachiller:284601,
      author       = {E. Creus-Bachiller and J. Fernández-Rodríguez and M.
                      Magallón-Lorenz and S. Ortega-Bertran and S. Navas-Rutete
                      and C. Romagosa and T. M. Silva and M. Pané and A. Estival
                      and D. P. Sidelnikova and M. Morell and H. Mazuelas and M.
                      Carrió and T. Lausová$^*$ and D. Reuss$^*$ and B. Gel and
                      A. Villanueva and E. Serra and C. Lázaro},
      title        = {{E}xpanding a precision medicine platform for malignant
                      peripheral nerve sheath tumors: new patient-derived
                      orthotopic xenografts, cell lines and tumor entities.},
      journal      = {Molecular oncology},
      volume       = {18},
      number       = {4},
      issn         = {1574-7891},
      address      = {Hoboken, NJ},
      publisher    = {John Wiley $\&$ Sons, Inc.},
      reportid     = {DKFZ-2023-02021},
      pages        = {895-917},
      year         = {2024},
      note         = {2024 Apr;18(4):895-917},
      abstract     = {Malignant peripheral nerve sheath tumors (MPNSTs) are
                      aggressive soft tissue sarcomas with a poor survival rate,
                      presenting either sporadically or in the context of
                      neurofibromatosis type 1 (NF1). The histological diagnosis
                      of MPNSTs can be challenging, with different tumors
                      exhibiting great histological and marker expression overlap.
                      This heterogeneity could be partly responsible for the
                      observed disparity in treatment response due to the inherent
                      diversity of the preclinical models used. For several years,
                      our group has been generating a large patient-derived
                      orthotopic xenograft (PDOX) MPNST platform for identifying
                      new precision medicine treatments. Herein, we describe the
                      expansion of this platform using six primary tumors
                      clinically diagnosed as MPNSTs, from which we obtained six
                      additional PDOX mouse models and three cell lines, thus
                      generating three pairs of in vitro-in vivo models. We
                      extensively characterized these tumors and derived
                      preclinical models, including genomic, epigenomic and
                      histological analyses. Tumors were reclassified after these
                      analyses: three remained as MPNSTs (two being classic
                      MPNSTs), one was a melanoma, another an Neurotrophic
                      tyrosine receptor kinase (NTRK)-rearranged spindle cell
                      neoplasm, and, finally, an unclassifiable tumor bearing
                      Neurofibromin-2 (NF2) inactivation, an Neuroblastoma RAS
                      Viral Oncogene Homolog (NRAS) oncogenic mutation and a
                      SWI/SNF-related Matrix associated Actin-dependent Regulator
                      of Chromatin (SMARCA4) heterozygous truncated variant. New
                      cell lines and PDOXs faithfully recapitulated histology,
                      marker expression and genomic characteristics of the primary
                      tumors. The diversity in tumor identity and their specific
                      associated genomic alterations impacted on treatment
                      responses obtained when we used the new cell lines for
                      testing compounds against known altered pathways in MPNSTs.
                      In summary, we present here an extension of our MPNST
                      precision medicine platform, with new PDOXs and cell lines,
                      including tumor entities confounded as MPNSTs in a real
                      clinical scenario. This platform may constitute a useful
                      tool for obtaining correct preclinical information to guide
                      MPNST clinical trials.},
      keywords     = {MPNST (Other) / NF1 (Other) / PDOX (Other) / cellular
                      models (Other) / treatment response (Other) / tumor entities
                      (Other)},
      cin          = {B300 / HD01},
      ddc          = {610},
      cid          = {I:(DE-He78)B300-20160331 / I:(DE-He78)HD01-20160331},
      pnm          = {312 - Funktionelle und strukturelle Genomforschung
                      (POF4-312)},
      pid          = {G:(DE-HGF)POF4-312},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:37798904},
      doi          = {10.1002/1878-0261.13534},
      url          = {https://inrepo02.dkfz.de/record/284601},
}