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Uveal melanoma and intraocular tumours in adults

Uveal melanoma is the commonest primary malignant intraocular tumour in adults, usually in the choroid, and metastasises mainly to the liver; diagnosis is clinical — fundus, ultrasound and imaging — without routine biopsy, and treatment is usually local radiation that preserves the eye. The commonest intraocular tumour overall in adults is a metastasis.

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Why it matters in the exam

Distinguishing a benign choroidal naevus from a small melanoma is a classic question. Signs that raise risk: thickness above 2 mm, subretinal fluid, symptoms (flashes, blur), orange pigment (lipofuscin), a margin close to the disc, "acoustic hollowness" — low internal reflectivity on ultrasound — and absence of drusen or a halo over the lesion. A "mushroom" shape (collar-button) shows a break through Bruch membrane and is typical of melanoma. Risk factors: fair skin, a light iris, ocular melanocytosis and BAP1. GNAQ and GNA11 mutations are common; monosomy 3 predicts a poor prognosis.

Treatment: brachytherapy (a radioactive plaque sutured to the sclera) for medium tumours — the COMS study showed survival identical to removal of the eye; proton beam radiation; enucleation for large tumours or when there is no chance of vision. About half of patients develop metastases later, mainly in the liver, so liver imaging follow-up is done every 6–12 months. Tebentafusp prolongs survival in metastatic disease in patients with HLA-A*02:01. Iris melanoma — a better prognosis.

Choroidal metastases: from breast cancer in women and lung cancer in men — flat yellowish lesions, sometimes multiple and bilateral, with subretinal fluid; sometimes they are the first sign of the cancer. Choroidal haemangioma — an orange-red lesion; a diffuse "tomato ketchup" form in Sturge-Weber syndrome. In the conjunctiva: ocular surface squamous neoplasia (linked to HPV, HIV and sun) and conjunctival melanoma from primary acquired melanosis with atypia. Vitreoretinal lymphoma masquerades as uveitis in an older adult.

Key points

• The commonest primary malignant tumour in the adult eye.
• Risk signs: thickness, fluid, orange pigment, collar-button.
• COMS: brachytherapy equals enucleation in a medium tumour.
• Liver metastases → liver follow-up every 6–12 months.
• Choroidal metastases: breast in women, lung in men.

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Sources

• Collaborative Ocular Melanoma Study (COMS) Group, 2006 — Brachytherapy versus enucleation for medium choroidal melanoma
• NCCN Guidelines: Uveal Melanoma, 2025 — Diagnosis, treatment and surveillance
• AAO Basic and Clinical Science Course, Section 4: Ocular Pathology and Intraocular Tumors, 2024-2025 — Melanocytic tumours and metastatic tumours

Sources are given for further reading; the text is written afresh and not copied from them.

Reviewed by the Examqo AI Professor · 2026-11-06. The text may contain errors; when in doubt, check the source.