Uveal melanoma
The most common primary eye cancer in adults, usually in the choroid. Often found on a routine exam; it can spread to the liver.
What it is
A malignant tumour of the melanocytes of the uvea, most often in the choroid, less often in the ciliary body or iris. It is the most common primary intraocular cancer in adults. Many are found on a routine fundus exam; others cause blurred vision, floaters or field loss from an associated retinal detachment. Its main threat is liver metastasis, which can appear years later.
See it in 3D
Who gets it
Usually after 50; much more common in fair-skinned people of European ancestry and rare in people with darker skin.
Causes
- Malignant transformation of uveal melanocytes, sometimes from a pre-existing naevus
- Mutations such as GNAQ, GNA11 and BAP1
Risk factors
- Fair skin and light iris colour
- Ocular melanocytosis
- Choroidal naevus with high-risk features
- BAP1 tumour predisposition syndrome
Pathophysiology
The tumour grows from the choroid, often breaking through Bruch membrane into a mushroom shape, and causes serous retinal detachment. It spreads by blood, mainly to the liver.
Symptoms and signs
- Often none
- Blurred vision, floaters or flashes
- A shadow in the visual field
- A dark spot on the iris that grows
Diagnosis
- Clinical
- Dilated fundus examination by ophthalmology; most diagnoses are clinical, without biopsy.
- Tests
- Liver function tests and systemic staging; tumour biopsy for genetic prognosis in selected cases.
- Imaging
- Ocular ultrasound (low internal reflectivity, mushroom shape), OCT and autofluorescence; liver imaging for staging and follow-up.
Differential diagnosis
- Choroidal naevus
- Choroidal metastasis
- Choroidal haemangioma
- Peripheral exudative haemorrhagic chorioretinopathy
Goal of treatment
Control the tumour, preserve the eye and vision when possible, and watch for metastasis.
Treatment
- Procedure
- Plaque brachytherapy or proton beam radiotherapy for most small and medium tumours.
- Surgical
- Enucleation for large tumours or a blind, painful eye; local resection in selected cases.
- Follow-up
- Lifelong liver surveillance; systemic treatment by oncology for metastasis.
Complications
- Retinal detachment
- Radiation retinopathy and optic neuropathy after treatment
- Liver metastasis
Prognosis
Local control is good, but metastasis occurs in a substantial share of patients, with risk linked to tumour size and genetics.
Prevention
- Regular examination of choroidal naevi with risk features
Test yourself
Think of the answer, then tap the question to see it.
What is the most common primary intraocular cancer in adults?
Uveal melanoma.
Where does it most often metastasise?
To the liver.
How are most small and medium tumours treated?
With plaque brachytherapy or proton beam radiotherapy.
In ophthalmology
Connections
Educational information, not medical advice. For any symptom, see a health professional.
Sources: Ocular Melanoma (StatPearls Publishing (NCBI Bookshelf), 2024).
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Representative model, for educational purposes only.