Objective: To describe a case of two uveal melanomas in a child with mild ocular melanocytosis
Methods: 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.
Results: 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.
Conclusions: Ocular melanocytosis can predispose to one or multiple uveal melanomas. Lifetime ophthalmic monitoring of affected patients is warranted.
A 6-year-old girl manifested a slowly enlarging, ill-defined pigmented choroidal lesion over 5 years. After ocular oncology evaluation, mild ocular melanocytosis and two individual uveal melanomas were detected, necessitating enucleation.
From the *Ocular Oncology Service and the †Department of Pathology, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania; and the ‡Department of Ophthalmology and Visual Sciences, University of Wisconsin, Madison, Wisconsin.
Supported by a donation from Michael, Bruce, and Ellen Ratner, New York, NY (J.A.S. and C.L.S.), the Paul Kayser International Award of Merit in Retina Research, Houston, TX (J.A.S.), Mellon Charitable Giving from the Martha W. Rogers Charitable Trust (C.L.S.), the Macula Foundation, New York, NY (C.L.S.), Noel T. and Sara L. Simmonds Endowment for Ophthalmic Pathology, Wills Eye Hospital (R.C.E.), and the Eye Tumor Research Foundation, Philadelphia, PA (C.L.S. and J.A.S.).
Reprint requests: Carol L. Shields, MD, Ocular Oncology Service, Suite 1440, Wills Eye Hospital, 840 Walnut Street, Philadelphia, PA 19107.