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¹Ankara Numune Eğitim ve Araştırma Hastanesi
²Ankara Numune Eğitim ve Araştırma Hastanesi Patoloji Kliniği A 39-year-old man suffering from blurred vision on his left eye for four months was referred to our clinic with a diagnosis of retinal detachment. At his initial examination, visual acuities were 10/10 in both eyes. He had a mushroom shaped, dark black mass obscuring the optic disc margins and a flat, yellow-white, nasally located subretinal lesion contiguous with the disc portion of that mass in his left eye. Since it was diagnosed with malignant melanoma of the optic disc and juxtapapillary choroid according to the associated angiographic and ultrasonographic findings, the left eye was enucleated with a modified manner. Histopathologic studies demonstrated a mixed-cell malignant melanoma accompanied with a population of melanocytoma cells. Although there is a lack of clinical follow-up, findings of clinical and laboratory studies lead us to consider that this is a rare case of malignant transformation of a presumed optic disc melanocytoma. Keywords : Melanocytoma, Melanoma, Optic disc, Malignant transformation