Dermatofibrosarcoma Protuberans: A Surgeon's Enigma

Abstract

Dermatofibrosarcoma protuberans (DFSP) is a soft-tissue tumor arising from the dermis. It is a rare tumor, but the important point is that it has a higher incidence of local recurrence than other tumors. Management is primarily by wide local excision (WLE) with tumor negative margin (R0 resection) or Mohs micrographic surgery (MMS). In our case, a 57-year-old male patient presented with an anterior abdominal wall ulcerated mass. The patient had undergone surgery for the excision of the mass, twice before, at a different health-care facility. An incisional biopsy of the mass done at our hospital revealed it to be DFSP. The patient was treated by WLE with flap reconstruction. Post-operative histopathology examination (HPE) report confirmed DFSP with tumor-free margins (R0 resection). So if we fail to diagnose and manage DFSP correctly from the other commonly occurring tumors in the initial stages, there are very high chances of recurrence, and this causes significant morbidity to the patients.

Keywords: chemoradiotherapy (chemo-rt); dermatofibrosarcoma protuberans; immunohistochemistry (ihc); local recurrence; wide excision.

Figure 1. Pre-operative appearance of the tumor

Figure 2. Spindle-shaped tumor cells arranged in fascicle and stratiform pattern as seen under 10× magnification in biopsy

Figure 3. Wide local excision of the tumor, R0 resection

Figure 4. Flap coverage of tumor area

Figure 5. IHC staining showing tumor cells positive for CD34

IHC, immunohistochemistry; CD34, cluster of differentiation 34