% IMPORTANT: The following is UTF-8 encoded. This means that in the presence % of non-ASCII characters, it will not work with BibTeX 0.99 or older. % Instead, you should use an up-to-date BibTeX implementation like “bibtex8” or % “biber”. @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}, }