Intraocular Tumours

a) What is a Naevus?

A naevus is the medical term for a “mole”. Like on the skin, this can also occur in the eye and is commonly spotted on routine examination by your optometrist. They can be found on the choroid, ciliary body or iris. This can be referred to as uveal naevus. You are more likely to get a naevus if you have white skin or blue eyes. They can sometimes grow during puberty and pregnancy. Although naevi are benign, very rarely they can turn into melanoma (cancer of the eye). For this reason your optometrist might examine your eyes routinely every year.

b) What are the symptoms?

Naevi in the eye generally do not cause symptoms. If, however, the naevus turns into melanoma patients may experience:

• Decreased vision

• Flashing lights

• Shadow in the vision

c) Will I need any tests?

Simple uveal naevi are monitored in the community by your opitcian. If examined in the eye clinic, photographs of the naevus help us to detect any changes at later visits. Other tests may include:

• Ultrasound scan

• Optical Coherence Tomography (OCT)

d) Will I need treatment for Naevus?

No treatment is needed for uveal naevus.

a) What is Melanoma?

Eye melanoma is cancer of the eye. This is the most common type of eye cancer in adults. Most patients are between 55 and 65 years old when diagnosed. It affects up to 50 people a year in Scotland (between 500 and 600 people in the UK). Choroidal melanoma is the most common type of uveal melanoma, followed by the ciliary body, then iris. Uveal melanoma can spread to other parts of the body; some patients have spread, some don’t. The most common place for melanoma to spread to is the liver. This can happen many years after diagnosis. Unfortunately treating the tumour does not guarantee spread will not happen.

b) What are the symptoms of eye melanoma?

Eye melanoma may have no symptoms and may be picked up on routine examination by your optician. Some patients, however, may report:

• Decreased vision

• Flashing lights

• Shadow in the vision

Choidal, ciliary body and iris melanomas may have different symptoms.

i) Choroidal melanoma: 

Patients may experience a shadow in the peripheral vision or poor central vision. As the melanoma grows it may cause a retinal detachment. This may cause floaters, flashing lights or a curtain over part of the vision.

ii) Ciliary body melanoma:

As the ciliary body is behind the iris, small tumours may be not be visible at first. They can, however, grow and cause a shadow in the vision. Clouding of the lens, known as cataract, can develop resulting in blurred vision. Floaters can be caused if the tumour bleeds into the back of the eye. Glaucoma (raised pressure in the eye) may occur causing poor vision and eye pain.

iii) Iris melanoma

Iris melanomas may appear as a growing pigmented area on the coloured part of the eye. This can change the shape of the pupil or grow in front of the pupil causing the vision to go down. Like Ciliary body melanoma, glaucoma (raised pressure in the eye) may occur causing poor vision and eye pain.

c) What are the risks of getting melanoma?

The following features may increase your risk of developing ocular melanoma:

• Pale skin

• Red or blond hair

• Blue eyes

• Over the age of 50

• Large number of moles or freckles

d) Will I need any tests?

Certain tests help confirm the diagnosis and check how the tumour is behaving. Tests may include:

• Photograph

• Ultrasound scan

• Optical Coherence Tomography (OCT)

All patients have an ultrasound of their liver every year to make sure the melanoma has not spread or started to grow here. This is usually organised at your local hospital.

e) Will I need a biopsy?

A biopsy may be offered to confirm the type of tumour and likelihood of spread. This, however, has risks and may not provide enough tissue to give a definite answer. If we do decide to biopsy, options will be discussed in clinic.

f) What is the treatment of eye melanoma?

In treating eye melanoma, our aim is remove or destroy the tumour and keep as much normal vision as possible. Unfortunately, removing the tumour does not guarantee spread will not happen later in life. Treatments may include:

• Plaque radiotherapy
• Proton beam radiotherapy
• Laser (Transpupillary thermotherapy)
• Photodynamic Therapy (PDT)
• Removal of eye (enucleation)
• Removal of tumour (local resection)

g) Is treatment always needed?

