Desmoplastic small round cell tumor: review of therapy including surgery followed by continuous hyperthermic peritoneal perfusion of chemotherapy

  • Andrea Hayes-Jordan | ahjordan@mdanderson.org University of Texas MD Anderson Cancer Center, Houston, United States.
  • Peter Anderson University of Texas MD Anderson Cancer Center, Houston, United States.

Abstract

Desmoplastic small round cell tumor (DSRCT) is a very rare disease of children, adolescents, and young adults and involves the abdominal cavity. DSRCT has characteristic fusion gene involving EWS1 and WT1 translocation, t(11;22)(p13;q12). Unlike Ewing’s sarcoma of bone, DSRCT usually presents with diffuse peritoneal implants that are prone to recur. The primary organ of origin of DSRCT is mesenchyme of the peritoneum. This makes it a very unique tumor that is difficult to treat because of the infiltrative and diffuse nature of the peritoneum. The challenge of local control is to remove dozens to hundreds of tumors studding the peritoneal cavity, and then eliminate microscopic disease. We review a sequential multimodality strategy to reduce macroscopic and microscopic disease including neoadjuvant chemotherapy, aggressive surgery including an emerging new therapy to use after surgery to treat microscopic residual disease: continuous hyperthermic peritoneal chemotherapy,

Dimensions

Altmetric

PlumX Metrics

Downloads

Download data is not yet available.
Published
2011-12-14
Info
Issue
Section
Reviews
Keywords:
Desmoplastic small round cell tumor - Ewing’s family of tumors - Pediatric surgery - Hyperthermia - Intraperitoneal chemotherapy - Peritoneal metastases
Statistics
  • Abstract views: 1214

  • FULL TEXT: 182
How to Cite
Hayes-Jordan, A., & Anderson, P. (2011). Desmoplastic small round cell tumor: review of therapy including surgery followed by continuous hyperthermic peritoneal perfusion of chemotherapy. Oncology Reviews, 3(3), 195-200. https://doi.org/10.4081/oncol.2009.94