European Journal of Molecular & Clinical Medicine

Abstract

Dermatofibrosarcoma protuberans (DFSP) is a rare type of skin cancer which is locally aggressive. Slow growing fibrohistiocytic neoplasm considered of low to medium malignancy.  It starts in connective tissue cells in the middle layer of skin (dermis) and poorly circumscribed usually involves dermis and subcutis. DFSP might look like a pimple or feel like a rough patch of skin at first. It is a rare tumor with an incidence rate of 0.8 to 4.5 cases per million persons per year (2,3,4,5,6). Represents less than 5% of all soft tissue sarcomas. DFSP occurs most often in adults in 3^rd to 5^th decades of life but has been reported in all age groups, including congenital presentation. Tumour is usually positive for CD-34. Treatment consists of surgical resection with negative margins. In cases where surgical resection with negative margins is not possible, radiation and systemic therapy with tyrosine kinase inhibitors, such as imatinib mesylate, has been shown to be effective. Regular follow up of the patients is essential.