Desmoplastic small round cell tumors: Prognostic indicators and results of surgical management Journal Article

  


Authors: Schwarz, R. E.; Gerald, W. L.; Kushner, B. H.; Coit, D. G.; Brennan, M. F.; La Quaglia, M. P.
Article Title: Desmoplastic small round cell tumors: Prognostic indicators and results of surgical management
Abstract: Background: Desmoplastic small round cell tumors (DSRCT or DSCT) are rare aggressive cancers of adolescence and early adulthood. There are few reported series to guide clinical therapy. This study correlates survival with treatment variables, including aggressive surgical debulking. Methods: Thirty-two patients with documented DSRCT received treatment at our institution. Demographic, clinical, and treatment variables were correlated with progression-free survival using log-rank statistics. Results: Thirty patients were male (96%), and two were female (4%), with a median age at diagnosis of 22 years. The primary site of disease in 97% of cases was the abdomen or pelvis. Twenty-nine patients (91%) had extensive disease involving peritoneal surfaces, lymph nodes, or discontinuous organs. All 32 patients received systemic chemotherapy. Fifteen (47%) underwent tumor debulking greater than 90% at diagnosis or during therapy. A complete or very good response to therapy occurred in 13 patients, and depended on surgical removal of bulk disease in all. Thirteen patients remained progression-free, but three of these patients died from treatment toxicity. Improved survival was correlated with a complete or very good partial response to multimodality therapy, surgical debulking of more than 90% either before or after chemotherapy, and use of the P6 protocol. Conclusions: DSRCT is an aggressive cancer that occurs predominantly in young males. Improved survival is correlated with intense chemotherapy and aggressive resection.
Keywords: adolescent; adult; cancer survival; child; clinical article; human tissue; treatment outcome; survival analysis; cancer surgery; retrospective studies; human cell; clinical feature; antineoplastic agents; combined modality therapy; chemotherapy; laparotomy; metastasis; computer assisted tomography; postoperative complication; surgery; surgical procedures, operative; sex difference; resection; bacterial infection; small cell carcinoma; desmoplastic small round cell tumor; carcinoma, small cell; abdominal neoplasms; abdominal tumor; correlation function; humans; prognosis; human; male; female; article
Journal Title: Annals of Surgical Oncology
Volume: 5
Issue: 5
ISSN: 1068-9265
Publisher: Springer  
Date Published: 1998-07-01
Start Page: 416
End Page: 422
Language: English
DOI: 10.1007/bf02303860
PUBMED: 9718171
PROVIDER: scopus
DOI/URL:

https://www.scopus.com/inward/record.uri?eid=2-s2.0-0031819284&doi=10.1007%2fBF02303860&partnerID=40&md5=7cf22e22a556123320ef7755a7f31900
Notes: Article -- Export Date: 12 December 2016 -- Source: Scopus
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MSK Authors
  1. Murray F Brennan
    1059 Brennan
  2. Brian Kushner
    311 Kushner
  3. Michael P La Quaglia
    411 La Quaglia
  4. William L Gerald
    375 Gerald
  5. Daniel Coit
    542 Coit
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