Very rarely we may choose not to treat. If the patient is frail, elderly, or has other serious medical illnesses we may decide to watch instead. Every patient is different. After discussion in clinic we will arrive at the right and best treatment option for you.

h) How likely is the melanoma to spread?

Risk of melanoma spreading (metastasising) to other parts of the body depends on the three factors outlined below.

i) Clinical tumour stage

Clinical tumour stage is based on how the tumour looks when examined in the clinic and the size of the tumour. The larger the tumour, the longer it is likely to have been there and the more likely it has had a chance to spread elsewhere in the body. If the tumour has grown quickly in size or grown through the wall of the eye, again this increases the risk of tumour spreading.

ii) Histology

This refers to how the tumour cells look under the microscope. Tumour tissue from biopsy or after the eye is removed is sent to the laboratory and examined by our pathologist. If the tumour cells appear large and round (epithelioid) then there is high chance of tumour spreading. If the cells appear long and narrow (spindle) then chance of spread is far less.

iii) Genetic typing

This refers to the arrangement of DNA in our cells (the basic building blocks for life). Our cell behaviour is controlled by DNA, which is stored in our genes. These genes are found in chromosomes, which are thread like structures found in every cell of our body. We have a total of 23 pairs of chromosomes. Each chromosome has long arms (coded by the letter q) and short arms (coded by the letter p). Tumour tissue is sent to the genetics laboratory where it is analysed. If a single chromosome 3 is missing from melanoma cells, this is called “monosomy 3”. This means the melanoma is very likely to spread. If chromosome 8q has gained a longer arm, again, this increases the chance of the melanoma spreading. If both “monosomy 3” and 8q gain are present then this there is an even stronger chance of tumour spread. If, however, there is a gain in chromosome 6p, this is protective and decreases the likelihood of metastasis.

i) Will I need any tests to check for spread?

We will perform an ultrasound scan (USS) of the liver every 6 months to check for spread of the melanoma. If we suspect there is spread on USS, we will organise an MRI scan of the liver to look at this in closer detail. 

Some patients find having an USS every 6 months too stressful and worrisome. If this is the case we can perform the USS every 12 months instead. 

j) What happens if the eye melanoma spreads to different parts of the body?

This can be scary and upsetting. If spread of disease has been picked up we involve other health professionals at our MDT (multidisciplinary team) meeting. Our clinical team consists of a radiologist, pathologist, and a medical oncologist. Collectively we will decide on the right tests and treatments for you as an individual. Accepting and coming to terms with the diagnosis can be challenging. For this reason, we have specialist ophthalmic nursing staff in the clinic who are here to council and help you through this difficult time.

a) What is uveal metastasis?

Cancer starting elsewhere in the body can spread to the eye, in particular, the uveal tract. This is called uveal metastasis. In the uveal tract the choroid is the most common sight for cancer to spread to, followed by the iris, then the ciliary body. In men cancer most commonly spreads to the eye from lung cancer, and in woman from breast cancer. This is most common in adults aged 55-65. Other sites for cancer to start before spreading to the eye include the kidneys, gastrointestinal tract, and the skin.

b) What are the symptoms of uveal metastasis?

Commonly there are no symptoms and the uveal metastases are noticed on routine examination. Some patients, however, may experience:

• Decreased vision

• Flashing lights

• Shadow in the vision

Sometimes the tumour causes the retina to detach, this is where the back lining of the eye comes away. This may cause floaters, flashing lights, and a curtain over the vision.  

c) Will I need any tests?

As well as taking pictures of the eye, we may perform:

• Ultrasound Scan

• Optical Coherence Tomography (OCT)

• Blood tests

If we suspect the cancer has spread to the eye from else where in the body, we may arrange scans to look for cancer. This may include:

• Chest X-ray

• CT scan

• PET CT scan

• MRI scan

d) What is the treatment for uveal metastasis?

Treatments may include:

• Radiotherapy (external beam radiotherapy or plaque radiotherapy)

• Chemotherapy

• Radiotherapy and chemotherapy

The treatment choice depends on where the cancer has started from in the body.  Discussion with our medical oncologist will helps us choose the best treatment for you. 

a) What is choroidal haemangioma?

