Malignant Transformation of Optic Nerve Melanocytoma into Melanoma Associated with Ocular Ischemic Syndrome and Oculocardiac Reflex: Case Report and Review of the Literature

Purpose: To describe the clinical and pathological features of malignant transformation of an optic nerve melanocytoma over 25 years, associated with spontaneous extensive tumor necrosis, neovascular glaucoma, ocular ischemic syndrome, and oculocardiac reflex. Procedures: A 60-year-old Caucasian female presented with acute severe left ocular pain and glaucoma with an intraocular pressure of 80?mmHg and bradycardia (28–40 beats/min). Results: Histopathological examination of the enucleated eye showed features of malignant transformation of a spontaneously necrotic optic nerve melanocytoma associated with ocular ischemic syndrome and neovascular glaucoma. Conclusions: Yearly follow-up and awareness of possible but well-documented malignant transformation, even over several decades, is recommended. This case presents malignant transformation of an optic nerve melanocytoma associated with the common complications described in the literature over the years. Of particular interest was the development of oculocardiac reflex in this patient.