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  • Heinz, Amadeus T.Univ Childrens Hosp Tuebingen, Dept Pediat Hematol & Oncol, Tubingen, Germany.;Klinikum Landeshauptstadt Stuttgart, Stuttgart Canc Ctr, Padiat Padiat Onkol Hamatol Immunol 5, Zentrum Kinder Jugend & Frauenmed Olgahosp, Stuttgart, Germany. (author)

Significance of fusion status, Oberlin risk factors, local and maintenance treatment in pediatric and adolescent patients with metastatic rhabdomyosarcoma : Data of the European Soft Tissue Sarcoma Registry SoTiSaR

  • Article/chapterEnglish2024

Publisher, publication year, extent ...

  • John Wiley & Sons,2024
  • electronicrdacarrier

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  • LIBRIS-ID:oai:DiVA.org:uu-533256
  • https://urn.kb.se/resolve?urn=urn:nbn:se:uu:diva-533256URI
  • https://doi.org/10.1002/pbc.30707DOI

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  • Language:English
  • Summary in:English

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  • Subject category:ref swepub-contenttype
  • Subject category:art swepub-publicationtype

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  • Background: Outcome of primary metastatic rhabdomyosarcoma (RMS) is poor. Certain risk factors as fusion status, Oberlin score, and local treatment of primary tumor are known to influence prognosis.Procedure: Patients with metastatic RMS were treated according to Cooperative Weichteilsarkom Studiengruppe (CWS) guidance with chemotherapy (CHT), radiotherapy (RT) excluding total lung irradiation (TLI), complete resection of the primary tumor, and metastasectomy if possible. Kaplan-Meier estimators and Cox proportional hazard models were used to examine event-free survival (EFS) and overall survival (OS) involving also landmark analyses.Results: In the European Soft Tissue Sarcoma Registry SoTiSaR (2009-2018), 211 patients were analyzed. Many patients had fusion-positive alveolar RMS (n = 83; 39%). Median age was 9.4 years [0.1-19.7 years]. Treatment primarily consisted of CHT with CEVAIE (carboplatin, epirubicine, vincristine, actinomycin-D, ifosfamide, etoposide: 86%, other regimens: 14%), RT (71%), resection of primary tumor (37%), metastasectomy (19%), and lymph node sampling/dissection (21%). Maintenance treatment (MT) (oral trofosfamide, idarubicin, etoposide) was added in 74% of patients. Oberlin factors, fusion status, and MT were predictive for EFS and OS. MT with O-TIE was not improving outcome when adjusting for the immortal time bias. Local treatment of the primary tumor and radical irradiation (except TLI) improved EFS, not OS, when adjusting for the Oberlin score. Patients with fusion-negative alveolar RMS (n = 9) had an excellent outcome with a 5-year EFS and OS of 100%, compared to patients with embryonal RMS (49%/62%), PAX7- (22%/47%) and PAX3/FOXO1-positive ARMS (10/13%), respectively (p < .001).Conclusions: Prognosis of metastatic RMS primarily depends on fusion status and Oberlin score. Fusion status needs to be considered in future trials to optimize treatment outcome. The role of radical irradiation needs further investigation.

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Added entries (persons, corporate bodies, meetings, titles ...)

  • Schoenstein, AntonHeidelberg Univ, Network Aging Res, Heidelberg, Germany. (author)
  • Ebinger, MartinUniv Childrens Hosp Tuebingen, Dept Pediat Hematol & Oncol, Tubingen, Germany. (author)
  • Fuchs, JoergUniv Childrens Hosp, Dept Pediat Surg & Urol, Tubingen, Germany. (author)
  • Timmermann, BeateUniv Med Ctr Essen, German Canc Consortium DKTK, West German Canc Ctr WTZ, Dept Particle Therapy,West German Proton Therapy, Essen, Germany. (author)
  • Seitz, GuidoUniv Hosp Giessen Marburg, Dept Pediat Surg & Urol, Marburg, Germany. (author)
  • Vokuhl, ChristianDept Pathol, Sect Pediat Pathol, Bonn, Germany. (author)
  • Muenter, MarcKlinikum Landeshauptstadt Stuttgart, Dept Radiat Oncol, Stuttgart Canc Ctr, Stuttgart, Germany. (author)
  • Pajtler, Kristian W.Heidelberg Univ, Hopp Childrens Canc Ctr Heidelberg KiTZ, Heidelberg, Germany.;German Canc Res Ctr, Div Pediat Neurooncol, Heidelberg, Germany.;Heidelberg Univ, German Canc Consortium DKTK, Heidelberg, Germany.;Heidelberg Univ Hosp, Dept Pediat Oncol Hematol & Immunol, Heidelberg, Germany. (author)
  • Stegmaier, SabineKlinikum Landeshauptstadt Stuttgart, Stuttgart Canc Ctr, Padiat Padiat Onkol Hamatol Immunol 5, Zentrum Kinder Jugend & Frauenmed Olgahosp, Stuttgart, Germany. (author)
  • von Kalle, TheklaKlinikum Landeshauptstadt Stuttgart, Zentrum Kinder Jugend & Frauenmed Olgahosp, Dept Radiol, Stuttgart Canc Ctr, Stuttgart, Germany. (author)
  • Kratz, Christian P.Hannover Med Sch, Pediat Hematol & Oncol, Hannover, Germany. (author)
  • Ljungman, Gustaf,1958-Uppsala universitet,Barnonkologisk forskning - särskilt fokus på komplikationer(Swepub:uu)gustaflm (author)
  • Juntti, HannaOulu Univ Hosp, Dept Pediat & Adolescence, Oulu, Finland. (author)
  • Klingebiel, ThomasUniv Hosp Frankfurt, Dept Children & Adolescents, Frankfurt, Germany. (author)
  • Koscielniak, EwaKlinikum Landeshauptstadt Stuttgart, Stuttgart Canc Ctr, Padiat Padiat Onkol Hamatol Immunol 5, Zentrum Kinder Jugend & Frauenmed Olgahosp, Stuttgart, Germany.;Univ Tubingen, Med Fac, Tubingen, Germany. (author)
  • Sparber-Sauer, MonikaKlinikum Landeshauptstadt Stuttgart, Stuttgart Canc Ctr, Padiat Padiat Onkol Hamatol Immunol 5, Zentrum Kinder Jugend & Frauenmed Olgahosp, Stuttgart, Germany.;Univ Tubingen, Med Fac, Tubingen, Germany. (author)
  • Univ Childrens Hosp Tuebingen, Dept Pediat Hematol & Oncol, Tubingen, Germany.;Klinikum Landeshauptstadt Stuttgart, Stuttgart Canc Ctr, Padiat Padiat Onkol Hamatol Immunol 5, Zentrum Kinder Jugend & Frauenmed Olgahosp, Stuttgart, Germany.Heidelberg Univ, Network Aging Res, Heidelberg, Germany. (creator_code:org_t)

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  • In:Pediatric Blood & Cancer: John Wiley & Sons71:11545-50091545-5017

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