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.
Adolescent
Adult
Antineoplastic Combined Chemotherapy Protocols
/ therapeutic use
Bone Neoplasms
/ mortality
Child
Child, Preschool
Combined Modality Therapy
Female
Follow-Up Studies
Hematopoietic Stem Cell Transplantation
/ mortality
Humans
Lung Neoplasms
/ mortality
Male
Prognosis
Retrospective Studies
Sarcoma, Ewing
/ mortality
Survival Rate
Young Adult
Ewing sarcoma family of tumors
chemotherapy
metastatic
stem cell transplantation
Journal
Pediatric blood & cancer
ISSN: 1545-5017
Titre abrégé: Pediatr Blood Cancer
Pays: United States
ID NLM: 101186624
Informations de publication
Date de publication:
03 2021
03 2021
Historique:
pubmed:
20
12
2020
medline:
3
8
2021
entrez:
19
12
2020
Statut:
ppublish
Résumé
The prognosis of patients with metastatic Ewing sarcoma family of tumors (ESFT) remains poor. We retrospectively analyzed 57 patients diagnosed with metastatic ESFT between 2000 and 2018 to identify prognostic and therapeutic factors affecting the clinical outcome. 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. 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.
Sections du résumé
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.
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
e28844Informations de copyright
© 2020 Wiley Periodicals LLC.
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