Optic nerve melanocytoma is a variant of melanocytic nevus that has typical clinical and histopathologic features. In addition to occurring in the optic nerve, melanocytoma can also be found in the uveal tract, including the iris, ciliary body, and choroid. Most ophthalmologists are not familiar with these extrapapilary melanocytomas. This review covers the history, terminology, demographics, clinical futures, clinical course, histopathology, differential diagnosis, diagnostic techniques, management, and prognosis for melanocytomas that develop in the uveal tract, separate from the optic nerve. Like standard uveal nevi, melanocytoma can rarely spawn a malignant melanoma.
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