Abstract
Dermatofibrosarcoma Protuberans (DFSP) is a slow-growing, low-grade, malignant fibroblastic mesenchymal tumor that arises from the dermis and invades deeper tissues. The precise origin of DFSP is not well known but evidence hints that the cellular origin is fibroblastic, histiocytic, or neuroectodermal.Cytogenetic abnormalities have been found in patients with DFSP, such as reciprocal translocations of chromosomes 17 and 22, t(17;22), and supernumerary ring chromosomes composed of interspersed sequences from bands 17 (17q22) and 22 (22q12). It is a relatively uncommon soft tissue neoplasm with an estimated incidence of 4.2 to 4.5 cases per million persons per year in the United States. DFSP may present as an asymptomatic, skin-colored plaque with possible dark red or blue discoloration. Clinical suspicion is confirmed by biopsy. Histologically, DFSP shows a storiform or fascicular proliferation of bland spindled cells that extend from the dermis into the subcutaneous tissues. Almost all cases of DFSP are CD34-positive (Figure 1) and factor XIIIa- negative. The treatment of choice for a DFSP is wide local excision. Every effort should be made to completely remove the tumor at the time of initial operation, considering the proclivity that DFSP has for irregular and frequently deep subclinical extensions. The margins of resection vary in the medical literature anywhere between with 2 to 4 cm. Imatinib mesylate was approved by the FDA for the treatment of unresectable, recurrent, and/or metastatic DFSP. We are reporting a case of a 28-year old male patient with a DFSP treated by our multidisciplinary team.
Citation
Arrangoiz R, Cordera F, Caba D, Muñoz M, Moreno E and de Leon EL. Dermatofibrosarcoma Protuberans (DFSP): Case Report and Literature Review. SM J Surg. 2017; 3(1): 1010.