What is intraocular melanoma?

Melanoma is a serious kind of skin cancer. This cancer starts in cells called melanocytes. These cells make the pigment that colors your skin. You also have melanocytes in your eyes. When these cells become cancer, it’s called intraocular melanoma (also called uveal melanoma). It’s not as common as skin melanoma, but it can be just as serious.

Intraocular melanoma is rare. But it is the most common type of cancer that starts in the eye in adults. In most cases, it starts in the middle layer of the eye called the uvea. The most common part of the uvea affected is the choroid. This thin, pigmented layer of blood vessels brings oxygen and nutrients to the eye. Other parts of the uvea can also be affected. These include the ciliary body and the iris (the colored part of the eye).

Intraocular melanoma can spread to other parts of your body. These include both nearby tissues and more distant parts of the body. This happens if the cancer cells travel through your lymph system or blood. It can also affect other tissues in and around your eye. These include the thin membrane that covers the eye (conjunctiva), and the eyelid.

What causes intraocular melanoma?

Experts aren’t exactly sure what causes intraocular melanoma. It occurs when cells in the body change and grow out of control. These abnormal cells may grow to form a lump or mass called a tumor. These abnormal cells can grow into nearby areas if they are in the body long enough. Or they can spread to other parts of the body (metastasis).

Who is at risk for intraocular melanoma?

A risk factor is anything that may increase your chance of having a disease. The exact cause of someone’s cancer may not be known. But certain risk factors can make it more likely for a person to develop cancer. Some risk factors may not be in your control. But others may be things you can change.

Anyone can develop intraocular melanoma. But certain factors may make you more likely to get it. They include:

• Having fair skin that burns easily and freckles, and light-colored eyes

• Being older

• Certain inherited skin problems, such as dysplastic nevus syndrome

• Being white

Talk with your healthcare provider about your risk factors for intraocular melanoma and what you can do about them.

What are the symptoms of intraocular melanoma?

A small growth may not cause any symptoms. As it grows larger, your symptoms may include:

• Blurry vision, sudden vision loss, or other changes in vision

• Soreness in an eye, or bulging of the eye (uncommon)

• Flashes of light or “floaters” in your vision

• Dark spot on your iris, the colored ring at the front of your eye

• Change in the shape of your pupil, the black circle in the center of your eye

• A change in the way your eye moves or looks

Many of these may be caused by other health problems. So it’s important to see a healthcare provider if you have these symptoms. Your healthcare provider will do an exam and testing to find out if you have cancer.

How is intraocular melanoma diagnosed?

You will need to see a specially trained eye care provider (ophthalmologist). The ophthalmologist will ask you about your health history, symptoms, risk factors, and family history of disease. They will give you a detailed eye exam. During the exam, your eyes will be dilated to enlarge the pupil. Also a special scope with a magnifying lens and light (ophthalmoscope) will be used to look at the inside of your eye.

Your eye care provider may be able to diagnose intraocular melanoma just by looking at your eye. But you may also need one of these tests:

• Ultrasound. This test uses high-frequency sound waves to see the inside of your eye. It’s often used because melanomas in your eye have a certain look when seen on ultrasound. It can also show where the tumor is and how big it is. Your eyes will be numbed with eye drops. A special ultrasound called biomicroscopy may also be used for to see more detailed pictures.

• MRI. MRIs use radio waves and strong magnets to make an image on a computer. This test is helpful in learning the size of the tumor. It’s often used to check if the cancer has spread beyond the eye.

• Angiography. During this procedure, your healthcare provider injects dye into a blood vessel in your arm. Then they take pictures of your eye as the dye moves through it. The dye helps to show any changes more clearly.

• Biopsy. Your healthcare provider may need to take a small tissue sample from the growth. This is rarely done because other tests work well to diagnose intraocular melanoma.

After a diagnosis of intraocular melanoma, you’ll likely need other tests. These help your healthcare providers learn more about the cancer and find out the stage of the cancer. The stage is how much and how far the cancer has spread (metastasized) in your body. Your healthcare provider may also use the term “category." This is a description of how thick and wide the tumor is. These are important things to know when deciding how to treat the cancer.

