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

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

Keywords

Desmoplastic small round cell tumor - Ewing’s family of tumors - Pediatric surgery - Hyperthermia - Intraperitoneal chemotherapy - Peritoneal metastases

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Submitted: 2011-12-14 11:23:12
Published: 2011-12-14 00:00:00
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Copyright (c) 2011 Andrea Hayes-Jordan, Peter Anderson

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