{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,14]],"date-time":"2026-09-14T07:46:35Z","timestamp":1789371995652,"version":"build-2803163510"},"reference-count":0,"publisher":"American Society of Clinical Oncology (ASCO)","issue":"5","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCO"],"published-print":{"date-parts":[[1996,5]]},"abstract":"PURPOSE<\/jats:title> To test intensive alkylator-based therapy in desmoplastic small round-cell tumor (DSRCT). <\/jats:p><\/jats:sec>PATIENTS AND METHODS<\/jats:title> Patients received the P6 protocol, which has seven courses of chemotherapy. Courses 1, 2, 3, and 6 included cyclophosphamide 4,200 mg\/m2, doxorubicin 75 mg\/m2, and vincristine (HD-CAV). Courses 4, 5, and 7 consisted of ifosfamide 9 g\/m2 and etoposide 500 mg\/m2 for previously untreated patients, or ifosfamide 12 g\/m2 and etoposide 1,000 mg\/m2 for previously treated patients. Courses started after neutrophil counts reached 500\/microL and platelet counts reached 100,000\/microL. Tumor resection was attempted. Post-P6 treatment options included radiotherapy and a myeloablative regimen of thiotepa (900 mg\/m2) plus carboplatin (1,500 mg\/m2), with stem-cell rescue. <\/jats:p><\/jats:sec>RESULTS<\/jats:title> Ten previously untreated and two previously treated patients have completed therapy. The male-to-female ratio was 11:1. Ages were 7 to 22 years (median, 14). The largest masses were infradiaphragmatic (n = 11) or intrathoracic (n = 1). Other findings included serosal implants (n = 11), regional lymph node invasion (n = 8), ascites or pleural effusion (n = 7), and metastases to liver (n = 5), lungs (n = 4), distant lymph nodes (n = 3), spleen (n = 2), and skeleton (n = 2). Tumors uniformly responded to HD-CAV, but there were no complete pathologic responses. One patient died at 1 month from tumor-related Budd-Chiari syndrome. Of seven patients who achieved a complete remission (CR), five remain in CR 9, 12, 13, 33, and 38 months from the start of P6, one patient died of infection at 12 months (autopsy-confirmed CR), and one patient relapsed 4 months off therapy. Of four patients who achieved a partial remission (PR), one remains progression-free at 34 months and three developed progressive disease. Five patients received local radiotherapy: three were not assessable for response, but in two patients, antitumor effect was evident. Four patients received thiotepa\/carboplatin: two were in CR and remain so, and two patients had measurable disease that did not respond. <\/jats:p><\/jats:sec>CONCLUSION<\/jats:title> For control of DSRCT, our experience supports intensive use of HD-CAV, aggressive surgery to resect visible disease, radiotherapy to high-risk sites, and myeloablative chemotherapy with stem-cell rescue in selected cases. <\/jats:p><\/jats:sec>","DOI":"10.1200\/jco.1996.14.5.1526","type":"journal-article","created":{"date-parts":[[2017,2,24]],"date-time":"2017-02-24T09:48:15Z","timestamp":1487929695000},"page":"1526-1531","source":"Crossref","is-referenced-by-count":295,"title":["Desmoplastic small round-cell tumor: prolonged progression-free survival with aggressive multimodality therapy."],"prefix":"10.1200","volume":"14","author":[{"given":"B H","family":"Kushner","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M P","family":"LaQuaglia","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"N","family":"Wollner","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P A","family":"Meyers","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"K L","family":"Lindsley","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"F","family":"Ghavimi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"T E","family":"Merchant","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"F","family":"Boulad","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"N K","family":"Cheung","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M A","family":"Bonilla","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G","family":"Crouch","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"suffix":"Jr","given":"J F","family":"Kelleher","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P G","family":"Steinherz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"W L","family":"Gerald","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"233","container-title":["Journal of Clinical Oncology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/ascopubs.org\/doi\/pdfdirect\/10.1200\/JCO.1996.14.5.1526","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,5,25]],"date-time":"2022-05-25T22:07:15Z","timestamp":1653516435000},"score":1,"resource":{"primary":{"URL":"https:\/\/ascopubs.org\/doi\/10.1200\/JCO.1996.14.5.1526"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1996,5]]},"references-count":0,"journal-issue":{"issue":"5","published-print":{"date-parts":[[1996,5]]}},"alternative-id":["10.1200\/JCO.1996.14.5.1526"],"URL":"https:\/\/doi.org\/10.1200\/jco.1996.14.5.1526","relation":{},"ISSN":["0732-183X","1527-7755"],"issn-type":[{"value":"0732-183X","type":"print"},{"value":"1527-7755","type":"electronic"}],"subject":[],"published":{"date-parts":[[1996,5]]}}}