Dermatofibrosarcoma protuberans: a surgical disease with a molecular savior.

PURPOSE OF REVIEW: Dermatofibrosarcoma protuberans is a low-grade malignancy of the skin and subcutaneous tissues with low potential for the development of distant metastases. This tumor is characterized by infiltrative growth, and a chromosomal translocation involving the COL1A1 gene on chromosome 17 and the gene platelet-derived growth factor B gene on chromosome 22. This review will examine recent data confirming the central role of surgery in managing this disease and new findings for the application of molecularly targeted therapy in patients with unresectable disease. RECENT
FINDINGS: The largest published series of dermatofibrosarcoma protuberans cases has reported that surgery can lead to excellent local control in dermatofibrosarcoma protuberans in over 95% of cases. In patients with metastatic disease, or with locally advanced disease not suitable for surgical excision, inhibition of platelet-derived growth factor receptors with imatinib (Gleevec) can lead to striking clinical results, including complete responses in some patients.
SUMMARY: The identification of platelet-derived growth factor B as a near universal translocation partner in chromosomal rearrangements in dermatofibrosarcoma protuberans has led to the successful application of therapy targeted at platelet-derived growth factor receptors. Although wide surgical excision remains the standard of care, patients with locally advanced disease not suitable for wide surgical excision or with metastatic disease can be managed with the platelet-derived growth factor receptors inhibitor imatinib with high probability of response.