A Rare Orbital Pathology: A Large Orbital Dermatofibrosarcoma Protuberans

Abstract

Dermatofibrosarcoma protuberans (DFSP) is a rare sarcoma of the dermis. It is a malignant, locally aggressive, and infiltrative tumor with frequent recurrence. In this case, a 44-year-old woman presented with a 15-year history of a swelling in the medial canthus of the right eye that caused tearing. Imaging revealed a septated mass isodense to soft tissue that had eroded the medial wall of the orbit. Macroscopic examination showed an elastic, gray-brown, encapsulated, irregular mass measuring 45x35x22 mm. The surgical margins were positive, so adjuvant radiotherapy was started. The patient was followed for 2 years without recurrence. According to the literature, this mass is the largest orbital DFSP treated by globe-sparing primary resection.