A primary dermatofibrosarcoma protuberans of the orbit.
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Abstract
Dermatofibrosarcoma protuberans (DFSP) is an uncommon soft tissue sarcoma known for its local aggressiveness and high recurrence rate after surgical excision. While DFSP most frequently involves the trunk and extremities, rare cases of orbital DFSP have been reported.
Here we describe the case of a 40-year-old Asian male who presented with a slow-growing mass over the right lacrimal sac area. MRI revealed an enhancing mass in the anteromedial orbit abutting the globe and nasolacrimal duct. The excised lesion was firm and well-circumscribed. Histopathology showed pleomorphic spindle cells positive for CD34, S-100, and vimentin, confirming a diagnosis of DFSP. The surgical margin was involved. After exploring treatment options, the patient opted for adjuvant orbital radiation. He remained recurrence-free at 1 year follow-up.
This case highlights the challenge of diagnosing and treating DFSP arising in an unusual location like the orbit. The nonspecific clinical presentation can delay recognition, while the orbit's complex anatomy complicates achieving clear margins. A high index of suspicion, careful histopathologic examination with immunostains, and long-term monitoring are crucial for managing this rare orbital neoplasm. Advances in molecular diagnostics and targeted therapy are expanding treatment possibilities for orbital DFSP.
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