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: the iris, the ciliary body, and the choroid.

Having a fair complexion and certain conditions can affect the risk of 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 child's doctor if you think your child may be at risk.

Risk factors for childhood intraocular melanoma include the following:

• Light eye color.
• Fair skin color.
• Not being able to tan.
• Oculodermal melanocytosis.
• Cutaneous nevi.

Signs and symptoms of intraocular melanoma include trouble seeing or a dark spot on the iris.

These and other signs and symptoms may be caused by intraocular melanoma or by other conditions.

Check with your child's doctor if your child has any of the following:

• Trouble seeing.
• Dark spot on the iris (colored part of the eye).
• A bulging eye.

Tests that examine the eye are used to diagnose intraocular melanoma.

The following tests and procedures may be used:

• Physical exam and health 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.
• Ultrasound: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. The picture can be printed to be looked at later.
• Fluorescein angiography: A test used to take pictures of the retina in the eye. A yellow dye is injected into a vein and travels throughout the body including the blood vessels in the eye. The yellow dye causes the vessels in the eye to fluoresce when a picture is taken.

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 to areas near the eye or to other parts of the body is called staging. There is no standard staging system for childhood intraocular melanoma.

The tests and procedures used to diagnose cancer and other tests and procedures may be used to find out if cancer has spread and plan treatment:

• 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 that cancer has spread to the liver.
• CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the chest or liver, 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.
• Computed tomography (CT) scan of the abdomen. The child lies on a table that slides through the CT scanner, which takes x-ray pictures of the inside of the abdomen.
• MRI: A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body, such as the liver. This procedure is also called nuclear magnetic resonance imaging (NMRI).​​​​
• ​​​ Magnetic resonance imaging (MRI) of the abdomen. The child lies on a table that slides into the MRI scanner, which takes pictures of the inside of the body. The pad on the child’s abdomen helps make the pictures clearer.
• Chest x-ray: An x-ray of 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.

There are three ways cancer spreads in the body.

Cancer can spread through tissue, the lymph system, and the blood:

• Tissue. The cancer spreads from where it began by growing into nearby areas.
• Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body.
• Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body.

Cancer may spread from where it began to other parts of the body.

When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood.

• Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body.
• Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body.

The metastatic tumor is the same type of cancer as the primary tumor. For example, if intraocular melanoma spreads to the liver, the cancer cells in the liver are actually intraocular melanoma cells. The disease is metastatic intraocular melanoma, not liver cancer.

Treatment Option Overview

KEY POINTS

• There are different types of treatment for children with intraocular melanoma.
• Children with intraocular melanoma should have their treatment planned by a team of doctors who are experts in treating childhood cancer.
• Three types of standard treatment are used:
  • Surgery
  • Radiation therapy
  • Laser surgery
• New types of treatment are being tested in clinical trials.
  • Targeted therapy
• Treatment for childhood intraocular melanoma may cause side effects.
• Patients may want to think about taking part in a clinical trial.
• Patients can enter clinical trials before, during, or after starting their cancer treatment.
• Follow-up tests may be needed.

There are different types of treatment for children 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.

Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment.

Children with intraocular melanoma should have their treatment planned by a team of doctors who are experts in treating childhood cancer.

Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other pediatric health professionals who are experts in treating children with cancer and who specialize in certain areas of medicine. This may include the following specialists and others:

• Pediatrician.
• Pediatric surgeon.
• Radiation oncologist.
• Pediatric ophthalmologist.
• Social worker.
• Rehabilitation specialist.
• Psychologist.
• Child-life specialist.

Three types of standard treatment are used:

Surgery

During surgery, all or part of the eye with cancer is removed. Whether all or part of the eye is removed during surgery depends on the size of the cancer and where it is in the eye.

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 area of the body with cancer.
• Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. For intraocular melanoma, localized plaque radiation therapy is used. Radioactive seeds are attached to one side of a disk, called a plaque, and placed directly on the outside wall of the eye near the tumor. The side of the plaque with the seeds on it faces the eyeball, aiming radiation at the tumor. The plaque helps protect other nearby tissue from the radiation.

Laser surgery

Laser surgery uses a laser beam (a narrow beam of intense light) to turn the cancer cells into a gas that evaporates (dissolves into the air).

Targeted therapy

Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do.

Targeted therapy is being studied for the treatment of childhood intraocular melanoma that has recurred (come back).

Treatment for childhood intraocular melanoma may cause side effects.

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.

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.

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 child's condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.

Treatment of Childhood Intraocular Melanoma

Treatment of newly diagnosed intraocular melanoma in children is like treatment for adults and may include the following:

• Surgery to remove the tumor.
• Radiation therapy.
• Laser surgery.

Treatment of Recurrent Childhood Intraocular Melanoma

Treatment of recurrent intraocular melanoma in children may include the following:

• A clinical trial that checks a sample of the patient's tumor for certain gene changes. The type of targeted therapy that will be given to the patient depends on the type of gene change.

Certain factors affect prognosis (chance of recovery).

Prognosis depends on the following:

• The size of the tumor.
• The child's age.
• Whether the tumor is in the ciliary body.
• Whether the tumor is outside the sclera.
• 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.