How is dermatofibrosarcoma protuberans (DFSP) treated?

Updated: Mar 06, 2020
  • Author: Raman K Madan, MD; Chief Editor: William D James, MD  more...
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Answer

Currently, conventional chemotherapy is rarely used in the treatment of dermatofibrosarcoma protuberans (DFSP). Limited case reports have not shown any significant value of conventional chemotherapy in the treatment of DFSP. [30, 31]

Radiation therapy (RT) has had a limited role in the past, but, recently, it has been used as an adjunct to surgery. Radiation therapy may be recommended for patients if the margins of resection are positive or for situations in which adequate wide excision alone may result in major cosmetic or functional deficits. Postoperative adjuvant RT may reduce the risk of recurrence when clear surgical margins are not confident. [32] The complete radiation therapy dose ranges from 50-70 Gy. Overall, the risk of severe complications from RT is low. Close follow-up care after radiation therapy is warranted because some DFSP tumors may become more aggressive. [1, 14, 33, 34]

Based on the knowledge that constitutively activated PDGFB-PDGFR-beta signaling pathway plays a central role in the proliferation of DFSP tumor cells, the development of molecularly targeted therapy holds promise as an additional treatment option. [35] Originally approved for the treatment of chronic myelogenic leukemia, imatinib mesylate has been found to have significant therapeutic value in the treatment of DFSP. [36] Imatinib is a potent and specific inhibitor of several protein-tyrosine kinases, including the platelet-derived growth factor (PDGF) receptors. [5, 37]

On October 19, 2006, the US Food and Drug Administration granted approval for imatinib mesylate (Gleevec) as a single agent for the treatment of DFSP. Imatinib mesylate is indicated for the treatment of adult patients with unresectable, recurrent, and/or metastatic DFSP. The recommended oral dose is 800 mg/d. [16, 38]

With limited clinical data to date, a response rate of approximately 65% has been achieved among DFSP patients treated with imatinib. A small subset of DFSP patients lacking the classic t(17,22) gene aberration seems to have no response to imatinib. [1]

Neoadjuvant imatinib therapy for DFSP has been proposed in recent studies. [25, 39] Using imatinib as a preoperative therapy agent in locally advanced or recurrent DFSP may decrease tumor load, promote tumor cell apoptosis, and subsequently reduce the extent of surgery. Caution should be used when applying such a therapeutic strategy, because the potential exists for creating a skip area wherein discontiguous tumor may obscure the accurate pathology assessment of surgical margins.


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