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Dermatofibrosarcoma protuberans is a rare malignancy comprising approximately one percent of all sarcomas and approximately less than 0.1% of malignancies. Although rarely metastatic, significant tissue loss and deformity can occur with wide local excision. The mass tends to be slow growing and disregarded until significant growth or skin changes occur. Recent advances in molecular diagnostics have led to the discovery of a translocation of COL1A1 and PDGFRβ (17q22;22q13) and thusly the implementation of treatment with Imitinib mesylate (GLEEVEC TM). In several small case series and phase II trials, imatinib has shown promise with recurrent and unresectable disease. Radiotherapy is useful in cases with microscopically positive margins to achieve local control. [ABSTRACT FROM AUTHOR]