Cancer Information

Intraocular (Uveal) Melanoma Treatment (PDQ®)


General Information About Intraocular (Uveal) Melanoma

Intraocular melanoma is a disease in which malignant (cancer) cells form in the tissues of the eye.

Intraocular melanoma begins in the middle of three 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 three main parts:

  • Iris 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. Intraocular melanoma of the iris is usually a small tumor that grows slowly and rarely spreads to other parts of the body.
  • Ciliary bodyThe 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. Intraocular melanoma of the ciliary body is often larger and more likely to spread to other parts of the body than intraocular melanoma of the iris.
  • ChoroidThe choroid is a layer of blood vessels that bring oxygen and nutrients to the eye. Most intraocular melanomas begin in the choroid. Intraocular melanoma of the choroid is often larger and more likely to spread to other parts of the body than intraocular melanoma of the iris.
Eye anatomy; two-panel drawing shows the outside and inside of the eye. The top panel shows outside of the eye including the eyelid, pupil, sclera, and iris; the bottom panel shows inside of the eye including the cornea, lens, ciliary body, retina, choroid, optic nerve, and vitreous humor.

Intraocular melanoma is a rare cancer that forms from cells that make melanin in the iris, ciliary body, and choroid. It is the most common eye cancer in adults.

Being older and having fair skin may increase the risk of 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. Talk with your doctor if you think you may be at risk.

Risk factors for intraocular melanoma include the following:

  • Having a fair complexion, which includes the following: Fair skin that freckles and burns easily, does not tan, or tans poorly.Blue or green or other light-colored eyes.
  • Older age.
  • Being white.

Possible signs of intraocular melanoma include blurred vision or a dark spot on the iris.

Intraocular melanoma may not cause any early symptoms. It is sometimes found during a regular 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. Check with your doctor if you have any of the following problems:

  • Blurred vision or other change in vision.
  • Floaters (spots that drift in your field of vision) or flashes of light.
  • A dark spot on the iris.
  • A change in the size or shape of the pupil.
  • A change in the position of the eyeball in the eye socket.

Tests that examine the eye are used to help detect (find) and diagnose intraocular melanoma.

The following tests and procedures may be used:

  • Physical exam and history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.
  • Eye exam with dilated pupil: An exam of the eye in which the pupil is dilated (enlarged) with medicated eye drops to allow the doctor to look through the lens and pupil to the retina. The inside of the eye, including the retina and the optic nerve, is checked. Pictures may be taken over time to keep track of changes in the size of the tumor. There are several types of eye exams:Ophthalmoscopy: An exam of the inside of the back of the eye to check the retina and optic nerve using a small magnifying lens and a light.Slit-lamp biomicroscopy: An exam of the inside of the eye to check the retina, optic nerve, and other parts of the eye using a strong beam of light and a microscope.Gonioscopy: An exam of the front part of the eye between the cornea and iris. A special instrument is used to see if the area where fluid drains out of the eye is blocked.
  • Ultrasound exam of the eye: A procedure in which high-energy sound waves (ultrasound) are bounced off the internal tissues of the eye to make echoes. Eye drops are used to numb the eye and a small probe that sends and receives sound waves is placed gently on the surface of the eye. The echoes make a picture of the inside of the eye and the distance from the cornea to the retina is measured. The picture, called a sonogram, shows on the screen of the ultrasound monitor.
  • High-resolution ultrasound biomicroscopy: A procedure in which high-energy sound waves (ultrasound) are bounced off the internal tissues of the eye to make echoes. Eye drops are used to numb the eye and a small probe that sends and receives sound waves is placed gently on the surface of the eye. The echoes make a more detailed picture of the inside of the eye than a regular ultrasound. The tumor is checked for its size, shape, and thickness, and for signs that the tumor has spread to nearby tissue.
  • Transillumination of the globe and iris: An exam of the iris, cornea, lens, and ciliary body with a light placed on either the upper or lower lid.
  • Fluorescein angiography: A procedure to look at blood vessels and the flow of blood inside the eye. An orange fluorescent dye (fluorescein) is injected into a blood vessel in the arm. As the dye travels through blood vessels of the eye, a special camera takes pictures of the retina and choroid to find any areas that are blocked or leaking.

A biopsy of the tumor is rarely needed to diagnose intraocular melanoma.

A biopsy is the removal of cells or tissues so they can be viewed under a microscope to check for signs of cancer. A biopsy of the tumor is usually not needed to diagnose intraocular melanoma. A biopsy may damage the eye or spread the tumor from the inside of the eye to the outside of the eye. Rarely, a biopsy of the tumor is needed for diagnosis. Tissue that is removed during a biopsy or surgery to remove the tumor may be tested to get more information about prognosis and which treatment options are best.

The following tests may be done on the sample of tissue:

  • Cytogenetic analysis: A laboratory test in which cells in a sample of tissue are viewed under a microscope to look for certain changes in the chromosomes.
  • Gene expression profiling: A laboratory test in which cells in a sample of tissue are checked for certain types of RNA.

Certain factors affect prognosis (chance of recovery) and treatment options.

