Eye Cancer & Treatment

What is eye cancer?
An eye cancer is a cancer that starts in the eye. There are different types of eye cancers. There are two main areas to the eye – the front of the eye and the back, the eyeball. You can see these in the diagram below.

The eyeball
The eyeball has three layers sandwiched together

• The outer white fibrous layer, the sclera
• The middle blood rich layer, the choroid
• The inner coloured (pigmented) layer, the retina.

The inside of the eyeball is filled with a clear jelly like substance called vitreous humour. This, and the fibrous white sclera help to keep the shape of your eyeball.

The retina lines the inside of the eyeball. This is the nerve layer of the eye. The cells of the retina react to light. They send messages to the brain through the optic nerve, making it possible for you to see.

The front of the eye
The front of the eye is the bit you can see. The 3 layers continue round from the eyeball but they make up different structures in the front of the eye.

The fibrous sclera becomes clear, instead of white. This part of it is called the cornea and covers your pupil and iris.

The middle choroid layer becomes the iris and the ciliary body. The iris is the coloured part around your pupil that covers the lens of the eye. It controls how much light enters your eye.

The ciliary body lies just behind the iris. It has two functions. It is the muscle that controls the focusing of the eye. And it makes the clear fluid (aqueous humour) that fills and shapes the front of your eye.

The uvea
The middle layer of the eye is called the uvea. The front (anterior) uvea includes the iris and ciliary body. The back (posterior) uvea is the choroid. The uveal layer is the most common place for eye cancers to start. You may hear your doctor talk about uveal, iris, ciliary body or choroidal melanomas.

The tissues surrounding the eyeball (orbit)
The orbit is the tissue surrounding the eyeball. It includes

Muscles that allow the eyeball to move in different directions
Nerves attached to the eye
Cancers in this part of the eye are called orbital cancers. They are very rare.

Structures around the eye
Structures around the eye include the eyelids and tear glands. They are called accessory or adnexal structures. So doctors call cancers that develop in these tissues adnexal cancers.


Symptoms of Eye Cancer

There are several symptoms. Other eye conditions that aren’t eye cancer can cause many of these symptoms. But it is important that you report any of them to your doctor. People with eye cancer may have

• Bulging of one eye
• Complete or partial loss of sight
• Pain in or around the eye (rare with eye cancer)
• Blurred vison
• Change in the appearance of the eye

Eye cancer can also cause

• Seeing spots or flashes of light or wiggly lines in front of your eyes
• Blinkered vision (loss of peripheral vision) – you can see what is straight ahead clearly, but not what is at the sides
• A dark spot on the coloured part of the eye (the iris) that is getting bigger

Causes of Eye Cancer 

Eye colour

People with blue, grey or green eyes are more likely to develop eye melanoma than people with brown eyes. In the same way, fair skinned people are more likely to get melanoma of the skin than people with darker skin.

People who have abnormal brown spots (pigmentation) on their uvea (called oculodermal melanocytosis) are at an increased risk of developing eye melanoma too.

Moles

Some families tend to have large numbers of moles on their skin, or moles that are unusual (doctors call them atypical). The atypical moles tend to be an irregular shape or colour. They also have a tendency to become cancerous. People with moles like this have a higher than average risk of skin melanoma and eye melanoma.

Sunlight

We know that over exposure to sunlight is a definite risk factor for melanoma of the skin. It has also possibly been linked to melanoma of the eye. Men who have higher exposure to sunlight through their work have an increased risk of eye melanoma. Exposure before the age of 30 seems to be most important. Natural ageing changes in the eye mean it is unlikely that exposure to sunlight in middle age causes eye melanoma.

Squamous cell eye cancer

The risk factors for squamous cell eye cancer include

• Infection with HIV
• Drugs that suppress the immune system
• Human papilloma virus infection
• Sunlight

Infection with HIV

People who have HIV are at a higher risk of squamous cell carcinoma of the eye. This is almost certainly because of the effect of the virus on their immune systems.

