Ewa Koscielniak, Monika Sparber-Sauer, Monika Scheer, Christian Oliver Vokuhl, Bernarda Kazanowska, Ruth Ladenstein, Felix Niggli, Gustaf Ljungman, Michael Paulussen, Stefan S. Bielack, Guido Seitz, Jörg Fuchs, Erika Hallmen, Thomas Klingebiel
- 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.
MetadatenVerfasserangaben: | 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 |
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URN: | urn:nbn:de:hebis:30:3-733628 |
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DOI: | https://doi.org/10.1002/pbc.29145 |
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ISSN: | 1545-5017 |
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Titel des übergeordneten Werkes (Englisch): | Pediatric Blood & Cancer |
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Verlag: | Wiley |
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Verlagsort: | New York, NY |
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Dokumentart: | Wissenschaftlicher Artikel |
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Sprache: | Englisch |
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Datum der Veröffentlichung (online): | 05.06.2021 |
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Datum der Erstveröffentlichung: | 05.06.2021 |
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Veröffentlichende Institution: | Universitätsbibliothek Johann Christian Senckenberg |
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Datum der Freischaltung: | 09.05.2023 |
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Freies Schlagwort / Tag: | Ewing sarcoma; extraskeletal; pediatric solid tumors; soft tissue sarcoma |
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Jahrgang: | 68 |
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Ausgabe / Heft: | 10, art. e29145 |
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Aufsatznummer: | e29145 |
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Seitenzahl: | 10 |
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Erste Seite: | 1 |
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Letzte Seite: | 10 |
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Bemerkung: | 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. |
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Institute: | Medizin |
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DDC-Klassifikation: | 6 Technik, Medizin, angewandte Wissenschaften / 61 Medizin und Gesundheit / 610 Medizin und Gesundheit |
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Sammlungen: | Universitätspublikationen |
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Lizenz (Deutsch): | Creative Commons - CC BY-NC-ND - Namensnennung - Nicht kommerziell - Keine Bearbeitungen 4.0 International |
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