Desmoplastic small round cell tumor

A nationwide study of a rare sarcoma

Zachary E. Stiles, Paxton V. Dickson, Evan Glazer, Andrew J. Murphy, Andrew M. Davidoff, Stephen W. Behrman, Michael W. Bishop, Michael Martin, Jeremiah Deneve

Research output: Contribution to journalArticle

3 Citations (Scopus)

Abstract

Background and Objectives: Desmoplastic small round cell tumor (DSRCT) is a rare peritoneal surface malignancy. Current research is limited by the scarcity of this disease. Methods: Patients with DSRCT were identified in the 2004-2014 NCDB. Factors affecting overall survival (OS) were assessed. Additionally, trends were examined based on the volume of cases treated at individual facilities. Results: A total of 125 patients were identified with a median age of 21 (IQR 15-27). Six had extra-abdominal disease and 15 (12%) had liver involvement. Median OS was 28 months. Systemic chemotherapy (HR 0.4, P = 0.015) and surgery (HR 0.6, P = 0.047) 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, P = 0.073) and residual macroscopic disease after resection correlated with increased risk of mortality (HR 5.3, P = 0.071). High-volume facilities (≥5 cases) experienced improved OS (median 59.1 vs 28.8 months), albeit not significantly (P = 0.135), compared to low-volume centers. Conclusion: Despite multimodal treatment, DSRCT is associated with dismal outcomes. Facilities familiar with treating this uncommon disease may experience superior outcomes.

Original languageEnglish (US)
Pages (from-to)1759-1767
Number of pages9
JournalJournal of Surgical Oncology
Volume117
Issue number8
DOIs
StatePublished - Jun 15 2018

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Desmoplastic Small Round Cell Tumor
Sarcoma
Survival
Mortality
Drug Therapy
Combined Modality Therapy
Liver
Adjuvant Chemotherapy
Research
Neoplasms

All Science Journal Classification (ASJC) codes

  • Surgery
  • Oncology

Cite this

Stiles, Z. E., Dickson, P. V., Glazer, E., Murphy, A. J., Davidoff, A. M., Behrman, S. W., ... Deneve, J. (2018). Desmoplastic small round cell tumor: A nationwide study of a rare sarcoma. Journal of Surgical Oncology, 117(8), 1759-1767. https://doi.org/10.1002/jso.25071

Desmoplastic small round cell tumor : A nationwide study of a rare sarcoma. / Stiles, Zachary E.; Dickson, Paxton V.; Glazer, Evan; Murphy, Andrew J.; Davidoff, Andrew M.; Behrman, Stephen W.; Bishop, Michael W.; Martin, Michael; Deneve, Jeremiah.

In: Journal of Surgical Oncology, Vol. 117, No. 8, 15.06.2018, p. 1759-1767.

Research output: Contribution to journalArticle

Stiles, ZE, Dickson, PV, Glazer, E, Murphy, AJ, Davidoff, AM, Behrman, SW, Bishop, MW, Martin, M & Deneve, J 2018, 'Desmoplastic small round cell tumor: A nationwide study of a rare sarcoma', Journal of Surgical Oncology, vol. 117, no. 8, pp. 1759-1767. https://doi.org/10.1002/jso.25071
Stiles ZE, Dickson PV, Glazer E, Murphy AJ, Davidoff AM, Behrman SW et al. Desmoplastic small round cell tumor: A nationwide study of a rare sarcoma. Journal of Surgical Oncology. 2018 Jun 15;117(8):1759-1767. https://doi.org/10.1002/jso.25071
Stiles, Zachary E. ; Dickson, Paxton V. ; Glazer, Evan ; Murphy, Andrew J. ; Davidoff, Andrew M. ; Behrman, Stephen W. ; Bishop, Michael W. ; Martin, Michael ; Deneve, Jeremiah. / Desmoplastic small round cell tumor : A nationwide study of a rare sarcoma. In: Journal of Surgical Oncology. 2018 ; Vol. 117, No. 8. pp. 1759-1767.
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