Giant Dermatofibrosarcoma Protuberans With Bilateral Orbital Involvement

Dermatofibroma sarcoma protuberans (DFSP) is a rare, locally aggressive soft tissue sarcoma with a tendency for recurrence after excision. Although reports of unilateral orbital and bilateral eyelid disease exist, there have been no prior reports of DFSP with bilateral orbital involvement and no previously described cases of DFSP associated with transient optic neuropathy. The authors present a case report of a 34-year-old woman with a giant scalp DFSP involving the bilateral orbits. Despite radical resection with 5 cm margins where possible, multiple positive margins remained including deep positive margins at the bilateral superomedial retroseptal soft tissue. The patient completed adjuvant radiation for surgically unresectable disease. This case highlights the challenge of achieving local control given the disease extent and infiltration of the bilateral eyelids and orbits. This is the first reported case of DFSP with bilateral orbital involvement and associated transient optic neuropathy. Accepted for publication November 29, 2018. The authors have no financial or conflicts of interest to disclose. Presented at the American Society of Ophthalmic Plastic and Reconstructive Surgery, 48th Annual Fall Scientific Symposium on November 9–10, 2017 in New Orleans, LA. Address correspondence and reprint requests to Neda Esmaili, M.D., Eye Institute, Medical College of Wisconsin, 925 North 87th Street, Milwaukee, WI 53226. E-mail: nesmaili@mcw.edu © 2019 by The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc., All rights reserved.

from #Head and Neck by Sfakianakis via simeraentaxei on Inoreader http://bit.ly/2GeYYhh