A 71-year-old African American man presented with an indolent growth above the left medial canthus. CT of the orbits revealed a circumscribed, homogeneously enhancing 2.1 × 2.2 × 2.5 cm mass that appeared to extend into the medial orbit and proximal nasolacrimal duct without obstructing it. Intraoperatively, the mass was readily dissected free and excised in toto. Histopathology demonstrated spindle cells in a storiform pattern. Immunohistochemical staining was consistent with dermatofibrosarcoma protuberans (DFSP) although the t(17;22) translocation was absent. The patient underwent two-thirds of the recommended intensity-modulated radiation therapy dose before refusing further treatment. He declined additional interventions in favor of serial imaging. He remained recurrence free at 11 months. To the best of the authors' knowledge, this is the first case of recurrent DFSP invading the orbit managed with conservative resection and adjuvant therapy. A comprehensive review of the literature pertaining to DFSP of the orbit is presented.
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