Intraocular melanoma is a disease in which malignant (cancer) cells form in the tissues of the eye.
Intraocular melanoma begins in the middle of 3 layers of the wall of the eye. The outer layer includes the white sclera (the "white of the eye") and the clear cornea at the front of the eye. The inner layer has a lining of nerve tissue, called the retina, which senses light and sends images along the optic nerve to the brain.
The middle layer, where intraocular melanoma forms, is called the uvea or uveal tract, and has 3 main parts:
The iris is the colored area at the front of the eye (the "eye color"). It can be seen through the clear cornea. The pupil is in the center of the iris and it changes size to let more or less light into the eye.
The ciliary body is a ring of tissue with muscle fibers that change the size of the pupil and the shape of the lens. It is found behind the iris. Changes in the shape of the lens help the eye focus. The ciliary body also makes the clear fluid that fills the space between the cornea and the iris.
The choroid is the layer of blood vessels that bring oxygen and nutrients to the eye. Most intraocular melanomas begin in the choroid.
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Intraocular melanoma is a rare cancer, but it is the most common eye cancer in adults.
Age and sun exposure may increase the risk of developing intraocular melanoma.
Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. People who think they may be at risk should discuss this with their doctor. Risk factors for intraocular melanoma include the following:
Possible signs of intraocular melanoma include a dark spot on the iris or blurred vision.
Intraocular melanoma may not cause any early symptoms. It is sometimes found during a routine eye exam when the doctor dilates the pupil and looks into the eye. The following symptoms may be caused by intraocular melanoma or by other conditions. A doctor should be consulted if any of these problems occur:
Glaucoma may develop if the tumor causes the retina to separate from the eye. If this happens, there may be no symptoms, or symptoms may include the following:
Tests that examine the eye are used to help detect (find) and diagnose intraocular melanoma.
The following tests and procedures may be used:
Certain factors affect prognosis (chance of recovery) and treatment options.
The prognosis (chance of recovery) and treatment options depend on the following:
In patients with small tumors that have not spread, intraocular melanoma can be cured and vision can usually be saved.
After intraocular melanoma has been diagnosed, tests are done to find out if cancer cells have spread within the eye or to other parts of the body.
The process used to find out if cancer has spread within the eye or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The following tests and procedures may be used in the staging process:
Sometimes pictures of the tumor are taken over a period of time and compared to see if the tumor has grown.
The following sizes are used to describe intraocular melanoma:
Small
The tumor is at least 5 millimeters in diameter and from 1 to 3 millimeters thick.
Medium
The tumor is less than 16 millimeters in diameter and from 2 to 10 millimeters thick.
Large
The tumor is at least 16 millimeters in diameter or more than 10 millimeters thick.
Diffuse
The tumor is flat and grows widely across the uvea.
There are three ways that cancer spreads in the body.
The three ways that cancer spreads in the body are:
When cancer cells break away from the primary (original) tumor and travel through the lymph or blood to other places in the body, another (secondary) tumor may form. This process is called metastasis. The secondary (metastatic) tumor is the same type of cancer as the primary tumor. For example, if breast cancer spreads to the bones, the cancer cells in the bones are actually breast cancer cells. The disease is metastatic breast cancer, not bone cancer.
Intraocular melanoma may spread to nearby tissues or to other parts of the body.
If intraocular melanoma spreads to the optic nerve or nearby tissue of the eye socket, it is called extraocular extension. Intraocular melanoma may also be metastatic and spread to the liver, lung, or bone, or to areas under the skin.
Recurrent intraocular melanoma is cancer that has recurred (come back) after it has been treated. The melanoma may come back in the eye, or in other parts of the body.
There are different types of treatments for patients with intraocular melanoma.
Different types of treatments are available for patients with intraocular melanoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Five types of standard treatment are used:
Surgery
Surgery is the most common treatment for intraocular melanoma. The following types of surgery may be used:
Watchful Waiting
Watchful waiting is closely monitoring a patient’s condition without giving any treatment until symptoms appear or change. A series of pictures is taken over time to keep track of changes in the size of the tumor and how fast it is growing.
Radiation therapy
Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy. External radiation therapy uses a machine outside the body to send radiation toward the cancer. Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated.
Localized plaque radiation therapy is a type of internal radiation therapy that may be used for tumors of the eye. Radioactive seeds are attached to a disk, called a plaque, and placed directly on the wall of the eye where the tumor is located. The side with the seeds faces the eyeball and delivers radiation to the eye. The plaque, which is often made of gold, helps protect nearby tissues from radiation damage.
Charged-particle radiation therapy is a type of external radiation therapy. A special radiation therapy machine aims tiny, invisible particles, called protons or helium ions, at the cancer cells to kill them with little damage to nearby normal tissues. Charged-particle radiation therapy uses a different type of radiation than the x-ray type of radiation therapy.
Gamma Knife radiosurgery may be used for some melanomas. This non-surgical treatment aims tightly focused gamma rays directly at the tumor so there is little damage to healthy tissue. Gamma Knife is a type of stereotactic radiosurgery.
Photocoagulation
Photocoagulation is a procedure that uses laser light to destroy blood vessels that supply nutrients to the tumor, causing the tumor cells to die. Photocoagulation may be used to treat small tumors. This is also called light coagulation.
Thermotherapy
Thermotherapy is the use of heat to destroy cancer cells. Thermotherapy may be given using:
New types of treatment are being tested in clinical trials.
Information about clinical trials is available from the NCI Web site.
Patients may want to think about taking part in a clinical trial.
For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment.
Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.
Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.
Patients can enter clinical trials before, during, or after starting their cancer treatment.
Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.
Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's clinical trials database.
Follow-up tests may be needed.
Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. This is sometimes called re-staging.
Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.
A link to a list of current clinical trials is included for each treatment section. For some types or stages of cancer, there may not be any trials listed. Check with your doctor for clinical trials that are not listed here but may be right for you.
Treatment of iris melanoma may include the following:
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with iris melanoma.
Treatment of tumors in the ciliary body and choroid may include the following:
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with ciliary body and choroid melanoma, small size.
Treatment of small choroid melanoma may include the following:
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with ciliary body and choroid melanoma, small size.
Treatment of medium choroid melanoma may include the following:
Treatment of large choroid melanoma may include surgery (enucleation) with or without radiation therapy before surgery.
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with ciliary body and choroid melanoma, medium/large size.
Treatment of extraocular extension melanoma or metastatic melanoma may include surgery (exenteration) with or without radiation therapy.
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with extraocular extension melanoma and metastatic intraocular melanoma.
Treatment of recurrent intraocular melanoma is usually within a clinical trial.
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with recurrent intraocular melanoma.
For more information from the National Cancer Institute about intraocular (eye) melanoma, see the Melanoma Home Page.
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The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.
Several enhancements have been made to this summary to better explain certain medical concepts and to help readers find information about clinical trials. The following changes were made: