ABSTRACT
Epithelioid sarcoma is a rare soft tissue neoplasm of uncertain differentiation that typically affects the distal extremities. Primary orbital epithelioid sarcoma is rare, and no reports exist characterizing metastases of this tumor to the orbit and ocular adnexa. In this article, the authors describe a rare case of eyelid metastasis in a 47-year-old man with epithelioid sarcoma of the right fibula diagnosed 16 months earlier who was otherwise doing well on the adjuvant tazemetostat therapy. In addition, the authors perform a retrospective review of cases of primary orbital epithelioid sarcoma reported in the literature, of which 4 patients responded favorably to surgical excision whereas 2 others eventually died as a direct result of their disease.