Drugs that suppress the immune system

People who have an organ transplant need to take drugs to stop their immune systems rejecting the new organ. These drugs damp down the immune system generally. Because of this, these people are at an increased risk of some types of cancer, including squamous cell carcinoma of the eye.

Human papilloma virus infection

Human papilloma virus (HPV) may cause squamous cell carcinoma of the eye in combination with other factors. The virus causes squamous cell cancers elsewhere in the body. Researchers have found DNA from HPV in squamous cell cancers of the eye and in abnormal precancerous cells. People with squamous cell cancer of the eye have higher levels of antibodies to HPV in their blood than people without this type of cancer. Infection with HPV is very common and not everyone infected will get cancer. So there are probably other factors working with the HPV that explain why some people get it and others don’t. One of these co factors may be sunlight.

Sunlight

Sun exposure has been linked to a higher risk of squamous cell cancer of the eye. This type of cancer is more common in areas of the world where the sun’s light is stronger than in the UK.

Lymphoma of the eye

Anyone whose immune system is not working as well as it should be may be more likely to develop a lymphoma of the eye. This includes people who

• Are taking drugs to stop organ rejection after a transplant
• Have AIDS
• Have autoimmune diseases such as rheumatoid arthritis
• Are born with rare medical syndromes which affect their immunity

A bacteria called chlamydophila psittaci may increase the risk of a type of eye lymphoma called ocular adnexal malt lymphoma. Chlamydophila psittaci is caught by exposure to infected birds or domestic animals such as cats. It usually causes lung infections but can cause long term inflammation of the eye (conjunctivitis). Italian studies have linked ocular adnexal malt lymphoma to long term conjunctivitis due to chlamydophila psittaci. But studies in the United States have not shown an increased risk. So we need more research to see whether there is a link.

Treatment of Eye Cancer

Eye cancer treatment can fall in following categories:

• Surgery
• Radiotherapy
• Chemotherapy

Surgery

Surgery is used to treat some intraocular melanomas but is not used to treat intraocular lymphoma. It is used less often than in the past as the use of radiation therapy has grown.

The type of surgery depends on the location and size of the tumor. Patients are under general anesthesia (in a deep sleep) during these operations, and they usually leave the hospital 1 or 2 days afterward. The operations used to treat people with melanoma include:

• Iridectomy: Removal of part of the iris. This operation may be an option for very small iris melanomas.

• Iridotrabeculectomy: Removal of part of the iris, plus a small piece of the outer part of the eyeball. Small iris melanomas may be treated with this technique.

• Iridocyclectomy: Removal of a portion of the iris and the ciliary body. This operation is also used for small iris melanomas.

• Resection: Doctors in some cancer centers may try to surgically resect (remove) a melanoma of the ciliary body or choroid. This can be done for small melanomas but it is hard to remove the tumor without damaging the rest of the eye. This can lead to severe vision problems.

• Enucleation: Removal of the entire eyeball. This is used for larger melanomas (T4 or large T3 tumors), but it may also be done for some smaller melanomas if other treatment options would destroy useful vision in the eye anyway. During the same operation, an orbital implant is usually put in to take the place of the eyeball. The implant is made out of silicone or hydroxyapatite (a substance similar to bone). It is attached to the muscles that moved the eye, so it should move the same way as the eye would have. Within a few weeks after surgery, you visit an ocularist (a specialist in eye prostheses) to be fitted with an artificial eye that will match the size and color of the remaining eye. The artificial eye is a thin shell that fits over the orbital implant and under the eyelids. Once the eye is in place, it will be hard to tell it apart from the real eye.

Radiotherapy for eye melanomas

Radiotherapy uses high energy rays to kill cancer cells. It is really only a suitable treatment for small or medium sized eye melanomas.

If you have radiotherapy for a small or medium sized eye melanoma, it may be possible to save the eye and keep your sight. This will depend on where the tumour is within the eye, as well as its size. Sometimes you have surgery for your eye melanoma before you have radiotherapy.

