TY  - JOUR
A1  - Koscielniak, Ewa
A1  - Sparber-Sauer, Monika
A1  - Scheer, Monika
A1  - Vokuhl, Christian Oliver
A1  - Kazanowska, Bernarda
A1  - Ladenstein, Ruth
A1  - Niggli, Felix
A1  - Ljungman, Gustaf
A1  - Paulussen, Michael
A1  - Bielack, Stefan S.
A1  - Seitz, Guido
A1  - Fuchs, Jörg
A1  - Hallmen, Erika
A1  - Klingebiel, Thomas
T1  - Extraskeletal Ewing sarcoma in children, adolescents, and young adults. An analysis of three prospective studies of the Cooperative Weichteilsarkomstudiengruppe (CWS)
T2  - Pediatric Blood & Cancer
N2  - 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.
KW  - Ewing sarcoma
KW  - extraskeletal
KW  - pediatric solid tumors
KW  - soft tissue sarcoma
Y1  - 2021
UR  - http://publikationen.ub.uni-frankfurt.de/frontdoor/index/index/docId/73362
UR  - https://nbn-resolving.org/urn:nbn:de:hebis:30:3-733628
SN  - 1545-5017
N1  - 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.
VL  - 68
IS  - 10, art. e29145
SP  - 1
EP  - 10
PB  - Wiley
CY  - New York, NY
ER  -