Dermatofibrosarcoma protuberans

Abstract

In view of the important clinicopathologic, evolutional, and ultrastructural characteristics of dermatofibrosarcoma Protuberans, a review was made of 30 patients seen during the last 25 years in Colombia's Instituto Nacional de Cancerologia. The incidence is low, accounting for 0.06% of all malignant tumors and 1.17% of all soft-parts sarcomas. The series comprises 19 women and 11 men, with the highest incidence found between ages of 30 and 50 years, although the study includes two children, ages 8 and 13. The chest was the most common site. Forty percent of the lesions were multinodular, and 23.3% showed ulceration. Histologic appearance is characterized in all cases by fusiform cells arranged like spokes around an empty hub. The fibroblastic origin was ascertained by electron-microscopic studies. The treatment used was broad surgical excision, removing tissue down to the aponeurosis to avoid recurrence.