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Melanocytomas of the Optic Nerve and Uvea

Melanocytomas are an example of changing attitudes toward clinical management of intraocular tumors. They also might exemplify the power of pathological examination and follow-up in patient care and treatment. Prior to the observations of Zimmerman [10], most patients with melanocytomas underwent enucleation. Today, these patients are followed up conservatively and continue to enjoy vision. Melanocytomas are fairly uncommon tumors found more often in blacks and hyperpigmented individuals than in whites. Women are slightly more likely to have this tumor than are men. Melanocytomas are uniformly densely hyperpigmented lesions found on the optic nerve, choroid, ciliary body, and iris and are benign, stationary tumors that seldom cause symptoms or secondary complications. In patients with papillary melanocytomas, the most common visual disturbance is enlargement of the blind spot. With larger tumors, an afferent pupillary defect may be noted. Ciliary body and iris tumors may cause secondary anterior segment complications such as glaucoma, hyphema, or ectopia. Dispersion of pigment by melanocytomas is common and should not necessarily be interpreted as a sign of malignant transformation. Ancillary tests such as fluorescein angiography, ICG angiography, ultrasonography, CT, MRI, and the 32P test are of little clinical benefit in differentiating between melanocytomas and malignant melanomas and may lead to erroneous conclusions. Treatment for melanocytomas is conservative, its mainstay being serial clinical examinations, fundus photography, and visual field examinations to record tumor size and document growth. Lesions that grow rapidly may have transformed to a malignant melanoma and should be treated accordingly.