Clinical features and outcomes of 20 patients with abdominopelvic desmoplastic small round cell tumor

F. A. Angarita, S. Hassan, A. J. Cannell, B. C. Dickson, R. A. Gladdy, C. J. Swallow, A. Gupta, M. E. Blackstein, J. A. McCart

Research output: Contribution to journalArticlepeer-review

Abstract

Introduction Desmoplastic small round cell tumor (DSRCT) is a rare mesenchymal malignancy. We describe our experience with treating DSRCT at a large sarcoma referral center. Methods A retrospective chart review was performed on DSRCT patients referred to our institution (1998–2014). Pathology specimens were reviewed to confirm the diagnosis. Clinical and imaging were extracted and summarized with descriptive statistics. Univariate analysis was performed to evaluate the association between patient, tumor, and treatment variables and overall survival (OS). Results In this study cohort of 20 patients, median age at presentation was 29 y (range 18–43) and 90% were male. Fifty-five percent presented with metastasis. Patients underwent chemotherapy (n = 20), radiation therapy (n = 3), and cytoreductive surgery (CRS) (n = 5). Median OS was 22 m (interquartile range: 12–28 m). Five-year OS rate was 20%. Extra-abdominal metastasis was associated with a higher hazard ratio (HR) of mortality (HR: 3.1, 95% C.I. 1.0–9.4, p = 0.04), while CRS improved OS (HR: 0.1, 95% C.I. 0.03–0.7, p = 0.02). Conclusions Despite aggressive treatment, less than half of the patients were dead of DSRCT within 2 years of presentation. Although a select group of patients who underwent CRS had improved OS, novel treatments are urgently needed.

Original languageEnglish (US)
Pages (from-to)423-431
Number of pages9
JournalEuropean Journal of Surgical Oncology
Volume43
Issue number2
DOIs
StatePublished - Feb 1 2017

Keywords

  • Chemotherapy
  • Desmoplastic small round cell tumor
  • Multimodal management
  • Small round blue cell tumors
  • Surgery

ASJC Scopus subject areas

  • Surgery
  • Oncology

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