Journal article
Outcome of metastatic non-rhabdomyosarcoma soft tissue sarcoma in children and young adults treated on children's oncology group ARST0332 trial
JNCI : Journal of the National Cancer Institute
06/27/2026
DOI: 10.1093/jnci/djag211
PMID: 42366679
Abstract
We evaluated clinical characteristics, response to therapy, event-free and overall survival (EFS and OS), patterns of recurrence/progression, and factors associated with survival in patients with metastatic non-rhabdomyosarcoma soft tissue sarcoma (NRSTS) treated on the Children's Oncology Group ARST0332 trial.
All patients with metastatic disease enrolled in ARST0332 were included. Treatment involved multimodal therapy with ifosfamide and doxorubicin, surgery ± radiotherapy. EFS was time from enrollment to progression, recurrence, second malignancy, or death; OS was defined as the time to death from any cause.
The analysis included 80 patients, with synovial sarcoma as the most common histology (n = 21, 26%). Two-thirds of patients (n = 60) had >1 metastatic site, with the lung being the most common site of metastasis. At the week 13 therapy timepoint, response rate [complete or partial response (CR/PR)] was 41%, and 11% of patients had progressive disease. At a median follow-up of 7.5 years, 61 patients had relapse or progression, most commonly occurring at metastatic sites present at diagnosis (41%), followed by new metastatic sites (11%). The 5-year EFS and OS were 21% [95% Confidence Intervals (CI), 11 to 31] and 36% (95% CI, 24 to 47), respectively. In univariable analysis, histologic subtype was associated with EFS, whereas having a single metastatic site and achieving CR or PR at week 13 were associated with improved EFS and OS (p < 0.05).
Survival of pediatric metastatic NRSTS remained dismal on ARST0332. Prognosis varied by histology, metastatic burden, and early treatment response, underscoring the urgent need for novel biologically informed, response-adapted treatment strategies.
Details
- Title: Subtitle
- Outcome of metastatic non-rhabdomyosarcoma soft tissue sarcoma in children and young adults treated on children's oncology group ARST0332 trial
- Creators
- Sapna Oberoi - Health Sciences CentreZhengya Gao - University of Florida HealthWei Xue - University of Florida HealthRobert L Randall - University of California Davis Medical CenterCheryl M Coffin - Vanderbilt UniversityLynn Million - Stanford UniversitySuzanne Wolden - Memorial Sloan Kettering Cancer CenterMary Beth McCarville - St. Jude Children's Research HospitalSimon C Kao - University of IowaRajkumar Venkatramani - Texas Children's HospitalSheri L Spunt - Stanford UniversityAaron R Weiss - MaineHealth
- Resource Type
- Journal article
- Publication Details
- JNCI : Journal of the National Cancer Institute
- DOI
- 10.1093/jnci/djag211
- PMID
- 42366679
- NLM abbreviation
- J Natl Cancer Inst
- ISSN
- 1460-2105
- eISSN
- 1460-2105
- Publisher
- Oxford University Press
- Grant note
- the St. Baldrick's Foundation the National Cancer Institute/National Institutes of Health (NIH): U10CA180886, U10CA180899
This work was supported by grants U10CA180886 and U10CA180899 from the National Cancer Institute/National Institutes of Health (NIH) and the St. Baldrick's Foundation. The ARST0332 clinical trial is registered at clinicaltrials.gov under ID NCT00346164. This manuscript is the result of funding in whole or in part by the NIH. It is subject to the NIH Public Access Policy. Through acceptance of this federal funding, NIH has been given a right to make this manuscript publicly available in PubMed Central upon the Official Date of Publication, as defined by NIH.
- Language
- English
- Electronic publication date
- 06/27/2026
- Academic Unit
- Radiology; Stead Family Department of Pediatrics
- Record Identifier
- 9985179855202771
Metrics
1 Record Views