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Clinicopathologic Reports, Case Reports, and Small Case Series
December 2003

Intraocular Melanocytoma in Association With Bone Formation

Arch Ophthalmol. 2003;121(12):1791-1794. doi:10.1001/archopht.121.12.1791

Intraocular melanocytoma is an uncommon tumor. To our knowledge, in none of the cases reported to date, either individually or in case series, has this tumor been associated with bone formation. We report 2 such cases.

Case 1. A 14-year-old white girl had pain in her left eye of 5 weeks' duration. There was no history of ocular trauma or disease. Her visual acuity was 20/60 OS, and the intraocular pressure was 11 mmHg. There was no evidence of glaucoma or uveitis. The lens was displaced inferonasally by a superotemporal ciliary body mass.

Local resection of this mass was performed with deep scleral lamellar dissection. Postoperatively, the visual acuity was 20/60 OS; 6 months later, it had decreased to 20/200 OS because of ocular hypotony, macular edema, and epimacular membrane formation. The hypotony resolved with a short course of systemic steroids. At the last follow-up visit (28 months postoperatively), her visual acuity was 20/40 OS. The gross pathologic specimen consisted of deep sclera (18 × 11 × <1 mm) with an attached, deeply pigmented mass (15 × 11 × 5 mm). Microscopy revealed a melanocytoma with extensive necrosis and bone formation (Figure 1). There was partial necrosis of the heavily pigmented tumor with extensive cholesterol cleft formation and adjacent fibrosis. The tumor extended into the sclera. Extensive calcium deposition consisted of an admixture of granules, coarse clumps, and calcospherites. Bone was present at the outer, scleral margin of the tumor, where densely pigmented viable tumor cells involved the sclera (Figure 1). The tumor cells had abundant cytoplasm and were large and either polyhedral or spindle shaped (Figure 2). Most nuclei were round or oval with small nucleoli; a proportion of the cells were more variable in nuclear and nucleolar size (Figure 2).