Updates on the multimodality management of desmoplastic small round cell tumor

J Surg Oncol. 2012 May;105(6):617-21. doi: 10.1002/jso.22130. Epub 2012 Jan 3.

Abstract

Desmoplastic Small Round Cell Tumor (DSRCT) is a rare and an aggressive malignancy with poor outcome. This tumor can co-express epithelial, neural, and mesenchymal markers. The molecular hallmark of DSRCT is the EWS-WT1 fusion protein. Despite the diversities in treatment modality, the best results have been seen with radical surgery and adjuvant or neoadjuvant chemotherapy.

Publication types

  • Review

MeSH terms

  • Abdominal Neoplasms / genetics
  • Abdominal Neoplasms / mortality
  • Abdominal Neoplasms / pathology
  • Abdominal Neoplasms / therapy*
  • Antibodies, Monoclonal / therapeutic use
  • Chemotherapy, Adjuvant
  • Chemotherapy, Cancer, Regional Perfusion
  • Desmoplastic Small Round Cell Tumor / genetics
  • Desmoplastic Small Round Cell Tumor / mortality
  • Desmoplastic Small Round Cell Tumor / pathology
  • Desmoplastic Small Round Cell Tumor / therapy*
  • Humans
  • Hyperthermia, Induced
  • Immunohistochemistry
  • Neoplasm Recurrence, Local
  • Neoplasm Staging
  • Oncogene Proteins, Fusion / genetics
  • Radiotherapy, Adjuvant
  • Tomography, X-Ray Computed
  • Translocation, Genetic

Substances

  • Antibodies, Monoclonal
  • EWS1-WT1 fusion protein, human
  • Oncogene Proteins, Fusion