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A 35-year-old black man developed abrupt visual loss in his left eye. Ophthalmic examination revealed a deeply pigmented mass obscuring the optic disc, hemorrhagic retinopathy, and signs of central retinal vascular obstruction. Fluorescein angiography disclosed sluggish filling of the retinal blood vessels; ultrasonography disclosed an acoustically solid mass in the optic nerve head. Cytopathologic findings of a fine needle aspiration biopsy specimen demonstrated probable benign tumor cells, but melanoma could not be excluded. Histopathologic findings in the enucleated eye revealed a large, necrotic melanocytoma of the optic disc and hemorrhagic necrosis of the retina secondary to obstruction of the central retinal artery and vein. Melanocytoma of the optic nerve can undergo spontaneous necrosis and induce central retinal vascular obstruction. Abrupt visual loss in a patient with a melanocytoma does not necessarily imply malignant transformation.

Objectives: To present and discuss 2 patients with acquired peripapillary pigmented lesions., Methods: We reviewed the patients' clinical records and histopathologic findings., Results: The first patient was diagnosed with a pigmented papillary lesion that was followed up for 38 years. The second patient was a child with neurofibromatosis type 1 who developed a pigmented peripapillary lesion following excision of an optic nerve glioma. Histologic findings in both cases demonstrated hyperplasia of the retinal pigment epithelium with associated findings., Conclusions: The lesions presented an idiopathic reactive hyperplasia of the retinal pigment epithelium. The clinical and histopathologic findings resemble findings reported with the combined hamartoma. We suggest that such lesions are reactive in nature, rather than hamartomatous.