Once your cancer is staged, your healthcare provider will talk with you about what the stage means for your treatment. Ask your healthcare provider to explain the stage of your cancer to you in a way you can understand.

How is intraocular melanoma treated? 

Your treatment choices depend on test results, where the tumor is, and the stage of the cancer. Your ability to see in the unaffected eye, your age, overall health, and personal choice will also be considered. The goals of treatment include saving the eye and keeping your ability to see. And keeping the cancer from spreading. Talk with your healthcare team about your treatment choices, the goals of treatment, and what the risks and side effects may be.

Treating this cancer can cause eye damage. For this reason, your healthcare provider may advise not to start treatment right away if you don’t have symptoms and the tumor isn’t growing. This is called watchful waiting. Your provider will see you regularly and take pictures of the tumor to track its growth. You can start treatment if the tumor starts to grow.

Types of treatment for cancer are either local or systemic:

• Local treatments. These remove, destroy, or control cancer cells in one area. Surgery and radiation are local treatments.

• Systemic treatment. This destroys or controls cancer cells that may have traveled around your body. When taken by pill or injection, chemotherapy is a systemic treatment.

Based on your situation, you may have several choices for treating this disease. They include:

• Surgery. This is the most common treatment used. It can be used to only remove the growth and a small area of tissue around it. But in some cases, the entire eye needs to be removed. Other nearby tissues are also sometimes removed. These can include your eyelid and muscles around the eye.

• Radiation. Different types of radiation can be used to treat this disease. One approach is using a machine that directs radiation beams into your eye to treat the cancer. Another approach is to attach a small radioactive disk on your eye next to the tumor. This may be called plaque brachytherapy. Medicines will be used to put you into a deep sleep before it starts if you have this procedure. The plaque disk often stays in place for several days before it is removed surgically.

• Photocoagulation. A special laser that destroys the tumor as well as blood vessels that feed the tumor might be used. This is mostly used for small tumors.

• Transpupillary thermotherapy. This is the use of heat to destroy cancer cells.

You may need other treatments if the cancer spreads to other parts of your body.

Talk with your healthcare providers about your treatment choices. Make a list of questions. Think about the benefits and possible side effects of each choice. Talk about your concerns with your healthcare provider before making a decision.

What can I do to prevent intraocular melanoma?

It's hard to know how to prevent intraocular melanoma since experts aren't sure exactly what causes it. You might be able to help prevent intraocular melanoma by:

• Wearing sunglasses with 99% to 100% ultraviolet A and B (UVA and UVB) protection when outside in sunlight. Some experts think this may help, but more research is needed.

When should I call my healthcare provider?

Call your healthcare provider if you have:

• New symptoms or symptoms that get worse

• Signs of an infection, such as a fever

Key points about intraocular melanoma

• Intraocular melanoma is cancer that starts in the melanocytes in your eyes.

• It’s rare, but it's still the most common type of cancer of the eye in adults.

• Risk factors for it are being older and having fair skin and light-colored eyes.

• Symptoms may include blurry vision, eye soreness, or “floaters” in your vision.

• Treatments for this cancer include surgery, radiation, and photocoagulation.

Next steps

Tips to help you get the most from a visit to your healthcare provider:

• Know the reason for your visit and what you want to happen.

• Before your visit, write down questions you want answered.

• Bring someone with you to help you ask questions and remember what your provider tells you.

• At the visit, write down the name of a new diagnosis, and any new medicines, treatments, or tests. Also write down any new instructions your provider gives you.

• Know why a new medicine or treatment is prescribed, and how it will help you. Also know what the side effects are.

• Ask if your condition can be treated in other ways.

• Know why a test or procedure is recommended and what the results could mean.

• Know what to expect if you do not take the medicine or have the test or procedure.

• If you have a follow-up appointment, write down the date, time, and purpose for that visit.

• Know how you can contact your healthcare provider if you have questions, especially after office hours and on weekends and holidays.