Dermatofibrosarcoma protuberans

Cancer. 2004 Dec 1;101(11):2503-8. doi: 10.1002/cncr.20678.


In the current review, the authors set out to discuss the natural history and treatment of dermatofibrosarcoma protuberans (DFSP), a rare indolent cutaneous tumor. Approximately 10-15% of all DFSPs contain areas of fibrosarcoma (DFSP-FS), and such cases tend to exhibit more aggressive behavior. The optimal treatment for DFSP is resection with wide margins; the likelihood of local control associated with this procedure exceeds 90%. The probability of regional or distant metastases is </= 5%. Patients with positive or close surgical margins have an elevated risk of local recurrence after resection alone; however, postoperative radiotherapy results in local control rates of >/= 85% in such patients. Postoperative radiotherapy also is indicated in the rare event that a patient has unresectable macroscopic disease. Experience with the use of radiotherapy alone to treat macroscopic disease is limited.

Publication types

  • Review

MeSH terms

  • Dermatofibrosarcoma / pathology*
  • Dermatofibrosarcoma / radiotherapy
  • Dermatofibrosarcoma / surgery
  • Diagnosis, Differential
  • Disease Progression
  • Humans
  • Neoplasm Recurrence, Local*
  • Prognosis
  • Radiotherapy, Adjuvant
  • Risk Factors
  • Skin Neoplasms / pathology*
  • Skin Neoplasms / radiotherapy
  • Skin Neoplasms / surgery*