In:
Pediatric Blood & Cancer, Wiley, Vol. 68, No. 10 ( 2021-10)
Abstract:
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.
Type of Medium:
Online Resource
ISSN:
1545-5009
,
1545-5017
Language:
English
Publisher:
Wiley
Publication Date:
2021
detail.hit.zdb_id:
2130978-4
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