Melanoma of the eye This information is about a rare cancer called melanoma of the eye (ocular melanoma). Although melanoma is a cancer that usually starts in the skin, it can sometimes develop in other parts of the body. In this information: • • • • • • • • • • • •

What is melanoma of the eye? The eye Risk factors and causes of eye melanoma Signs and symptoms of eye melanoma How eye melanoma is diagnosed Staging and grading Treatment Clinical trials Follow-up Your feelings Useful organisations References and thanks

What is melanoma of the eye? Melanoma is a cancer that develops from cells called melanocytes. Melanocytes give our skin its colour. Melanoma usually develops in the skin. But because there are melanocytes in different parts of the body, it can start in other places, such as the eye. Most eye melanomas start in an area of the eye called the uvea (see diagram below). This is called uveal melanoma. It is the most common type of cancer to affect the eye, although it’s still quite rare. Between 500 and 600 people are diagnosed with uveal melanoma in the UK each year. It’s more common in people in their 50s. Very rarely, melanoma starts in the conjunctiva, which is the outer lining of the eye. This is called conjunctival melanoma. These two types of eye melanoma are treated in slightly different ways.

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The eye Lens

Cornea Iris Ciliary body

Uvea

Choroid

Diagram showing the structure of the eye The eye is made up of several layers of tissue and filled with a clear jelly. Each part of the eye does an important job that helps us see. The uvea is the middle layer around the eyeball. It includes the choroid, the ciliary body and the iris, which is the coloured disc at the front of your eye. The uvea controls how light comes into the eyeball and helps the eye to focus. It also makes a fluid around the lens to keep your eye healthy. The conjunctiva is a thin, clear lining that covers and protects the white outer surface of the eye and the inside of the eyelids.

Risk factors and causes of eye melanoma The cause of eye melanoma is not known. The main risk factor for skin melanoma is exposure to ultraviolet (UV) rays, either from the sun, sunbeds or sunlamps. Eye melanoma is more common in people with fair or red hair, blue eyes and whose skin burns easily. But it’s still not clear whether there is a link between UV ray exposure and eye melanoma. Eye melanoma is more common in people who have atypical mole syndrome, which is also called dysplastic naevus syndrome. People with this condition often have more than 100 moles on their body, and are more likely to develop a skin melanoma.

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Conjunctival melanoma usually develops from a rare condition called primary acquired melanosis (PAM), which causes brown or dark patches (pigmentation) on the conjunctiva. Sometimes the melanoma will develop from an existing freckle or mole on the conjunctiva.

Signs and symptoms of eye melanoma Eye melanoma doesn’t usually cause any symptoms. It may be found by an optician during a routine eye test. However, signs and symptoms can include:

• blurred vision • seeing flashing lights and shadows • brown or dark patches on the white area of the eye. These symptoms are common to other conditions of the eye, but tell your GP or optician if you notice them.

How eye melanoma is diagnosed You usually start by seeing your optician or GP. If they are unsure what the problem is, they will refer you to a hospital eye specialist doctor (ophthalmologist). At the hospital, the specialist doctor will examine you. They may use the following tests to diagnose eye melanoma.

Eye drops The doctor may put eye drops in your eye before or during some of these tests. This opens up (dilates) your pupil and makes it easier to examine your eyes. The drops will make your eyesight blurry for several hours, and you might find bright lights uncomfortable. Don’t drive until your eyesight returns to normal.

Examining your eye The doctor looks at the inside of your eye using a small, handheld lens and light (ophthalmoscope). Or they may use a larger microscope that sits on a table (slit-lamp bimicroscope), which you look into. They may put eye drops in your eye to open up your pupil.

Ultrasound scan This scan uses sound waves to build up a picture on a computer screen of the inside of your eye and nearby areas. The doctor gently presses a small probe against your closed eyelid and moves it over the skin. This is painless and usually only takes a few minutes.

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Fluorescein angiography The doctor uses this test to examine the back of your eye. They put eye drops in your eye to open up your pupil, and they inject a dye, called fluorescein, into a vein in your arm. The doctor uses a special camera to take photos of the dye as it moves through the blood vessels at the back of your eye. You may feel warm or flushed for a short time after the injection. After the test, your urine will be bright yellow and your skin may be slightly yellow. This is caused by the dye. It’s harmless and only lasts a few days.

Biopsy Doctors can often diagnose eye melanoma by examining the eye with the tests we mention above. But some people may need to have a small piece of tissue or cells (biopsy) removed from the eye using a fine needle. You are more likely to have a biopsy if your specialist thinks you may have a conjunctival melanoma. A biopsy is only done by expert eye doctors who can do it quickly and without causing you pain. You can have it done using a local anaesthetic, or sometimes with a general anaesthetic. Afterwards, a pathologist (doctor who specialises in analysing cells) examines the tissue under a microscope to look for cancer cells or conditions that may develop into melanoma if left untreated.