The prognosis (chance of recovery) and treatment options depend on the following:

  • How the melanoma cells look under a microscope.
  • The size and thickness of the tumor.
  • The part of the eye the tumor is in (the iris, ciliary body, or choroid).
  • Whether the tumor has spread within the eye or to other places in the body.
  • Whether there are certain changes in the genes linked to intraocular melanoma.
  • The patient's age and general health.
  • Whether the tumor has recurred (come back) after treatment.

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Stages of Intraocular (Uveal) Melanoma

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:

  • Blood chemistry studies: A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that makes it.
  • Liver function tests: A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by the liver. A higher than normal amount of a substance can be a sign the cancer has spread to the liver.
  • Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs, such as the liver, and make echoes. The echoes form a picture of body tissues called a sonogram.
  • Chest x-ray: An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body.
  • MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI).
  • CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the liver or brain, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
  • PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A very small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. Sometimes a PET scan and a CT scan are done at the same time. If there is any cancer, this increases the chance that it will be found.

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 5 to 16 millimeters in diameter and from 1 to 3 millimeters thick. Millimeters; drawing shows millimeters (mm) using everyday objects. A sharp pencil point shows 1 mm, a new crayon point shows 2 mm, and a new pencil-top eraser shows 5 mm.

Medium

The tumor is 16 millimeters or smaller in diameter and from 3.1 to 8 millimeters thick.

Large

The tumor is:

  • more than 8 millimeters thick and any diameter; or
  • at least 2 millimeters thick and more than 16 millimeters in diameter.

Though most intraocular melanoma tumors are raised, some are flat. These diffuse tumors grow widely across the uvea.

There are three ways that cancer spreads in the body.

The three ways that cancer spreads in the body are:

  • Through tissue. Cancer invades the surrounding normal tissue.
  • Through the lymph system. Cancer invades the lymph system and travels through the lymph vessels to other places in the body.
  • Through the blood. Cancer invades the veins and capillaries and travels through the blood to other places in the body.

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.

There are two staging systems for intraocular melanoma.

Intraocular melanoma has two staging systems. The staging system used depends on where in the eye the cancer first formed:

The following stages are used for intraocular melanoma of the iris:

Stage I

In stage I, the tumor is in the iris only and is not more than one fourth the size of the iris.

Stage II

Stage II is divided into stages IIA and IIB.

Stage III

Stage III is divided into stages IIIA and IIIB.

  • In stage IIIA, the tumor has spread through the sclera to the outside of the eyeball. The part of the tumor outside the eyeball is not more than 5 millimeters thick.
  • In stage IIIB, the tumor has spread through the sclera to the outside of the eyeball. The part of the tumor outside the eyeball is more than 5 millimeters thick.

Stage IV

In stage IV, the tumor may be any size and has spread:

  • to nearby lymph nodes; or
  • to other parts of the body.

The following stages are used for intraocular melanoma of the ciliary body and choroid:

Intraocular melanoma of the ciliary body and choroid is grouped into four size categories. The category depends on how wide and thick the tumor is. Category 1 tumors are the smallest and category 4 tumors are the biggest.

Category 1:

  • The tumor is not more than 12 millimeters wide and not more than 3 millimeters thick; or
  • the tumor is not more than 9 millimeters wide and 3.1 to 6 millimeters thick.

Category 2:

  • The tumor is 12.1 to 18 millimeters wide and not more than 3 millimeters thick; or
  • the tumor is 9.1 to 15 millimeters wide and 3.1 to 6 millimeters thick; or
  • the tumor is not more than 12 millimeters wide and 6.1 to 9 millimeters thick.

Category 3:

  • The tumor is 15.1 to 18 millimeters wide and 3.1 to 6 millimeters thick; or
  • the tumor is 12.1 to 18 millimeters wide and 6.1 to 9 millimeters thick; or
  • the tumor is 3.1 to 18 millimeters wide and 9.1 to 12 millimeters thick; or
  • the tumor is 9.1 to 15 millimeters wide and 12.1 to 15 millimeters thick.

Category 4:

  • The tumor is more than 18 millimeters wide and may be any thickness; or
  • the tumor is 15.1 to 18 millimeters wide and more than 12 millimeters thick; or
  • the tumor is 12.1 to 15 millimeters wide and more than 15 millimeters thick.

Stage I

In stage I, the tumor is size category 1 and is in the choroid only.

Stage II

Stage II is divided into stages IIA and IIB.

  • In stage IIA, the tumor: is size category 1 and has spread to the ciliary body; oris size category 1 and has spread through the sclera to the outside of the eyeball. The part of the tumor outside the eyeball is not more than 5 millimeters thick. The tumor may have spread to the ciliary body; oris size category 2 and is in the choroid only.
  • In stage IIB, the tumor:is size category 2 and has spread to the ciliary body; oris size category 3 and is in the choroid only.

Stage III

Stage III is divided into stages IIIA, IIIB, and IIIC.

