Ocular perivascular epithelioid cell tumor: report of 2 cases with distinct clinical presentations

Perivascular epithelioid cell tumors comprise a rare and recently described family of neoplasms that characteristically coexpress melanocytic and myoid markers. We describe the clinicopathologic features of 2 ocular cases. Case 1 occurred in a 26-year-old woman with a recurrent left upper eyelid tumor, and case 2 was diagnosed in a 7-year-old boy with a left ciliary body mass. This is the first report of perivascular epithelioid cell tumor arising in the ciliary body or eyelid. Neither patient in our series had documented evidence of the tuberous sclerosis complex. Despite its rarity, perivascular epithelioid cell tumor should be considered in the differential diagnosis of ocular melanocytic lesions. Although most examples appear cytologically bland, experience is limited regarding their malignant potential; and therefore, complete surgical resection and close follow-up are recommended.