{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,2]],"date-time":"2026-10-02T17:59:11Z","timestamp":1790963951302,"version":"4.1.0"},"reference-count":19,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2018,6,7]],"date-time":"2018-06-07T00:00:00Z","timestamp":1528329600000},"content-version":"vor","delay-in-days":6,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Journal of Surgical Oncology"],"published-print":{"date-parts":[[2018,6]]},"abstract":"<jats:sec><jats:title>Background and Objectives<\/jats:title><jats:p>Desmoplastic small round cell tumor (DSRCT) is a rare peritoneal surface malignancy. Current research is limited by the scarcity of this disease.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Patients with DSRCT were identified in the 2004\u20102014 NCDB. Factors affecting overall survival (OS) were assessed. Additionally, trends were examined based on the volume of cases treated at individual facilities.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>A total of 125 patients were identified with a median age of 21 (IQR 15\u201027). Six had extra\u2010abdominal disease and 15 (12%) had liver involvement. Median OS was 28 months. Systemic chemotherapy (HR 0.4, <jats:italic>P<\/jats:italic>\u2009=\u20090.015) and surgery (HR 0.6, <jats:italic>P\u2009<\/jats:italic>=\u20090.047) were associated with reduced mortality. For the 74 patients undergoing surgery, absence of liver involvement and receipt of postoperative chemotherapy were associated with improved OS on univariate analysis. On multivariable analysis, two factors approached significance: adjuvant chemotherapy was associated with a reduced risk of mortality (HR 0.3, <jats:italic>P<\/jats:italic>\u2009=\u20090.073) and residual macroscopic disease after resection correlated with increased risk of mortality (HR 5.3, <jats:italic>P<\/jats:italic>\u2009=\u20090.071). High\u2010volume facilities (\u22655 cases) experienced improved OS (median 59.1 vs 28.8 months), albeit not significantly (<jats:italic>P<\/jats:italic>\u2009=\u20090.135), compared to low\u2010volume centers.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Despite multimodal treatment, DSRCT is associated with dismal outcomes. Facilities familiar with treating this uncommon disease may experience superior outcomes.<\/jats:p><\/jats:sec>","DOI":"10.1002\/jso.25071","type":"journal-article","created":{"date-parts":[[2018,6,7]],"date-time":"2018-06-07T14:30:29Z","timestamp":1528381829000},"page":"1759-1767","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":43,"title":["Desmoplastic small round cell tumor: A nationwide study of a rare sarcoma"],"prefix":"10.1002","volume":"117","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6393-5079","authenticated-orcid":false,"given":"Zachary E.","family":"Stiles","sequence":"first","affiliation":[{"name":"Division of Surgical Oncology Department of Surgery University of Tennessee Health Science Center Memphis Tennessee"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paxton V.","family":"Dickson","sequence":"additional","affiliation":[{"name":"Division of Surgical Oncology Department of Surgery 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