  • In stage IIIA, the tumor:is size category 2 and has spread through the sclera to the outside of the eyeball. The part of the tumor outside the eyeball is not more than 5 millimeters thick. The tumor may have spread to the ciliary body; oris size category 3 and has spread to the ciliary body; oris size category 3 and has spread through the sclera to the outside of the eyeball. The part of the tumor outside the eyeball is not more than 5 millimeters thick. The tumor has not spread to the ciliary body; oris size category 4 and is in the choroid only.
  • In stage IIIB, the tumor:is size category 3 and has spread through the sclera to the outside of the eyeball. The part of the tumor outside the eyeball is not more than 5 millimeters thick. The tumor has spread to the ciliary body; oris size category 4 and has spread to the ciliary body; oris size category 4 and has spread through the sclera to the outside of the eyeball. The part of the tumor outside the eyeball is not more than 5 millimeters thick. The tumor has not spread to the ciliary body.
  • In stage IIIC, the tumor:is size category 4 and has spread through the sclera to the outside of the eyeball. The part of the tumor outside the eyeball is not more than 5 millimeters thick. The tumor has spread to the ciliary body; ormay be any size and has spread through the sclera to the outside of the eyeball. The part of the tumor outside the eyeball is more than 5 millimeters thick. The tumor has not spread to the ciliary body.

Stage IV

In stage IV, the tumor may be any size and has spread:

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Recurrent Intraocular (Uveal) Melanoma

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.

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Treatment Option Overview

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:

  • Resection: Surgery to remove the tumor and a small amount of healthy tissue around it.
  • Enucleation: Surgery to remove the eye and part of the optic nerve. This is done if vision cannot be saved and the tumor is large, has spread to the optic nerve, or causes high pressure inside the eye. After surgery, the patient is usually fitted for an artificial eye to match the size and color of the other eye.
  • Exenteration: Surgery to remove the eye and eyelid, and muscles, nerves, and fat in the eye socket. After surgery, the patient may be fitted for an artificial eye to match the size and color of the other eye or a facial prosthesis.

Watchful Waiting

Watchful waiting is closely monitoring a patient’s condition without giving any treatment until symptoms appear or change. Pictures are taken over time to keep track of changes in the size of the tumor and how fast it is growing.

Watchful waiting is used for patients who do not have symptoms and the tumor is not growing. It is also used when the tumor is in the only eye with useful vision.

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-beam 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. The plaque is 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.Plaque radiotherapy of the eye; drawing shows a cross-section of the eye. An inset shows a plaque with radioactive seeds; it is placed on the outside of the eye with the seeds aimed at the cancer. Also shown are the sclera, choroid, retina, and optic nerve.

Charged-particle external beam radiation therapy is a type of external-beam 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 therapy is a type of stereotactic radiosurgery used for some melanomas. This treatment can be given in one treatment. It aims tightly focused gamma rays directly at the tumor so there is little damage to healthy tissue. Gamma Knife therapy does not use a knife to remove the tumor and is not an operation.

Photocoagulation

Photocoagulation is a procedure that uses laser light to destroy blood vessels that bring 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 from a laser to destroy cancer cells and shrink the tumor.

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 listing of clinical trials.

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.

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Treatment Options for Intraocular (Uveal) Melanoma

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.

Iris Melanoma

Treatment of iris melanoma may include the following:

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with iris melanoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.

Ciliary Body Melanoma

Treatment of tumors in the ciliary body and choroid may include the following:

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with ciliary body and choroid melanoma, small size. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.

Choroid Melanoma

Treatment of small choroid melanoma may include the following:

Treatment of medium choroid melanoma may include the following:

  • Plaque radiation therapy with or without photocoagulation or thermotherapy.
  • Charged-particle external-beam radiation therapy.
  • Surgery (resection or enucleation).

Treatment of large choroid melanoma may include the following:

  • Enucleation when the tumor is too large for treatments that save the eye.

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with ciliary body and choroid melanoma, small size. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.

Extraocular Extension Melanoma and Metastatic Intraocular (Uveal) Melanoma

Treatment of extraocular extension melanoma that has spread to the bone around the eye may include the following:

An effective treatment for metastatic intraocular melanoma has not been found. A clinical trial may be a treatment option. Talk with your doctor about your treatment options.

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with extraocular extension melanoma and metastatic intraocular melanoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.

Recurrent Intraocular (Uveal) Melanoma

An effective treatment for recurrent intraocular melanoma has not been found. A clinical trial may be a treatment option. Talk with your doctor about your treatment options.

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with recurrent intraocular melanoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.

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Changes to This Summary (05/09/2013)

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.

Changes were made to this summary to match those made to the health professional version and images were added.

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PDQ is a comprehensive cancer database available on NCI's Web site.

PDQ is the National Cancer Institute's (NCI's) comprehensive cancer information database. Most of the information contained in PDQ is available online at NCI's Web site. PDQ is provided as a service of the NCI. The NCI is part of the National Institutes of Health, the federal government's focal point for biomedical research.

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A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." 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.

Listings of clinical trials are included in PDQ and are available online at NCI's Web site. Descriptions of the trials are available in health professional and patient versions. Many cancer doctors who take part in clinical trials are also listed in PDQ. For more information, call the Cancer Information Service 1-800-4-CANCER (1-800-422-6237).

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This information was last updated on 2013-05-09