What is Eye Cancer – Detection, Diagnosis, and Staging

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About Eye Cancer

Eye cancer starts in the cells in or around the eye. A cancerous (malignant) tumour is a group of cancer cells that can grow into and destroy nearby tissue. It can also spread (metastasize) to other parts of the body.

The eye is the organ that lets you see. The eyeball is the main part of the eye. It includes the iris (the thin, muscular coloured part of the eye) and the pupil (the small, black central area of the eye). The eyeball sits in and is protected by the eye socket (orbit). The orbit is a bowl-shaped cavity made up of bone formed from the skull that contains the eyeball, muscles, lacrimal gland (tear gland), nerves, fat and connective tissues. The parts surrounding the eyeball are called accessory (adnexal) structures and include the eyelid, conjunctiva (a clear mucous membrane that covers the inner surface of the eyelid and the outer surface of the eye) and the lacrimal glands.

Eye cancer is rare. There are a number of different cancers that can affect the eye. Eye (ocular) melanoma is the most common.

Eye melanoma usually doesn’t cause any symptoms and may be found by an optician during a routine eye test. 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 can be caused by other eye conditions but it’s a good idea to get them checked.

If you are diagnosed with eye melanoma, you will have further tests to find out the stage and grade of the cancer. This helps doctors plan the best treatment for you. The aim of treatment is to destroy the cancer cells, while doing as little damage to your eyesight as possible.

It’s natural to be feeling lots of different emotions at this difficult time. You may find it helps to talk about your worries with someone.

Cells in the eye sometimes change and no longer grow or behave normally. These changes may lead to non-cancerous (benign) tumours in the eye such as a choroidal hemangioma or an eye mole.

Changes to cells of the eye can also cause precancerous conditions. This means that the abnormal cells are not yet cancer, but there is a chance that they may become cancer if they aren’t treated. The most common precancerous conditions of the eye are primary acquired melanosis of the conjunctiva and ocular melanocytosis (having a high number of pigment-making cells and extra pigment in and around the eyes).

In some cases, changes to eye cells can cause eye cancer. Most often, eye cancer in adults starts in melanocytes. These cells make melanin, which is a pigment that gives the eyes, skin and hair their colour. This type of cancer is called melanoma. Melanoma of the eye can start in different parts of the eye. Melanoma most often starts to grow inside of the eyeball and is called intraocular melanoma.

Lymphoma is another type of cancer that can affect the eye. It is the 2nd most common type of eye cancer.

Rare types of eye cancer can also develop. These include squamous cell carcinoma, basal cell carcinoma and sebaceous carcinoma.

Retinoblastoma is the most common eye cancer in children. It starts in the cells of the retina. Find out more about retinoblastoma.

Other types of cancer can spread to the eye, but this is not the same disease as primary eye cancer. Cancer that starts in another part of the body and spreads to the eye is called eye metastasis. Eye metastasis is more common than primary eye cancer. It’s usually not treated in the same way as primary eye cancer. Most often, cancer spreads to the eye from the breast, lung or gastrointestinal (GI) tract.

Types of eye cancer

Intraocular melanoma (uveal melanoma). This is the most common type of eye cancer. It usually develops in the choroid or ciliary body.

Intraocular lymphoma. A very rare type of lymphoma (typically non-Hodgkin) that starts in the eyeball.

Eyelid tumors. Basal cell carcinoma, a type of skin cancer, is the most common type of eyelid cancer. It can be removed with surgery. Other types of eyelid tumors include squamous cell carcinoma, sebaceous cell carcinoma, and malignant melanoma.

Conjunctival tumors. These are tumors that grow on the surface of the eye. These tumors include squamous carcinomas, melanomas, and lymphomas.

Lacrimal gland tumors. Tumors found in the tear glands.

Retinoblastoma. This cancer of the eye is the most common type in children. It’s extremely rare in adults.

What is eye melanoma?

Eye cancer is rare. There are a number of different cancers that can affect the eye. Eye (ocular) melanoma is the most common and this information is mainly about this type.

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. ery 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.

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

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.

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

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.

Genetics is thought to play a role in eye cancer. In regards to retinoblastoma, it is believed that if a tumor develops only in one eye then it isn’t hereditary. However, if a tumor occurs in both eyes then it is hereditary. Those who have hereditary retinoblastoma have a rare risk of developing a tumor in the brain and should be monitored on a regular basis.

The causes of intraocular melanoma are not fully understood as of the early 2000s. Age is a factor as well as genetic inheritance. In 2004, a group of ophthalmologists in Scotland identified mutations in the BRAF gene in samples of tissue taken from conjunctival melanomas. Interestingly enough, this type of cancer is seen most often in white people from a northern European descent.

The symptoms of this type of cancer usually begin with blurred vision and tenderness of the eye. Advanced symptoms may include loss of vision. If these symptoms persist a person should make an appointment with an eye specialist.

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.

The diagnosis of eye cancer is usually made by an ophthalmologist, who is a doctor who specializes in treating eye disorders. In the case of cancerous growths, the doctor is usually able to see the tumor through the pupil or directly on the iris if the cancer is intraocular melanoma of the iris. Because the doctor can usually readily see the tumor a biopsy is rarely needed.

An ultrasound or a fluorescein angiography are two tests doctors use to further diagnose eye cancers. In an ultrasound sound waves are pointed at the tumor and depending on how they reflect off the tumor the doctor can better diagnose it. In a fluorescein angiography a fluorescent dye is injected into the patients arm. When this dye circulates through the body and reaches the eye a series of rapid pictures are taken through the pupil. The tumor will show up in these photos.

Once a diagnosis has been made, the treatment can begin.

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.

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.

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.

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.

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.

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.

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 it helpful to talk to our cancer support specialists at this time.

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.

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.

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.

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