Eye Cancer

Eye cancer occurs when cells in or around the eye undergo abnormal genetic changes and begin to grow uncontrollably, forming a malignant tumour. It is an uncommon group of cancers overall, but an important one to understand well, because the eye's specific anatomy means that even a small tumour can threaten both vision and, in some cases, life — and because several of its most common forms behave very differently from one another.

Eye cancer can be primary, meaning it starts within the eye itself, or secondary, meaning it has spread (metastasised) to the eye from a cancer elsewhere in the body, most often the breast or lung. Among primary eye cancers, uveal melanoma is the most common in adults, while retinoblastoma is the most common in children. Cancers can also arise from the eyelids and the conjunctiva (the clear membrane covering the white of the eye), which behave more like skin cancers and are managed somewhat differently from cancers arising inside the eyeball itself.

Understanding the Eye: Where Different Cancers Arise

The eye is made up of three main layers, and the type of eye cancer that can develop is closely tied to which layer or structure is affected:

  • The fibrous outer layer: Includes the sclera (the white of the eye) and the cornea (the clear front surface that helps focus light). Cancers of the conjunctiva, the thin membrane covering this layer, arise here.
  • The vascular middle layer (the uvea): Includes the choroid, ciliary body, and iris, which nourish the eye, control the lens, and regulate how much light enters. This pigmented, blood-vessel-rich layer is where uveal melanoma, the most common primary eye cancer in adults, develops.
  • The inner layer: Formed by the retina, which captures visual information and sends it to the brain via the optic nerve. This is the layer affected by retinoblastoma, the most common eye cancer in children.

Types of Eye Cancer

Uveal Melanoma

The most common primary intraocular cancer in adults, arising from pigment-producing cells in the choroid, ciliary body, or iris. Often detected on routine eye examination before it causes any symptoms. Carries a real risk of spreading to the liver, which shapes lifelong surveillance after treatment.

Retinoblastoma

The most common eye cancer in children, typically diagnosed before age 5, arising from immature retinal cells. Can be hereditary (linked to a mutation in the RB1 gene, often bilateral) or sporadic (usually one eye only). Highly treatable when caught early, with survival rates exceeding 95% in high-income healthcare settings.

Intraocular Lymphoma

An uncommon cancer, typically a form of non-Hodgkin lymphoma, that can affect the eye in adults. Often associated with lymphoma of the central nervous system, and can mimic inflammatory eye conditions, sometimes delaying diagnosis.

Eyelid and Conjunctival Cancers

Include basal cell carcinoma (the most common eyelid cancer), squamous cell carcinoma, sebaceous gland carcinoma, and conjunctival melanoma. These behave more like skin cancers, are strongly related to ultraviolet exposure, and are generally treated with surgical excision.

Secondary (Metastatic) Eye Cancer

Cancer that has spread to the eye from elsewhere in the body, most commonly the breast or lung. Metastatic disease to the eye is actually more common than primary intraocular cancer in adults overall, and its presence often indicates that cancer has also spread elsewhere.

Uveal Melanoma: Why Tumour Size Determines Treatment

For uveal melanoma, size is the single most important factor in deciding how a tumour is treated, since it directly affects both the risk of vision loss and the risk of the cancer spreading. Tumours are broadly classified as small, medium, or large based on their basal diameter (width) and apical height (thickness), using criteria established by major international studies such as the Collaborative Ocular Melanoma Study.

Very small tumors may initially be monitored closely with serial imaging, particularly where there is diagnostic uncertainty, since some small pigmented lesions are benign naevi (moles) rather than melanoma. Treatment is initiated if growth or high-risk features are documented.

For medium-sized tumors, eye-preserving treatment is usually the priority. Plaque brachytherapy (a radioactive disc temporarily stitched to the outer eye wall over the tumour) is the most widely used treatment for tumours in this range, offering survival outcomes equivalent to removing the eye, based on landmark clinical trial evidence.

Larger tumours are more likely to require enucleation (surgical removal of the eye) when preserving useful vision and controlling the tumour is not achievable with eye-sparing treatment, though proton beam therapy is increasingly used as an eye-preserving alternative for select large tumours at specialised centres

Beyond size, genetic testing of tumor tissue — typically obtained via fine-needle aspiration biopsy at the time of treatment — has become an important part of modern uveal melanoma care. Certain chromosomal changes and gene expression profiles are associated with a meaningfully higher risk of the cancer spreading to the liver, the most common site of metastasis. This information does not change the treatment of the eye itself, but it shapes how closely a patient is monitored afterward — patients found to carry a higher-risk genetic profile are typically followed with more frequent liver imaging and blood tests for early detection of metastatic disease, since early treatment of liver metastases improves outcomes.

Retinoblastoma: The Warning Sign Parents Shouldn't Miss

Retinoblastoma deserves particular attention because its most recognisable sign is easy to miss unless parents know what to look for — and because catching it early makes an enormous difference to both survival and the chance of preserving the eye and useful vision.

The hallmark sign is leukocoria — a white or cloudy reflection in the pupil, instead of the normal red-eye reflex, often first noticed by a parent in a flash photograph, or by a doctor during a routine check-up. Other possible signs include a new squint (strabismus), a change in eye colour, or redness and swelling of the eye without an obvious cause. Any of these findings in a young child warrants prompt ophthalmic assessment.