Choroidal haemangiomas are benign tumours that grow in the blood vessel layer beneath the retina called the choroid. They are either circumscribed or diffuse. Circumscribed means there is no underlying medical condition. Diffuse choroidal haemangiomas, however, are commonly found in a condition called Sturge-Weber syndrome. The vision is more likely to be affected in this type. Although not as common, haemangiomas can be found in the iris and ciliary body. This can sometimes cause glaucoma.

b) What are the symptoms of choroidal haemangioma?

Commonly there are no symptoms and your optician finds them on routine examination. Sometimes, however, the choroidal haemangioma can leak fluid underneath the retina at the back of the eye. This may cause:

• Blurred vision

• Distortion

• Hypermetropia (change in perscription making you long sighted)

• Flashing lights If there is a lot of fluid leakage at the back of the eye, this can cause a retinal detachment. This may cause floaters, flashing lights, and a curtain over the vision.

c) Will I need any tests?

Some tests can help us confirm the diagnosis and see if the haemangioma is leaking fluid. These may include:

• Ultrasound Scan

• Optical coherence tomography (OCT)

• Fundus fluorescein angiography (FFA)

• Indocyanine green chorioangiography (ICG)

d) What is the treatment for choroidal haemangioma?

If there are no symptoms, no treatment is required. If, however, the haemangioma is growing, leaking or starting to affect the vision, we may use the following treatments:

• Photodynamic therapy (PDT)
• Laser (Traspupillary thermotherapy)
• Plaque radiotherapy
• Proton Beam Radiotherapy

e) Is treatment of choroidal haemangioma successful?

Laser photocoagulation helps seal the leaking blood vessels. Unfortunately, fluid can build up again. PDT and Transpupillary thermotherapy have both shown great successful in stopping leakage from choroidal haemangiomas. If the retina has detached, however, giving these treatments can be too difficult. Radiotherapy treatment may be preferred in these cases. The longer fluid is at the back of the eye or the retina is detached, the smaller the chance of having complete recovery of vision after treatment.

f) Will choroidal haemangioma spread to other parts of the body?

No. This is a benign tumour and does not metastasise.

a) What is eccentric disciform degeneration?

Eccentric disciform degeneration is an abnormal growth of blood vessels underneath the retina. These vessels are very fragile and can bleed or scar over. This is not a type of cancer although the degeneration can look similar to melanoma or other cancers of the eye. This mainly occurs in elderly people with up to half of patients having macular degeneration. Other names for this condition include peripheral exudative haemorrhagic chorioretinopathy (PEHCR), extra-macular disciform degeneration and peripheral age related retinal degeneration.

b) What are the symptoms of Eccentric Disciform Degeneration?

There may be no symptoms. If leakage from the abnormal blood vessels track down to the macula (the centre of the back of the eye) then blurring or distortion of the vision may occur. Sometimes this can cause a retinal detachment. This may cause floaters, flashing lights, or a shadow in the corner of the vision. When looking at the back of the eye in the clinic the abnormal area appears flat irregular and red or pale.

c) What are the risks of getting eccentric disciform degeneration?

The following features increase your risk of getting this condition:

• Over 70 years of age

• Female

• High blood pressure (hypertension)

• On medicine that thin the blood (Anticoagulant therapy)

• Short or long sighted (Myopia or hypermetropia)

d) Will I need any tests?

To confirm eccentric disciform degeneration and exclude cancer we may have to perform a few tests. These may include:

• Photographs

• Optical Coherence Tomography (OCT)

• Ultrasound scan

• Fundus Fluorescein Angiography (FFA)

These tests may be repeated at future appointments and help us pick up changes.

e) What is the treatment for eccentric disciform degeneration?

Monitoring in the eye clinic is sometimes all that is required. This condition regularly gets better without treatment. Laser photocoagulation treatment can be used in the clinic to seal off the leaking blood vessels and prevent the swelling and leakage getting worse.