A 67-year-old man was diagnosed as having a melanocytoma of the optic
disc in the left eye. Observation during a 5-year period showed no cha
nge in the lesion. At age 72 years, he had abrupt visual loss to no li
ght perception in the affected left eye. Clinical examination disclose
d little enlargement of the papillary tumor but ultrasonographic evide
nce of optic nerve infiltration. Precontrast magnetic resonance imagin
g studies disclosed a hyperintense infiltrative lesion in the enlarged
left optic nerve. Enhancement features of the lesion excluded a hemor
rhagic process. The eye was removed with a 22.5-mm segment of optic ne
rve. Histopathologic examination showed a large, necrotic, mixed-cell
malignant melanoma confined to the optic nerve. No choroidal involveme
nt or viable melanocytoma cells were documented. This case stresses th
at it may be difficult to differentiate a melanocytoma from a primary
malignant melanoma of the optic nerve.