Dermatofibrosarcoma Protuberans

Dermatofibrosarcoma protuberansDermatofibrosarcoma Protuberans (DFSP) is a slow growing rare soft tissue sarcoma with unpredictable subclinical extension. Although this malignant tumor is not specific to a specialized anatomic site, the processing of a DFSP specimen presents many challenges given large specimen size with extensive subcutaneous tissue. DFSPs account for an estimated 0.1% of all cancers with 42% of all cases occurring in 20–39 age range with a slightly higher incidence among women. DFSPs are most commonly located on the trunk and extremities and present as ill-defined large indurated plaques with minimal surface change. Histologically DFSP is a dermal proliferation of spindle cells with diffuse infiltration into the subcutis in a honeycomb pattern. Excising DFSP with Mohs surgery offers comprehensive margin control, and meticulous mapping of tumor margins is necessary to achieve high cure rates and tissue preservation.