Desmoplastic small round cell tumor: A nationwide study of a rare sarcoma

J Surg Oncol. 2018 Jun;117(8):1759-1767. doi: 10.1002/jso.25071. Epub 2018 Jun 7.


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.

Keywords: carcinomatosis; desmoplastic small round cell tumor; national cancer database; peritoneum.

MeSH terms

  • Adult
  • Bone Neoplasms / secondary
  • Chemotherapy, Adjuvant
  • Databases, Factual
  • Desmoplastic Small Round Cell Tumor / mortality*
  • Desmoplastic Small Round Cell Tumor / pathology*
  • Desmoplastic Small Round Cell Tumor / therapy
  • Female
  • Humans
  • Liver Neoplasms / mortality
  • Liver Neoplasms / pathology
  • Liver Neoplasms / therapy
  • Lung Neoplasms / secondary
  • Male
  • Neoplasm, Residual
  • Peritoneal Neoplasms / mortality*
  • Peritoneal Neoplasms / pathology*
  • Peritoneal Neoplasms / therapy
  • Radiotherapy, Adjuvant
  • Rare Diseases
  • United States / epidemiology
  • Young Adult