{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,18]],"date-time":"2026-07-18T10:38:37Z","timestamp":1784371117178,"version":"3.55.0"},"reference-count":42,"publisher":"Wiley","issue":"10","license":[{"start":{"date-parts":[[2016,6,14]],"date-time":"2016-06-14T00:00:00Z","timestamp":1465862400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["U10 CA98543"],"award-info":[{"award-number":["U10 CA98543"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatric Blood &amp; Cancer"],"published-print":{"date-parts":[[2016,10]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>The prognostic significance of having extraskeletal (EES) versus skeletal Ewing sarcoma (ES) in the setting of modern chemotherapy protocols is unknown. The purpose of this study was to compare the clinical characteristics, biologic features, and outcomes for patients with EES and skeletal ES.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Patients had localized ES and were treated on two consecutive protocols using five\u2010drug chemotherapy (INT\u20100154 and AEWS0031). Patients were analyzed based on having an extraskeletal (n = 213) or skeletal (n = 826) site of tumor origin. Event\u2010free survival (EFS) was estimated using the Kaplan\u2013Meier method, compared using the log\u2010rank test, and modeled using Cox multivariate regression.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Patients with extraskeletal ES (EES) were more likely to have axial tumors (72% vs. 55%; <jats:italic>P<\/jats:italic> &lt; 0.001), less likely to have tumors &gt;8 cm (9% vs. 17%; <jats:italic>P<\/jats:italic> &lt; 0.01), and less likely to be white (81% vs. 87%; <jats:italic>P<\/jats:italic> &lt; 0.001) compared to patients with skeletal ES. There was no difference in key genomic features (type of <jats:italic>EWSR1<\/jats:italic> translocation, <jats:italic>TP53<\/jats:italic> mutation, <jats:italic>CDKN2A<\/jats:italic> mutation\/loss) between groups. After controlling for age, race, and primary site, EES was associated with superior EFS (hazard ratio = 0.69; 95% confidence interval: 0.50\u20130.95; <jats:italic>P<\/jats:italic> = 0.02). Among patients with EES, age \u226518, nonwhite race, and elevated baseline erythrocyte sedimentation rate were independently associated with inferior EFS.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Clinical characteristics, but not key tumor genomic features, differ between EES and skeletal ES. Extraskeletal origin is a favorable prognostic factor, independent of age, race, and primary site.<\/jats:p><\/jats:sec>","DOI":"10.1002\/pbc.26096","type":"journal-article","created":{"date-parts":[[2016,6,14]],"date-time":"2016-06-14T12:29:37Z","timestamp":1465907377000},"page":"1771-1779","source":"Crossref","is-referenced-by-count":101,"title":["Comparison of clinical features and outcomes in patients with extraskeletal versus skeletal localized Ewing sarcoma: A report from the Children's Oncology Group"],"prefix":"10.1002","volume":"63","author":[{"given":"Thomas","family":"Cash","sequence":"first","affiliation":[{"name":"Department of Pediatrics Children's Healthcare of Atlanta Emory University  Atlanta Georgia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elizabeth","family":"McIlvaine","sequence":"additional","affiliation":[{"name":"Department of Preventive Medicine Keck School of Medicine University of Southern California  Los Angeles California"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mark D.","family":"Krailo","sequence":"additional","affiliation":[{"name":"Department of Preventive Medicine Keck School of Medicine University of Southern California  Los Angeles California"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephen L.","family":"Lessnick","sequence":"additional","affiliation":[{"name":"Center for Childhood Cancer and Blood Disorders at Nationwide Children's Hospital, and the Division of Hematology Oncology and BMT at The Ohio State University  Columbus Ohio"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elizabeth R.","family":"Lawlor","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Michigan  Ann Arbor Michigan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nadia","family":"Laack","sequence":"additional","affiliation":[{"name":"Department of Radiation Oncology Mayo Clinic  Rochester 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