{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,22]],"date-time":"2026-10-22T19:10:20Z","timestamp":1792696220153,"version":"4.5.0"},"reference-count":32,"publisher":"Oxford University Press (OUP)","license":[{"start":{"date-parts":[[2026,6,29]],"date-time":"2026-06-29T00:00:00Z","timestamp":1782691200000},"content-version":"am","delay-in-days":2,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"name":"the National Cancer Institute\/National Institutes of Health","award":["U10CA180886"],"award-info":[{"award-number":["U10CA180886"]}]},{"name":"the National Cancer Institute\/National Institutes of Health","award":["U10CA180899"],"award-info":[{"award-number":["U10CA180899"]}]},{"name":"the St. Baldrick\u2019s Foundation"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>We evaluated clinical characteristics, response to therapy, event-free survival (EFS) and overall survival (OS), patterns of recurrence and progression, and factors associated with survival in patients with metastatic nonrhabdomyosarcoma soft tissue sarcoma treated on the Children\u2019s Oncology Group ARST0332 trial.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>All patients with metastatic disease enrolled in ARST0332 were included. Treatment involved multimodal therapy with ifosfamide and doxorubicin with or without surgery and radiotherapy. EFS was time from enrollment to progression, recurrence, second malignancy, or death; OS was defined as the time to death from any cause.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>The analysis included 80 patients, with synovial sarcoma as the most common histology (n\u2009=\u200921, 26%). Two-thirds (n\u2009=\u200960) of patients had more than 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) was 41%, and 11% of patients had progressive disease. At a median follow-up of 7.5\u2009years, 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% CI = 11% to 31%) and 36% (95% CI = 24% to 47%), respectively. In univariable analysis, histologic subtype was significantly associated with EFS, whereas having a single metastatic site and achieving complete response or partial response at week 13 were significantly associated with improved EFS and OS (P\u2009&amp;lt;\u2009.05).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Survival of pediatric metastatic nonrhabdomyosarcoma soft tissue sarcoma 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.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1093\/jnci\/djag211","type":"journal-article","created":{"date-parts":[[2026,6,24]],"date-time":"2026-06-24T11:56:22Z","timestamp":1782302182000},"source":"Crossref","is-referenced-by-count":1,"title":["Outcome of metastatic nonrhabdomyosarcoma soft tissue sarcoma in children and young adults treated on Children\u2019s Oncology Group ARST0332 trial"],"prefix":"10.1093","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6071-0042","authenticated-orcid":false,"given":"Sapna","family":"Oberoi","sequence":"first","affiliation":[{"name":"Department of Pediatrics and Child Health, University of Manitoba , Winnipeg,","place":["Canada"]},{"name":"Department of Pediatric Hematology-Oncology, CancerCare Manitoba , 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