Ophthalmologic aspects of Dermatofibrosarcoma Protuberans: A systematic review in the context of a rare case of primary orbital involvement

Objective: To provide a systematic review on ophthalmologic presentations of dermatofibrosarcoma protuberans (DFSP) in conjunction with a case report of primary orbital involvement by this tumor. Methods: A thorough electronic search on PubMed, Medline, Scopus, EMBASE and web of science databases was performed. All available data from reported cases along with our described case were extracted and analyzed. Results: We describe a 66-year-old male with primary orbital DFSP who was managed by total excision of the tumor without recurrence after 18 months of follow-up. In the literature, 14 studies reporting 15 cases were identified and reviewed. Our review topics included epidemiology, clinical presentations, pathologic features, differential diagnosis, imaging, treatment and prognosis. Conclusion: Rarely, but crucially, ophthalmologists may encounter DFSP in the orbital or periorbital area. They should be familiar with the pathologic nature and treatment options for this tumor. This diagnosis should be kept in mind when approaching a case with a non-tender, nodular, firm, mass or more rarely an indenting orbital mass.