Further tests You may have other tests to help plan your treatment or to check how effective treatment has been.

Transillumination If you need surgery, this test shows exactly where the melanoma is and helps the doctor plan the operation. The doctor turns down the lights in the room and shines a very bright light into your eye to look for abnormal areas.

Colour fundus photography This test may be used to show what the tumour looks like before and after a treatment, such as radiotherapy. The doctor gives you eye drops to open up your pupil. They use a special camera to take photographs of the back of your eye (fundus).

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PET/CT scan This is a combination of a CT (computerised tomography) scan and a PET (positron emission tomography) scan. A CT scan takes a series of x-rays that build up a three-dimensional picture of the part of the body being scanned. A PET scan uses low-dose radiation to measure the activity of cells in different parts of the body. PET/CT scans are a newer type of scan, and you may have to travel to a specialist centre to have one. You won’t be able to eat for six hours before the scan, although you may be able to drink. A mildly radioactive substance is injected into a vein, usually in your arm. The radiation dose used is very small. The scan is done after at least an hour’s wait. It usually takes about 30 minutes.

MRI (magnetic resonance imaging) scan This test uses magnetism to build up a detailed picture of the area being scanned. You may be asked to complete and sign a checklist to make sure it’s safe for you to have an MRI scan. Before the scan, you’ll be asked to remove any metal belongings, including jewellery. Some people are given an injection of dye into a vein in the arm. This is called a contrast medium and can help the images from the scan to show up more clearly. During the test, you’ll lie very still on a couch inside a long cylinder (tube) for about 30 minutes. It’s painless but can be slightly uncomfortable, and some people feel a bit claustrophobic. It’s also noisy, but you’ll be given earplugs or headphones.

Staging and grading Staging The stage of a cancer describes its size and whether it has spread. Knowing the stage helps doctors decide on the best treatment for you. There are different types of staging systems used for different cancers. Your doctor might use the following to describe eye melanoma. TNM staging system T describes the size of the tumour and whether it has grown into areas around the eye. For example, a T1 tumour is small and hasn’t spread, while a T4 is larger and has grown into areas around the eye. N describes whether the cancer has spread to lymph nodes nearby. For example, N0 means that no lymph nodes are affected, while N1 means there are cancer cells in the lymph nodes. The lymph nodes are part of your body’s system to protect you from infection and disease. M describes if the cancer has spread to another part of the body. This is called metastatic cancer. M0 means the cancer hasn’t spread and M1 means the cancer has spread to distant organs, such as the liver or lungs.

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Talking about staging Your doctor or nurse will be able to give you more information about staging. They may describe the tumour as small, medium or large. They may also use the following words:

• early or local – to describe a cancer that hasn’t spread outside the eye • advanced – to describe cancer that has spread to areas around the eye or to distant organs, such as the liver or lungs.

Grading The grade of a cancer gives the doctor an idea of how quickly it may develop. Doctors look at a sample of the cancer cells under a microscope to find the grade of the cancer. Your doctor doesn’t need this information to plan your treatment for eye melanoma, and it’s usually not tested unless you have a biopsy or surgery. Low-grade – the cancer cells tend to grow slowly and look like normal cells. High-grade – the cancer cells tend to grow more quickly and look very abnormal.

Treatment Treatment for eye melanoma may include:

• radiotherapy • surgery • transpupillary thermotherapy (TTT) • cryotherapy • chemotherapy eye drops • photodynamic therapy (PDT). You may have one or a combination of these treatments. Your treatment plan will depend on factors such as the size and position of the tumour, your general health and your eyesight. Uveal and conjunctival melanomas are treated in slightly different ways. But the aim is to destroy the cancer cells, while doing as little damage to your eyesight as possible. Some treatments for eye melanoma are very specialised and are only available at a few hospitals in the UK. You may have to travel to one of these hospitals for your treatment.

Radiotherapy Radiotherapy uses high-energy rays to destroy the cancer cells, while doing as little harm as possible to normal cells. Radiotherapy may be given either from outside the body (external radiotherapy) or from within the body (internal radiotherapy). If you have uveal melanoma, radiotherapy may be the only treatment you need or it may be given with other treatments.

