Orbitofacial dermatofibrosarcoma protuberans with intranasal extension

A 25-year-old Chinese woman presented with recurrent painless swelling over the left medial canthus region for 3 months and intranasal mass for an indeterminate duration. Initial incision biopsy of the mass was reported as nodular fasciitis but the lesion recurred 3 weeks later. Intraoperative findings during repeat biopsy showed a mass extending from the deep dermal tissue into the anterior orbit and polyp-like nasal mass. Histopathology findings were that of dermatofibrosarcoma protuberans (DFSP). The mass recurred 4 months later without orbital or intranasal recurrence. Wide excision biopsy under frozen section guidance was attempted however; clear surgical margins could not be achieved despite extensive resection. She was subsequently referred for adjuvant radiotherapy. We report an exceptionally rare case of local recurrence of DFSP in an unusual anatomic location. This case was surgically challenging in achieving negative margins, and thus neoadjuvant therapy may improve overall outcome to prevent local relapse.