Dermatofibrosarcoma protuberans.
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.
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000368:Aged; D002648:Child; D003105:Colombia; D005260:Female; D005354:Fibrosarcoma / Q000175:diagnosis* / Q000453:epidemiology / Q000601:surgery; D006801:Humans; D008297:Male; D008854:Microscopy, Electron; D008875:Middle Aged; D012737:Sex Factors; D012867:Skin / Q000473:pathology / Q000648:ultrastructure; D012878:Skin Neoplasms / Q000175:diagnosis* / Q000453:epidemiology / Q000601:surgery