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For conjunctival melanoma, you may have radiotherapy after surgery to reduce the risk of cancer coming back. There are different types of radiotherapy: External radiotherapy These types of radiotherapy aim high-energy rays from a machine at the tumour. This only treats the area of the body that the rays are aimed at. It doesn’t make you radioactive. Proton beam radiotherapy This treatment uses proton radiation to kill cancer cells. The proton beam is aimed directly at the tumour and causes very little damage to surrounding healthy tissue. Before the treatment, you have an operation to attach tiny metal clips to the white, outer wall of the eye. This is done under general anaesthetic. You won’t be able to see the clips, but they show up on scans and this helps your team plan the treatment. You have treatment in small doses (fractions) each day for four or five days. The clips are left in place after treatment, unless you are finding them uncomfortable. They don’t cause any harm and most people can’t feel them. Stereotactic radiotherapy This treatment uses many small beams of radiation to target the tumour. It delivers high doses of radiotherapy to very precise areas of the body, which reduces side effects. You usually only need one session of treatment. A number of different machines can give stereotactic radiotherapy. They include LINACs and specially designed machines such as CyberKnife™ and Gamma Knife™. Internal radiotherapy (brachytherapy) This treatment is given using a small radioactive disc called a plaque. It’s often called plaque brachytherapy. You have an operation to attach the plaque to the wall of the eye over the tumour. You usually have a general anaesthetic for this. But sometimes, the surgeon will give you a local anaesthetic to numb the area, and drugs to help you relax before putting the plaque in. The plaque is left in place, usually for 1–4 days, until the right amount of radiation has been given. After this, you have another short operation to remove the plaque. You stay in hospital for up to a week during this treatment. While the plaque is attached to your eye, there is a small risk of radiation exposure for the people around you. Your doctor or nurse will give you advice about keeping yourself and others safe. You’ll need to stay in one room, and your visitors will only be allowed in for a short time each day. Once the plaque is taken out, you are not radioactive and you should be able to go home.

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Side effects of radiotherapy This depends on the type of radiotherapy and where the tumour is in the eye. Your doctor, specialist nurse or radiographer will explain what to expect during treatment and any likely side effects. You may have side effects during treatment or months or years after treatment. Side effects may include pain in the eye, or changes to your eyesight. Often your doctor can improve these with drugs or other treatments, such as surgery. Sometimes, radiotherapy causes permanent damage to your eyesight. But this is becoming less common as newer radiotherapy treatments are developed. We have more information about radiotherapy that we can send you. If you’d like to order information, contact our cancer support specialists free on 0808 808 00 00, Monday–Friday, 9am–8pm. Or you can order online at be.macmillan.org.uk.

Surgery This may involve removing just the tumour, a small part of the eye, or sometimes the whole eye. The type of surgery you have depends on the size and position of the tumour. Conjunctival melanoma is often treated with surgery. The specialist surgeon is usually able to remove the tumour from the surface of the eye. You may only need a local anaesthetic to have this small operation. If you need surgery to remove a uveal melanoma, you will have a general anaesthetic and a slightly longer operation. The thought of having any type of surgery to your eye can be frightening, and you may have worries about how your sight will be affected. Your eye surgeon or specialist nurse will explain what to expect and answer any questions before your operation. Rarely, some people will need a bigger operation to remove the whole eye or the eye and nearby tissue. Your surgeon will always try to preserve your eye if possible. They will only advise this type of surgery if it’s absolutely necessary. For many people, this suggestion can be quite shocking. You may need to carefully talk this over several times with your doctor and specialist nurse before you make a decision. They may arrange extra support to help you cope during this difficult time, such as counsellors or patient support groups. You may also find the useful organisations listed below helpful. Removing the eye (enucleation) This operation is not common but is sometimes used to treat uveal melanoma. If the cancer is large or in a difficult position, or if your eye is already blind and painful, the surgeon may advise removing the whole eyeball. Page 8 of 12

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During the operation, the surgeon removes the eyeball, puts an eye-shaped implant into your eye socket and covers it with tissue. The implant usually fills most of the space where your eyeball was. The surgeon attaches your eye muscles to the implant so that it moves like your eye. Several weeks after the operation, you have an artificial eye (prosthesis) made. An artist makes the prosthesis to match your other eye as closely as possible. You wear the prosthesis over the implant. Because the implant moves, your prosthesis should also move quite naturally. Removing the eye and nearby tissue (orbital exenteration) This type of surgery is rarely needed. But it may be used to treat a large conjunctival melanoma that has spread to areas around the eye. The surgeon removes the eyeball and nearby tissue such as the eyelids. After the operation, you may have a facial prosthesis (false part) that covers the eye socket with false eyelids, lashes and an artificial eye. The false eye will not be able to move or blink. The prosthesis can be worn on a pair of glasses, or fixed to your face with special glue. Sometimes, the prosthesis can be fitted to studs that the surgeon fixes into the bone around the eye socket. Anyone needing this operation will be given a lot of support from their healthcare team before surgery. You may meet a counsellor or psychologist for more specialised support.