Roughly a third to a half of retinoblastoma cases are linked to an inherited mutation in the RB1 gene, and these cases are more likely to affect both eyes and to be diagnosed at a younger age. A child diagnosed with hereditary retinoblastoma also carries a lifelong increased risk of certain other cancers later in life, which is why genetic counselling and testing are recommended as part of care — not only for the affected child, but to guide screening for siblings and future children in the family, who may need eye examinations from birth if a hereditary mutation is confirmed or strongly suspected.

Symptoms of Eye Cancer

Many people with eye cancer, particularly uveal melanoma, have no symptoms at all in the early stages, and the tumour is often found incidentally during a routine eye examination. When symptoms do occur, they typically relate to how the tumour is affecting vision or the physical structure of the eye. Pain is uncommon unless the tumour has grown large enough to affect surrounding tissue or raise pressure within the eye.

  • Blurred vision or a loss of part of the visual field
  • Seeing flashes of light
  • Seeing spots, squiggly lines, or floating shapes (floaters)
  • A dark spot on the iris or visible on the white of the eye that is new or growing
  • Bulging of the eye, or a change in its position within the socket
  • A change in the size or shape of the pupil
  • In children: a white or cloudy reflection in the pupil (leukocoria), a new squint, or an eye that appears red and swollen without clear cause

Many of these symptoms are far more commonly caused by conditions other than cancer, such as retinal detachment, cataracts, or benign eye lesions. This is precisely why they should not be self-diagnosed at home — a detailed examination by an ophthalmologist is the only reliable way to distinguish between them.

Risk Factors for Eye Cancer

Some risk factors have been identified to increase your risk of Eye Cancer. They include:

  • Age: Most eye cancers are diagnosed in adults over 50; retinoblastoma is the exception, typically affecting children under 5
  • Fair skin : People with lighter skin that sunburns and freckles easily are at higher risk of uveal melanoma and eyelid skin cancers
  • Light eye colour People with blue, green, or grey eyes have a higher risk of developing uveal melanoma than those with brown eyes
  • Inherited medical conditions: dysplastic naevus (atypical mole) syndrome and certain inherited tumour predisposition syndromes, such as BAP1 tumour predisposition syndrome, raise the risk of uveal melanoma
  • Ultraviolet light exposure — prolonged UV exposure, including occupational exposure such as welding, is associated with increased risk of ocular surface and eyelid cancers
  • Family history — relevant both for uveal melanoma in the context of inherited cancer syndromes, and for retinoblastoma, where hereditary RB1 mutations run in families
  • Immunosuppression — conditions or medications that weaken the immune system are associated with a higher risk of intraocular lymphoma and certain eyelid cancers

How Eye Cancer is Diagnosed

  • Dilated Eye Examination: The essential first step. Using an ophthalmoscope and a slit lamp (a microscope with an attached light), the ophthalmologist examines the retina, choroid, and other internal structures of the eye in detail after the pupil has been dilated with eye drops.
  • Ocular Ultrasound: Measures the size, shape, and internal characteristics of a suspected tumour, and helps distinguish some tumour types from one another based on their internal reflectivity pattern. Also useful when the view into the eye is obscured, such as by bleeding.
  • Fluorescein Angiography: A fluorescent dye is injected into a vein in the arm and travels to the blood vessels at the back of the eye, allowing detailed imaging of the tumour's blood supply and helping to rule out other conditions that can mimic eye cancer.
  • Optical Coherence Tomography (OCT): Produces detailed cross-sectional images of the retina and underlying tumour, useful for assessing associated fluid (such as retinal detachment) and for monitoring subtle changes over time.
  • Fine-Needle Aspiration Biopsy: A small sample of tumour tissue is obtained, most often at the time of treatment, to confirm the diagnosis and, for uveal melanoma, to perform genetic testing that helps predict metastatic risk and guide surveillance.
  • MRI or CT Scan: Used to assess whether a tumour extends beyond the eye into surrounding tissue, and, for retinoblastoma, to check for any spread along the optic nerve or into the brain.
  • Systemic Staging Investigations: For uveal melanoma, liver function blood tests and liver imaging (ultrasound, CT, or MRI) are performed at diagnosis and at intervals afterward, since the liver is the most common site of metastatic spread.

Life After Treatment

  • Attend all recommended follow-up imaging, particularly lifelong liver surveillance after treatment for uveal melanoma, since early detection of metastatic disease meaningfully improves treatment options
  • If an eye has been removed, allow time to adjust to changes in depth perception, and discuss ocular prosthesis fitting and care with your surgical team
  • For children treated for retinoblastoma, ensure genetic counselling is completed for the family, and that recommended screening is arranged for siblings where relevant
  • Vision changes after eye-preserving radiotherapy are common and can continue to evolve for months to years afterward — low vision support and rehabilitation services can help maximise remaining vision
  • Protect both eyes from excessive ultraviolet exposure with sunglasses, particularly important for patients treated for ocular surface or eyelid cancers
  • Seek support for the emotional impact of an eye cancer diagnosis and its treatment — this is a valid and common part of recovery, not a separate issue from physical treatment