SURGICAL-TREATMENT OF DERMATOFIBROSARCOMA PROTUBERANS

Dermatofibrosarcoma protuberans is a rare dermal tumor that recurs aft er inadequate primary treatment. In a retrospective study, we analyzed the outcomes of 117 patients (mean age 39 years) treated surgically f or a dermatofibrosarcoma protuberans. In most cases (107 patients), su rgery was performed according to a protocol of taking wide peripheral resection margins of 5 cm and by resecting a disease-free anatomic zon e deep to the lesion. The mean follow-up was 61 months. The results su ggest a difference in prognosis between patients treated primarily wit h wide initial resection and those referred secondarily with recurrent disease following previous treatment by narrow resection margins. The re was no recurrent disease in the 66 patients treated primarily by wi de peripheral (5 cm) and deep resection of the tumor. Of the 41 patien ts referred secondarily at the time of recurrence, 2 developed further local disease within a year, despite equally aggressive local treatme nts. Both patients eventually died of metastatic fibrosarcoma. We emph asize the value of a highly aggressive local resection in the primary treatment of dermatofibrosarcoma protuberans to minimize local recurre nce and potential malignant transformation. Modern reconstructive tech niques provide satisfactory solutions for defects of almost any size a nd composition. Our findings suggest that radical primary resection of dermatofibrosarcoma protuberans in conjunction with immediate reconst ruction of the primary defect provides the best outcome for the patien t.