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The clinical features and outcomes of patients with ESL enrolled in a Children\u2019s Oncology Group (COG) clinical trial that evaluated a risk\u2010based strategy for treating soft tissue sarcomas in patients aged &lt;30 years were evaluated.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>This subset analysis included patients with ESL enrolled in COG study ARST0332. Central review of records, pathology, and imaging confirmed the diagnosis, presenting features, and surgery extent and complications. All patients received dose\u2010intensive ifosfamide\/doxorubicin chemotherapy, with cycle timing dependent on surgery and radiotherapy. Tumor resection occurred before study entry or after four cycles of chemotherapy; radiotherapy for residual tumor was optional.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Thirty\u2010nine eligible\/evaluable patients with ESL were analyzed. All tumors were &gt;10 cm in diameter; four were metastatic. Tumor resection was performed upfront in 23 and delayed in 16. Positive surgical margins (<jats:italic>n<\/jats:italic>\u00a0=\u00a06) and intraoperative tumor rupture (<jats:italic>n<\/jats:italic>\u00a0=\u00a06) occurred only in upfront resections. Eight patients received radiotherapy. Estimated 5\u2010year event\u2010free and overall survival were 79% (95% confidence interval [CI], 65%\u201393%) and 95% (95% CI, 87%\u2013100%), respectively. Positive margins increased the local recurrence risk. One of 13 patients with documented hemorrhagic ascites and\/or tumor rupture developed extrahepatic intra\u2010abdominal tumor recurrence.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>The treatment strategy used in ARST0332 achieved favorable outcomes for patients with ESL despite a substantial proportion having high\u2010risk disease features. Deferring tumor resection until after neoadjuvant chemotherapy may decrease the risk of intraoperative tumor rupture and improve the likelihood of adequate surgical margins.<\/jats:p><\/jats:sec>","DOI":"10.1002\/cncr.35305","type":"journal-article","created":{"date-parts":[[2024,4,3]],"date-time":"2024-04-03T05:59:32Z","timestamp":1712123972000},"page":"2683-2693","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Embryonal sarcoma of the liver in pediatric and young adult patients: A report from Children\u2019s Oncology Group study ARST0332"],"prefix":"10.1002","volume":"130","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1669-2676","authenticated-orcid":false,"given":"Sheri L.","family":"Spunt","sequence":"first","affiliation":[{"name":"Department of Pediatrics Stanford University School of Medicine  Palo Alto California USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wei","family":"Xue","sequence":"additional","affiliation":[{"name":"Department of Biostatistics University of Florida College of Public Health and Health Professions and College of Medicine  Gainesville Florida USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zhengya","family":"Gao","sequence":"additional","affiliation":[{"name":"Department of Biostatistics University of Florida College of Public Health and Health Professions and College of Medicine  Gainesville Florida USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrea","family":"Hayes Dixon","sequence":"additional","affiliation":[{"name":"Department of Surgery Howard University College of Medicine  Washington District of Columbia USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5931-4915","authenticated-orcid":false,"given":"Lynn","family":"Million","sequence":"additional","affiliation":[{"name":"Department of Radiation Oncology Stanford University School of Medicine  Palo Alto California USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephanie F.","family":"Polites","sequence":"additional","affiliation":[{"name":"Department of Surgery Mayo Clinic  Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sanjeev A.","family":"Vasudevan","sequence":"additional","affiliation":[{"name":"Division of Pediatric Surgery Department of Surgery Texas Children\u2019s Hospital Baylor College of Medicine  Houston Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Simon C.","family":"Kao","sequence":"additional","affiliation":[{"name":"Department of Radiology University of Iowa Carver College of Medicine  Iowa City Iowa USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M. 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