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@ARTICLE{Schoot:284824,
      author       = {R. A. Schoot and D. Orbach and V. Minard Colin and R.
                      Alaggio and D. Di Carlo and N. Corradini and F. Mercolini
                      and G. M. Milano and M. M. van Noesel and A. Rome and P.
                      Dall'Igna and K. Pajtler$^*$ and M. Sparber-Sauer and A.
                      Ferrari and M. Casanova},
      title        = {{I}nflammatory {M}yofibroblastic {T}umor {W}ith {ROS}1
                      {G}ene {F}usions in {C}hildren and {Y}oung {A}dolescents.},
      journal      = {JCO precision oncology},
      volume       = {7},
      number       = {7},
      issn         = {2473-4284},
      address      = {Alexandria, VA},
      publisher    = {American Society of Clinical Oncology},
      reportid     = {DKFZ-2023-02120},
      pages        = {e2300323},
      year         = {2023},
      abstract     = {Inflammatory myofibroblastic tumors (IMTs) are often driven
                      by anaplastic lymphoma kinase fusions and less frequently by
                      alternative fusions such as ROS1. We describe the clinical
                      characteristics, treatment approach, and outcome for a
                      series of young patients with IMTs and ROS1 alterations.This
                      was a retrospective, international, multicenter study
                      analyzing young patients (younger than 21 years) with
                      ROS1-altered IMTs treated in 10 European referral centers
                      between 2014 and 2022. Patients were included in the
                      European pediatric Soft tissue sarcoma Study Group
                      NRSTS-2005 protocol or registered in the Soft Tissue Sarcoma
                      Registry. Primary surgery was recommended if a microscopic
                      radical resection was feasible without mutilation. No
                      standard systemic treatment protocol was available, but
                      several medical options were recommended.A total of 19
                      patients (median age 8.3 years) were included. Most patients
                      had a biopsy at diagnosis (Intergroup Rhabdomyosarcoma Study
                      [IRS] I; n = 2, IRS II; n = 1, IRS III biopsy; n = 11, IRS
                      III resection; n = 3, IRS IV; n = 2). Twelve patients
                      received neoadjuvant systemic therapy in first line (four
                      received multiple treatments): high-dose steroids (n = 2),
                      vinorelbine/vinblastine with methotrexate (n = 6), or ROS1
                      inhibitors (n = 8). After a median follow-up of 2.8 years
                      (range, 0.2-13.4), seven patients developed an event. The
                      3-year event-free survival was $41\%$ $(95\%$ CI, 11 to 71),
                      and the 3-year overall survival was $100\%.Outcome$ for
                      ROS1-altered IMTs appears excellent. A complete resection at
                      diagnosis was often not feasible, and most patients needed
                      neoadjuvant therapy. Patients who developed a tumor event
                      could be cured with reinitiation of systemic therapy and/or
                      surgery. This approach illustrates a switch in treatment
                      philosophy moving from immediate, often mutilating, surgery
                      to systemic (targeted) therapy as a bridge to more
                      conservative surgery later in the treatment course.},
      cin          = {B062 / HD01},
      ddc          = {610},
      cid          = {I:(DE-He78)B062-20160331 / 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:37856763},
      doi          = {10.1200/PO.23.00323},
      url          = {https://inrepo02.dkfz.de/record/284824},
}