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Extraskeletal Ewing...
Extraskeletal Ewing sarcoma in children, adolescents, and young adults. An analysis of three prospective studies of the Cooperative Weichteilsarkomstudiengruppe (CWS)
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- Koscielniak, Ewa (author)
- Olga Hosp, Klinikum Stuttgart, Pediat Oncol Hematol Immunol 5, Stuttgart, Germany.;Univ Tubingen, Tubingen, Germany.
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- Sparber-Sauer, Monika (author)
- Olga Hosp, Klinikum Stuttgart, Pediat Oncol Hematol Immunol 5, Stuttgart, Germany.
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- Scheer, Monika (author)
- Charite, Dept Pediat Oncol Hematol, Berlin, Germany.
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- Vokuhl, Christian (author)
- Univ Bonn, Inst Childrens Pathol, Bonn, Germany.
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- Kazanowska, Bernarda (author)
- Univ Wroclaw, Dept Pediat Hematol Oncol, Wroclaw, Poland.;Univ Wroclaw, BMT, Wroclaw, Poland.
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- Ladenstein, Ruth (author)
- St Anna Childrens Hosp, Pediat Oncol, Vienna, Austria.
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- Niggli, Felix (author)
- Univ Zurich, Dept Pediat Oncol, Zurich, Switzerland.
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- Ljungman, Gustaf, 1958- (author)
- Uppsala universitet,Barnneurologi/Barnonkologi
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- Paulussen, Michael (author)
- Witten Herdecke Univ, Datteln, Germany.
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- Bielack, Stefan S. (author)
- Olga Hosp, Klinikum Stuttgart, Pediat Oncol Hematol Immunol 5, Stuttgart, Germany.
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- Seitz, Guido (author)
- Univ Childrens Hosp Marburg, Dept Pediat Surg, Marburg, Germany.
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- Fuchs, Joerg (author)
- Univ Tubingen, Dept Pediat Hematol & Oncol, Hosp Children & Adolescents, Tubingen, Germany.
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- Hallmen, Erika (author)
- Olga Hosp, Klinikum Stuttgart, Pediat Oncol Hematol Immunol 5, Stuttgart, Germany.
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- Klingebiel, Thomas (author)
- Goethe Univ, Univ Hosp, Dept Children & Adolescents, Frankfurt, Germany.
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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)
- 2021-06-05
- 2021
- English.
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In: Pediatric Blood & Cancer. - : John Wiley & Sons. - 1545-5009 .- 1545-5017. ; 68:10
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Abstract
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- 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.
Subject headings
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Hematologi (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Hematology (hsv//eng)
Keyword
- Ewing sarcoma
- extraskeletal
- pediatric solid tumors
- soft tissue sarcoma
Publication and Content Type
- ref (subject category)
- art (subject category)
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- By the author/editor
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Koscielniak, Ewa
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Sparber-Sauer, M ...
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Scheer, Monika
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Vokuhl, Christia ...
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Kazanowska, Bern ...
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Ladenstein, Ruth
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Niggli, Felix
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Ljungman, Gustaf ...
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Paulussen, Micha ...
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Bielack, Stefan ...
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Seitz, Guido
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Fuchs, Joerg
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Hallmen, Erika
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Klingebiel, Thom ...
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- About the subject
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- MEDICAL AND HEALTH SCIENCES
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MEDICAL AND HEAL ...
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and Clinical Medicin ...
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and Hematology
- Articles in the publication
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Pediatric Blood ...
- By the university
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Uppsala University