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@ARTICLE{Heinz:275451,
      author       = {A. T. Heinz and M. Ebinger and A. Schönstein and J. Fuchs
                      and B. Timmermann$^*$ and G. Seitz and C. Vokuhl and M. W.
                      Münter and K. Pajtler$^*$ and S. Stegmaier and T. von Kalle
                      and C. P. Kratz and J. Rößler and G. Ljungman and T.
                      Klingebiel and E. Koscielniak and M. Sparber-Sauer},
      collaboration = {C. W. Studiengruppe},
      title        = {{S}econd-line treatment of pediatric patients with relapsed
                      rhabdomyosarcoma adapted to initial risk stratification:
                      {D}ata of the {E}uropean {S}oft {T}issue {S}arcoma
                      {R}egistry ({S}o{T}i{S}a{R}).},
      journal      = {Pediatric blood $\&$ cancer},
      volume       = {70},
      number       = {7},
      issn         = {1545-5009},
      address      = {New York, NY},
      publisher    = {Wiley},
      reportid     = {DKFZ-2023-00771},
      pages        = {e30363},
      year         = {2023},
      note         = {2023 Jul;70(7):e30363},
      abstract     = {Outcome of relapsed disease of localized rhabdomyosarcoma
                      remains poor. An individual treatment approach considering
                      the initial systemic treatment and risk group was included
                      in the Cooperative Weichteilsarkom Studiengruppe (CWS)
                      Guidance.Second-line chemotherapy (sCHT) ACCTTIVE based on
                      anthracyclines (adriamycin, carboplatin, cyclophosphamide,
                      topotecan, vincristine, etoposide) was recommended for
                      patients with initial low- (LR), standard- (SR), and
                      high-risk (HR) group after initial treatment without
                      anthracyclines. TECC (topotecan, etoposide, carboplatin,
                      cyclophosphamide) was recommended after initial
                      anthracycline-based regimen in the very high-risk (VHR)
                      group. Data of patients with relapse (n = 68) registered in
                      the European Soft Tissue Sarcoma Registry SoTiSaR
                      (2009-2018) were retrospectively analyzed.Patients of
                      initial LR (n = 2), SR (n = 16), HR (n = 41), and VHR (n =
                      9) group relapsed. sCHT consisted of ACCTTIVE (n = 36), TECC
                      (n = 12), or other (n = 15). Resection was performed in
                      40/68 $(59\%)$ patients and/or radiotherapy in 47/68
                      $(69\%).$ Initial risk stratification, pattern/time to
                      relapse, and achievement of second complete remission were
                      significant prognostic factors. Microscopically incomplete
                      resection with additional radiotherapy was not inferior to
                      microscopically complete resection (p = .17). The 5-year
                      event-free survival (EFS) and overall survival (OS) were
                      $26\%$ $(±12\%)$ and $31\%$ $(±14\%).$ The 5-year OS of
                      patients with relapse of SR, HR, and VHR groups was $80\%$
                      $(±21\%),$ $20\%$ $(±16\%),$ and $13\%$ $(±23\%,$ p =
                      .008), respectively.Adapted systemic treatment of relapsed
                      disease considering the initial risk group and initial
                      treatment is reasonable. New treatment options are needed
                      for patients of initial HR and VHR groups.},
      keywords     = {Soft Tissue Sarcoma Registry (SoTiSaR) (Other) / localized
                      disease (Other) / relapsed disease (Other) /
                      rhabdomyosarcoma (Other) / second-line chemotherapy (Other)},
      cin          = {ED01 / HD01 / B062},
      ddc          = {610},
      cid          = {I:(DE-He78)ED01-20160331 / I:(DE-He78)HD01-20160331 /
                      I:(DE-He78)B062-20160331},
      pnm          = {312 - Funktionelle und strukturelle Genomforschung
                      (POF4-312)},
      pid          = {G:(DE-HGF)POF4-312},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:37066598},
      doi          = {10.1002/pbc.30363},
      url          = {https://inrepo02.dkfz.de/record/275451},
}