Orbital dermatofibrosarcoma protuberans with frontal and ethmoid sinus involvement: A case report and brief review of literature

Abstract

Dermatofibrosarcoma protuberans is a soft tissue sarcoma that is dermal in origin. The incidence is <0.1% of all malignancies and 1% of soft tissue sarcoma. Most commonly, it involves trunk (62%) followed by extremities (25%) and head and neck (13%). It is a slow growing tumor with locally aggressive behavior. Here, a 50-year-old female diagnosed with orbital dermatofibrosarcoma developed extra-axial component in right frontal region even on chemotherapy. Hence, the bad prognostic factors are yet to be established in dermatofibrosarcoma protuberans.

Keywords

• Dermatofibrosarcoma protuberans
• imatinib
• locally aggressive
• sarcoma