Other treatments Other treatments are sometimes used. They may be used as the main treatment for eye melanoma, or to reduce the risk of the melanoma coming back. Transpupillary thermotherapy (TTT) This can be used to treat very small uveal melanomas, or after radiotherapy to reduce the risk of the cancer coming back. The doctor gives you a local anaesthetic then uses a type of laser beam to destroy the cancer cells by heating them. You may need more than one treatment. You can usually go home a few hours after treatment. Cryotherapy This can be given after surgery to reduce the risk of conjunctival melanoma coming back. The doctor freezes the area to kill any cancer cells that may have been left behind after surgery. You may have a local or a general anaesthetic for this treatment. Chemotherapy eye drops Sometimes you are given chemotherapy eye drops after cryotherapy for conjunctival melanoma. Your doctor or specialist nurse will explain how to use the eye drops. The drops can make your eye and eyelid red and sore. Let your doctor know if this happens so they can help.

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Photodynamic therapy (PDT) PDT may sometimes be used to treat some uveal melanomas. This treatment uses a laser, or other light sources, combined with a light-sensitive drug to destroy cancer cells. The doctor gives you the drug as an injection into your vein. This makes cells in your body more sensitive to light. The doctor then directs a light at the eye tumour. This activates the drug and the cancer cells are destroyed. This treatment is painless, but you will be sensitive to light for several days after treatment.

Clinical trials Cancer research trials are carried out to try to find new and better treatments for cancer. Trials that are carried out on patients are known as clinical trials. Research into treatments for eye melanoma is ongoing and advances are being made. Cancer doctors use clinical trials to assess new treatments. You may be asked to take part in a clinical trial. Your doctor will discuss the treatment with you so that you have a full understanding of the trial and what it means to take part. You may decide not to take part, or to withdraw from a trial at any stage. You’ll still receive the best standard treatment available.

Follow-up After your treatment, you’ll have regular check-ups and possibly scans or x-rays. It’s helpful to ask your doctor how often these scans will be. These will probably continue for several years. If you have any problems or notice any new symptoms between these times, let your doctor know as soon as possible. Eye melanoma can sometimes spread to other parts of the body – most often to the liver, but also to the lungs and bones. It’s important to let your specialist know if you notice any new symptoms, wherever they are in the body, in case further treatment is needed.

Your feelings You may have many different emotions, from shock and disbelief to fear and anger. At times, these feelings can be overwhelming and hard to control. But they are natural and it’s important to be able to express them. Everyone has their own way of coping. Some people find it helpful to talk to friends or family, while others get help from people outside their situation. Sometimes it’s helpful to share your experiences at a local cancer support group. Some people prefer to keep their feelings to themselves. There is no right or wrong way to cope, but help is there if you need it. Some people with a rare cancer want to talk to someone with the same condition. Your specialist doctor may be able to put you in touch with someone. Some of the organisations listed below also provide this. Page 10 of 12

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You may also want to contact our cancer support specialists free on 0808 808 00 00, Monday–Friday, 9am–8pm for more information and support.

Useful organisations The Rarer Cancers Foundation Unit 7B, Evelyn Court, Grinstead Road, London SE8 5AD Tel 0800 334 5551 Email [email protected] The Rarer Cancers Foundation provides guidance, information and support for people affected by rare types of cancer. OcuMel UK 139 Langley Road, Slough, Berkshire SL3 7DZ Tel 0300 790 0512 Email [email protected] OcuMel UK provides information and support for people affected by eye melanoma. Eyecancerforum eyecancerforum.co.uk Eyecancerforum is an online forum aimed at UK and European eye melanoma patients who want to share their experience and knowledge.

References and thanks The information in this section has been produced in accordance with the following sources and guidelines including the electronic Medicines Compendium (eMC; medicines.org.uk). If you’d like further information on the sources we use, please feel free to contact us.

Thanks Thank you to all of the people affected by cancer who reviewed what you’re reading and have helped our information to grow. You could help us too when you join our Cancer Voices Network - find out more. Content last reviewed: 1 March 2015 Next planned review: 2017

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We make every effort to ensure that the information we provide is accurate and up-to-date but it should not be relied upon as a substitute for specialist professional advice tailored to your situation. So far as is permitted by law, Macmillan does not accept liability in relation to the use of any information contained in this publication or third party information or websites included or referred to in it. Macmillan Cancer Support, registered charity in England and Wales (261017), Scotland (SC039907) and the Isle of Man (604). A company limited by guarantee, registered in England and Wales company number 2400969. Isle of Man company number 4694F. Registered office: 89 Albert Embankment, London SE1 7UQ. For cancer support every step of the way, call Macmillan free on 0808 808 00 00 (Mon-Fri, 9am-8pm) or visit macmillan.org.uk

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