Third-line trabectedin for a metastatic desmoplastic small round cell tumour treated with multimodal therapy

Anticancer Res. 2014 Jul;34(7):3683-8.


Background: Desmoplastic small round cell tumour (DSRCT) is a rare and aggressive cancer that usually develops in the peritoneal cavity of young males. Its prognosis is dismal, with current treatment options including the combination of multi-agent chemotherapy, aggressive surgery, radiation therapy, and autologous stem cell transplantation. Hyperthermic intraperitoneal chemotherapy (HIPEC) may also be an option.

Case report: Herein we report the administration of the marine-derived multi-target antineoplastic agent, trabectedin, in a patient with DSRCT, heavily pre-treated with conventional multi-agent chemotherapy, HIPEC, and surgery.

Results: The patient achieved a prolonged partial response and an extended period of stable disease with third-line trabectedin, following disease progression after conventional multi-agent chemotherapy, HIPEC, and surgery.

Conclusion: Trabectedin may be a treatment option in multimodal therapy for the management of DSRCT and warrants further research to explore the impact of trabectedin in the treatment of this disease.

Keywords: Chemotherapy; desmoplastic small round cell tumour; multimodal therapy; trabectedin.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Antineoplastic Agents, Alkylating / therapeutic use*
  • Desmoplastic Small Round Cell Tumor / drug therapy*
  • Dioxoles / therapeutic use*
  • Humans
  • Male
  • Tetrahydroisoquinolines / therapeutic use*
  • Trabectedin
  • Young Adult


  • Antineoplastic Agents, Alkylating
  • Dioxoles
  • Tetrahydroisoquinolines
  • Trabectedin