Malignant melanoma associated with melanocytoma of the optic disc

A 61-year-old white man underwent enucleation because of progressive growth of a pigmented epipapillary tumor that was diagnosed 9 years earlier as an optic nerve and juxtapapillary melanocytoma. Histopathologic studies showed the tumor was a malignant melanoma of the optic disc and juxtapapillary retina and choroid. Foci of typical melanocytoma cells were within the tumor. The tumor produced segmental atrophy of the optic nerve. This is a rare example of a malignant melanoma developing in conjunction with a lesion that possessed typical clinical and histopathologic features of a melanocytoma of the optic disc.