Two discrete uveal melanomas in a child with ocular melanocytosis

To describe a case of two uveal melanomas in a child with mild ocular melanocytosis.

A 6-year-old girl was followed for 5 years with an ill-defined, slowly enlarging presumed choroidal nevus in the postequatorial fundus. Ocular oncology evaluation revealed mild sectorial scleral and uveal melanocytosis and an episcleral sentinel vessel superotemporally. Two discrete uveal melanomas were present. In the circumpapillary and macular region, tumor 1 was diffuse at 9.0 mm in base and 4.1 mm in thickness and with overlying subretinal fluid. In the ciliary body, tumor 2 was discovered by transillumination and was 6.0 mm in base and 2.2 mm in thickness. Enucleation was performed.

Histopathologic analysis disclosed two discrete uveal melanomas in a bed of diffuse mild uveal melanocytosis. Tumor 1 was a mixed, predominantly epithelioid cell melanoma with active mitotic figures, and tumor 2 was a mixed, predominantly spindle cell melanoma. The choroid between the melanomas showed only benign, dendritic melanocytes consistent with melanocytosis. There was no extrascleral extension.

Ocular melanocytosis can predispose to one or multiple uveal melanomas. Lifetime ophthalmic monitoring of affected patients is warranted.