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Title: Prognostic and therapeutic factors influencing the clinical outcome of metastatic Ewing sarcoma family of tumors: A retrospective report from the Japan Ewing Sarcoma Study Group
Authors: Umeda, Katsutsugu
Miyamura, Takako
Yamada, Kenji
Sano, Hideki
Hosono, Ako
Sumi, Minako
Okita, Hajime
Kamio, Takuya
Maeda, Naoko
Fujisaki, Hiroyuki
Jyoko, Ryoji
Watanabe, Atsuko
Hosoya, Yosuke
Hasegawa, Daiichiro
Takenaka, Satoshi
Nakagawa, Shunsuke
Chin, Motoaki
Ozaki, Toshifumi
Author's alias: 梅田, 雄嗣
Keywords: chemotherapy
Ewing sarcoma family of tumors
stem cell transplantation
Issue Date: Mar-2021
Publisher: Wiley
Journal title: Pediatric Blood & Cancer
Volume: 68
Issue: 3
Thesis number: e28844
Abstract: [Background] The prognosis of patients with metastatic Ewing sarcoma family of tumors (ESFT) remains poor. [Procedure] We retrospectively analyzed 57 patients diagnosed with metastatic ESFT between 2000 and 2018 to identify prognostic and therapeutic factors affecting the clinical outcome. [Results] The 3-year overall survival (OS) rate of the entire cohort was 46.8% (95% confidence interval [CI], 33.0-59.4%). Treatment-related death was not observed. Multivariate analysis identified stem cell transplantation (SCT), response to first-line chemotherapy, and bone metastasis as independent risk factors for OS. Objective response rate to first-line chemotherapy was 65.1% in the 43 evaluable patients. There was no significant difference in the response to different types of first-line chemotherapy. Among patients with lung metastasis alone, the 3-year OS rate was higher in 13 patients who received local treatment than in four who did not, although the difference was not significant. [Conclusions] One possible reason for the high OS rates was the absence of treatment-related mortality even in patients receiving SCT, which could be attributed to advances in the management of post-SCT complications. Novel first-line chemotherapy strategies need to be established to improve the disease status prior to SCT in a higher proportion of patients.
Rights: This is the peer reviewed version of the following article: [Umeda, K, Miyamura, T, Yamada, K, et al. Prognostic and therapeutic factors influencing the clinical outcome of metastatic Ewing sarcoma family of tumors: A retrospective report from the Japan Ewing Sarcoma Study Group. Pediatr Blood Cancer. 2021; 68:e28844.], which has been published in final form at This article may be used for non-commercial purposes in accordance with Wiley Terms and Conditions for Use of Self-Archived Versions. This article may not be enhanced, enriched or otherwise transformed into a derivative work, without express permission from Wiley or by statutory rights under applicable legislation. Copyright notices must not be removed, obscured or modified. The article must be linked to Wiley’s version of record on Wiley Online Library and any embedding, framing or otherwise making available the article or pages thereof by third parties from platforms, services and websites other than Wiley Online Library must be prohibited.
The full-text file will be made open to the public on 19 December 2021 in accordance with publisher's 'Terms and Conditions for Self-Archiving'.
This is not the published version. Please cite only the published version. この論文は出版社版でありません。引用の際には出版社版をご確認ご利用ください。
DOI(Published Version): 10.1002/pbc.28844
PubMed ID: 33340261
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