How is dermatofibrosarcoma protuberans (DFSP) treated?

Updated: Oct 07, 2019
  • Author: Guy J Petruzzelli, MD, PhD, MBA, FACS; Chief Editor: Gregory Gary Caputy, MD, PhD, FICS  more...
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Answer

Answer

Treatment

DFSP is a difficult lesion to treat because of the high rate of recurrences. Surgical excision requires both proper diagnosis at presentation and effective treatment.

DFSP on the face can be removed successfully with wide surgical excision and closure with rotational flaps. [93]

DFSP lesions smaller than 2 cm in diameter are often mistakenly identified as DFs or keloids. [94] D'Andrea and colleagues further argue for aggressive surgical treatment with excision of 5 cm of surrounding tissue in order to prevent local recurrences.

In a small retrospective study, Sun and colleagues concluded that radiation as adjuvant therapy helps reduce the rate of local recurrences. [95]

Originally effective for inhibiting the BCR/ABL fusion product in patients with chronic myelogenous leukemia (CML), the drug STI571 (Gleevac) has also been used to inhibit other tyrosine kinases, including c-kit and the platelet-derived growth factor receptors. Because the translocation in DFSP is associated with constitutive activation of the platelet-derived growth factor receptor, several labs have used tissue culture and animal models to demonstrate that Gleevac is able to inhibit proliferation of cells derived from DFSP or cells that express the COL1A1-PDGFB fusion product. [96, 97] These results are encouraging and may lead to the future use of Gleevac as either neoadjuvant or adjuvant therapy for DFSP.

Reports have also supported the use of imatinib mesylate. [98]


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