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

  • 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.
Author:Ewa KoscielniakORCiD, Monika Sparber-SauerORCiDGND, Monika ScheerORCiD, Christian Oliver VokuhlORCiDGND, Bernarda KazanowskaORCiD, Ruth LadensteinORCiD, Felix NiggliORCiDGND, Gustaf LjungmanORCiD, Michael Paulussen, Stefan S. BielackORCiD, Guido SeitzORCiDGND, Jörg FuchsGND, Erika Hallmen, Thomas KlingebielORCiDGND
Parent Title (English):Pediatric Blood & Cancer
Place of publication:New York, NY
Document Type:Article
Date of Publication (online):2021/06/05
Date of first Publication:2021/06/05
Publishing Institution:Universitätsbibliothek Johann Christian Senckenberg
Release Date:2023/05/09
Tag:Ewing sarcoma; extraskeletal; pediatric solid tumors; soft tissue sarcoma
Issue:10, art. e29145
Article Number:e29145
Page Number:10
First Page:1
Last Page:10
The data that support the findings of this study are available on request from the corresponding author. The data are not publicly available due to privacy or ethical restrictions.
Dewey Decimal Classification:6 Technik, Medizin, angewandte Wissenschaften / 61 Medizin und Gesundheit / 610 Medizin und Gesundheit
Licence (German):License LogoCreative Commons - CC BY-NC-ND - Namensnennung - Nicht kommerziell - Keine Bearbeitungen 4.0 International