Episcleral melanoma as a complication of ocular melanocytosis

Background: Primary episcleral melanoma is exceedingly rare. We report a case of primary episcleral melanoma in an eye with ocular melanocytosis. Methods: Interventional case report with presentation of clinical photographs and histopathology. Results: A 62-year-old man with long-standing ocular melanocytosis presented with a new, raised, darkly pigmented lesion on the episclera. Clinical examination and ultrasound biomicroscopy showed no evidence of intraocular mass or nevus. Lamellar scleroconjunctivectomy of the lesion was performed, and histopathologic analysis revealed episcleral melanoma with no evidence of intra- or extra-ocular extension. He was later treated with cryotherapy to the surgical bed. Continued observation shows no evidence for intraocular tumor or recurrence of episcleral tumor. Conclusions: Primary episcleral melanoma is a previously unreported complication of ocular melanocytosis, and should be considered in the differential diagnosis in patients with ocular melanocytosis and pigmented nodules on the ocular surface.