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  • Koscielniak, EwaOlga Hosp, Klinikum Stuttgart, Pediat Oncol Hematol Immunol 5, Stuttgart, Germany.;Univ Tubingen, Tubingen, Germany. (författare)

Extraskeletal Ewing sarcoma in children, adolescents, and young adults. An analysis of three prospective studies of the Cooperative Weichteilsarkomstudiengruppe (CWS)

  • Artikel/kapitelEngelska2021

Förlag, utgivningsår, omfång ...

  • 2021-06-05
  • John Wiley & Sons,2021
  • electronicrdacarrier

Nummerbeteckningar

  • LIBRIS-ID:oai:DiVA.org:uu-454521
  • https://urn.kb.se/resolve?urn=urn:nbn:se:uu:diva-454521URI
  • https://doi.org/10.1002/pbc.29145DOI

Kompletterande språkuppgifter

  • Språk:engelska
  • Sammanfattning på:engelska

Ingår i deldatabas

Klassifikation

  • Ämneskategori:ref swepub-contenttype
  • Ämneskategori:art swepub-publicationtype

Anmärkningar

  • Background We have analyzed the outcome of patients with localized extraskeletal Ewing sarcoma (EES) treated in three consecutive Cooperative Weichteilsarkomstudiengruppe (CWS) soft tissue sarcoma (STS) studies: CWS-91, CWS-96, and CWS-2002P. Methods Patients were treated in CWS-91 with four- (vincristine, dactinomycin, doxorubicin, and ifosfamide [VAIA] or cyclophosphamide [VACA II]) or five-drug (+etoposide [EVAIA]) cycles, in CWS-96 they were randomly assigned to receive VAIA or CEVAIE (+carboplatin and etoposide), and in CWS-2002P with VAIA III plus optional maintenance therapy (MT) with cyclophosphamide and vinblastine. Local therapy consisted of resection and/or radiotherapy (RT). Results Two hundred forty-three patients fulfilled the eligibility criteria. The 5-year event-free survival (EFS) and overall survival (OS) were 63% (95% confidence interval [CI] 57-69) and 73% (95% CI 67-79), respectively. The 5-year EFS by study was 64% (95% CI 54-74) in CWS-91, 57% (95% CI 48-66) in CWS-96, and 79% (95% CI 67-91) in CWS-2002P (n.s.). The 5-year OS was 72% (95% CI 62-82) in CWS-91, 70% (95% CI 61-79) in CWS-96, and 86% (95% CI 76-96) in CWS-2002P (n.s.). In CWS-96, 5-year EFS and OS in the VAIA arm versus the CEVAIE were 65% (95% CI 52-81) versus 55% (95% CI 39-76) log-rank p = .13, and 85% (95% CI 75-96) versus 61% (95% CI 45-82), log-rank p = .09. Conclusion Our analysis provides interesting information on the treatment and specificities of EES, which can be useful for a better understanding of this rare entity and should be considered in the development of future clinical trials for Ewing sarcoma defined as FET-ETS fusion positive tumors.

Ämnesord och genrebeteckningar

Biuppslag (personer, institutioner, konferenser, titlar ...)

  • Sparber-Sauer, MonikaOlga Hosp, Klinikum Stuttgart, Pediat Oncol Hematol Immunol 5, Stuttgart, Germany. (författare)
  • Scheer, MonikaCharite, Dept Pediat Oncol Hematol, Berlin, Germany. (författare)
  • Vokuhl, ChristianUniv Bonn, Inst Childrens Pathol, Bonn, Germany. (författare)
  • Kazanowska, BernardaUniv Wroclaw, Dept Pediat Hematol Oncol, Wroclaw, Poland.;Univ Wroclaw, BMT, Wroclaw, Poland. (författare)
  • Ladenstein, RuthSt Anna Childrens Hosp, Pediat Oncol, Vienna, Austria. (författare)
  • Niggli, FelixUniv Zurich, Dept Pediat Oncol, Zurich, Switzerland. (författare)
  • Ljungman, Gustaf,1958-Uppsala universitet,Barnneurologi/Barnonkologi(Swepub:uu)gustaflm (författare)
  • Paulussen, MichaelWitten Herdecke Univ, Datteln, Germany. (författare)
  • Bielack, Stefan S.Olga Hosp, Klinikum Stuttgart, Pediat Oncol Hematol Immunol 5, Stuttgart, Germany. (författare)
  • Seitz, GuidoUniv Childrens Hosp Marburg, Dept Pediat Surg, Marburg, Germany. (författare)
  • Fuchs, JoergUniv Tubingen, Dept Pediat Hematol & Oncol, Hosp Children & Adolescents, Tubingen, Germany. (författare)
  • Hallmen, ErikaOlga Hosp, Klinikum Stuttgart, Pediat Oncol Hematol Immunol 5, Stuttgart, Germany. (författare)
  • Klingebiel, ThomasGoethe Univ, Univ Hosp, Dept Children & Adolescents, Frankfurt, Germany. (författare)
  • Olga Hosp, Klinikum Stuttgart, Pediat Oncol Hematol Immunol 5, Stuttgart, Germany.;Univ Tubingen, Tubingen, Germany.Olga Hosp, Klinikum Stuttgart, Pediat Oncol Hematol Immunol 5, Stuttgart, Germany. (creator_code:org_t)

Sammanhörande titlar

  • Ingår i:Pediatric Blood & Cancer: John Wiley & Sons68:101545-50091545-5017

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