Beyond the Eye: A Unique Case of Choroidal Melanoma with Extra-Ocular Extension

Choroidal melanoma, the most common primary intraocular malignancy, can present diagnostic and therapeutic challenges, especially when complicated by unusual features such as extraocular extension and perineural invasion.

We report the case of a 45-year-old Caucasian male who initially presented with left-sided vision loss, hearing deficits, and symptoms resembling retinal detachment. Imaging revealed a hyperreflective vitreous mass, but a definitive diagnosis was delayed due to complex MRI findings, which suggested a haemorrhagic process without clear evidence of malignancy. The patient's symptoms rapidly worsened, leading to left eye enucleation. Histopathology confirmed necrotic choroidal melanoma with extraocular extension, optic nerve head infiltration, and perineural invasion, while BAP1-negative status indicated a poorer prognosis. Despite these aggressive features, no distant metastasis was detected on follow-up imaging, a notable and unusual finding.

This case highlights the diagnostic complexity of choroidal melanoma when complicated by secondary findings such as inflammation and necrosis. It also underscores the importance of multidisciplinary management, timely intervention, and vigilant surveillance in cases with high-risk features. This case contributes to the limited literature on choroidal melanoma with perineural invasion, a rare finding with important prognostic implications.