There are different ways of giving radiotherapy to the eye. They are

• Brachytherapy – small radioactive plates stitched to the eye give the radiotherapy (local radiotherapy)
• External beam therapy – a machine directs radiotherapy beams at the tumour from outside the eye

Radiotherapy for eye lymphomas

If you have lymphoma of the eye your doctor may suggest radiotherapy as for non Hodgkin’s lymphoma. Radiotherapy to your eye and brain can clear the cancer in the eye and also helps to stop it from coming back in the brain or spinal cord.

Radiotherapy to the brain may cause problems with memory and thinking clearly. This only happens in a small number of adults. If you do develop these side effects, they can appear a few months or several years after you were first treated. Unfortunately, these late side effects are usually permanent. And occasionally they can become worse over a long period of time.

Chemotherapy

Most people with eye lymphomas will have chemotherapy. You may have the chemotherapy into the fluid around your spinal cord (intrathecal chemotherapy). You are likely to have radiotherapy as well. The chemotherapy drugs that doctors use most often are

• Methotrexate
• Cytarabine (Ara-C)
• Thiotepa

The links above take you straight to information about the specific side effects of these chemotherapy drugs.

Doctors use chemotherapy to treat eye lymphoma in the same way as for other types of non Hodgkin’s lymphomas. Look in the non Hodgkin’s lymphoma section for information about chemotherapy for lymphoma.

Lifestyle changes after  Eye Cancer

You can't change the fact that you have had cancer. What you can change is how you live the rest of your life -- making choices to help you stay healthy and feel as well as you can. This can be a time to look at your life in new ways. Maybe you are thinking about how to improve your health over the long term. Some people even start during cancer treatment.

Making healthier choices

For many people, a diagnosis of cancer helps them focus on their health in ways they may not have thought much about in the past. Are there things you could do that might make you healthier? Maybe you could try to eat better or get more exercise. Maybe you could cut down on the alcohol, or give up tobacco. Even things like keeping your stress level under control may help. Now is a good time to think about making changes that can have positive effects for the rest of your life. You will feel better and you will also be healthier.

Eating better

If treatment caused weight changes or eating or taste problems, do the best you can and keep in mind that these problems usually get better over time. You may find it helps to eat small portions every 2 to 3 hours until you feel better. You may also want to ask your cancer team about seeing a dietitian, an expert in nutrition who can give you ideas on how to deal with these treatment side effects.

One of the best things you can do after cancer treatment is put healthy eating habits into place. You may be surprised at the long-term benefits of some simple changes, like increasing the variety of healthy foods you eat. Getting to and staying at a healthy weight, eating a healthy diet, and limiting your alcohol intake may lower your risk for a number of types of cancer, as well as having many other health benefits.

Rest, fatigue, and exercise

If you were sick and not very active during treatment, it is normal for your fitness, endurance, and muscle strength to decline. Any plan for physical activity should fit your own situation. An older person who has never exercised will not be able to take on the same amount of exercise as a 20-year-old who plays tennis twice a week. If you haven't exercised in a few years, you will have to start slowly – maybe just by taking short walks.

If you are very tired, you will need to balance activity with rest. It is OK to rest when you need to. Sometimes it's really hard for people to allow themselves to rest when they are used to working all day or taking care of a household, but this is not the time to push yourself too hard. Listen to your body and rest when you need to. (For more information on dealing with fatigue, please see Fatigue in People With Cancer and Anemia in People With Cancer.)

Keep in mind exercise can improve your physical and emotional health.

• It improves your cardiovascular (heart and circulation) fitness.
• Along with a good diet, it will help you get to and stay at a healthy weight.
• It makes your muscles stronger.
• It reduces fatigue and helps you have more energy.
• It can help lower anxiety and depression.
• It can make you feel happier.
• It helps you feel better about yourself.