Giant Dermatofibrosarcoma Protuberans With Bilateral Orbital Involvement. Issue 2 (March 2019)

• Record Type: Journal Article
• Authors: Liou, Victor; Chisholm, Smith Ann; Logunova, Valentina; Havlik, Robert; Esmaili, Neda
• Is Part Of: Ophthalmic plastic and reconstructive surgery. Volume 35:Issue 2(2019)
• Publication Date: 2019-03
• Subjects: Eye -- Surgery -- Periodicals; Ophthalmic plastic surgery -- Periodicals; Reconstructive Surgical Procedures -- Periodicals; Ophthalmologic Surgical Procedures -- Periodicals; Ophthalmology -- Periodicals; Surgery -- Periodicals; 617.70592
• DOI: 10.1097/IOP.0000000000001308
• Languages: English
• ISSNs: 0740-9303

Abstract

Abstract : 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. Abstract : The authors present the first known case of a dermatofibrosarcoma protuberans with bilateral orbital involvement and transient optic neuropathy.