{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,15]],"date-time":"2026-10-15T13:11:10Z","timestamp":1792069870793,"version":"4.4.0"},"reference-count":36,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2023,3,23]],"date-time":"2023-03-23T00:00:00Z","timestamp":1679529600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Cancer Medicine"],"published-print":{"date-parts":[[2023,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Objective<\/jats:title><jats:p>To report on a retrospective study of primary DSRCT aiming at characterizing long\u2010term survivors (LTS).<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>All consecutive patients treated at our institution for a primary DSRCT between 2000 and 2021 were retrospectively identified. Patients received multiagent chemotherapy \u00b1 surgery \u00b1 hyperthermic intraperitoneal chemotherapy (HIPEC)\u2009\u00b1\u2009whole abdomino\u2010pelvic radiotherapy (WAP\u2010RT)\u2009\u00b1 high\u2010dose chemotherapy \u00b1 maintenance chemotherapy (MC). Event\u2010free survival (EFS) and overall survival (OS) were estimated by Kaplan\u2013Meier method. Patients alive, without evidence of disease at \u226536\u2009months from diagnosis, were defined as LTS.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Thirty\u2010eight patients were identified. All received multiagent chemotherapy; 27\/38 (71%) surgery (7\/27 [26%] plus HIPEC), 9\/38 (24%) WAP\u2010RT, 12\/38 (32%) MC. At a median\u2010follow\u2010up of 37\u2009months (IQR 18\u201363), overall median\u2010EFS and median\u2010OS were 15 and 37\u2009months, respectively. All events occurred within 35\u2009months. In patients who underwent surgery, median\u2010EFS and median\u2010OS were 19 and 37\u2009months (23 and 43\u2009months after R0\/R1, and 10 and 19\u2009months after R2 resection), respectively. LTS were 5\/38 (13%), alive at 37, 39, 53, 64, 209\u2009months. None had liver or extra\u2010abdominal metastasis at diagnosis, they all received R0\/R1 resection, 3\/5 had WAP\u2010RT, 2\/5 MC, 1\/5 received high\u2010dose chemotherapy, none HIPEC.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>In our series cure was likely achieved in 13% of DSRCT. LTS had no liver\/extra\u2010abdominal disease, were treated with complete surgery, and possibly WAP\u2010RT\/MC.<\/jats:p><\/jats:sec>","DOI":"10.1002\/cam4.5829","type":"journal-article","created":{"date-parts":[[2023,3,23]],"date-time":"2023-03-23T13:51:01Z","timestamp":1679579461000},"page":"10694-10703","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":13,"title":["Long\u2010term survivors with desmoplastic small round cell tumor (<scp>DSRCT<\/scp>): Results from a retrospective single\u2010institution case series analysis"],"prefix":"10.1002","volume":"12","author":[{"given":"Claudia","family":"Giani","sequence":"first","affiliation":[{"name":"Department of Medical Oncology Fondazione IRCCS Istituto Nazionale dei Tumori  Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefano","family":"Radaelli","sequence":"additional","affiliation":[{"name":"Department of Surgery Fondazione IRCCS Istituto 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