Childhood Intraocular (Uveal) Melanoma Treatment (PDQ®): Treatment - Patient Information [NCI]

General Information About Intraocular 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: the iris, the ciliary body, and the choroid.

Anatomy of the eye, showing the outside and inside of the eye including the sclera, cornea, iris, ciliary body, choroid, retina, vitreous humor, and optic nerve. The vitreous humor is a liquid that fills the center of the eye.

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.

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.

Stages of Childhood Intraocular 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 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. The child lies on a table that slides through the CT scanner, which takes a series of detailed x-ray pictures of areas inside the body.
• 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) scan. The child lies on a table that slides into the MRI machine, which takes a series of detailed pictures of areas inside the body. The positioning of the child on the table depends on the part of the body being imaged.
• 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.

Sometimes intraocular melanoma comes back after treatment.

The cancer may come back in the eye or in other parts of the body, such as in the lung or liver.

Treatment Option Overview

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.
  
  Plaque radiotherapy of the eye. A type of radiation therapy used to treat eye tumors. Radioactive seeds are placed on one side of a thin piece of metal (usually gold) called a plaque. The plaque is sewn onto the outside wall of the eye. The seeds give off radiation which kills the cancer. The plaque is removed at the end of treatment, which usually lasts for several days.

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).

New types of treatment are being tested in clinical trials.

This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website.

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.

To learn more about side effects that begin during treatment for cancer, visit 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.

Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI's clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website.

Follow-up tests may be needed.

As your child goes through treatment, they will have follow-up tests or check-ups. Some tests that were done to diagnose or stage the cancer may be repeated 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).

Treatment of Childhood Intraocular Melanoma

For information about the treatments listed below, see the Treatment Option Overview section.

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.

See the PDQ summary on adult Intraocular (Uveal) Melanoma Treatment for more information.

Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.

Treatment of Recurrent Childhood Intraocular Melanoma

For information about the treatments listed below, see the Treatment Option Overview section.

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.

Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.

To Learn More About Childhood Intraocular Melanoma

For more information from the National Cancer Institute about intraocular melanoma, see the following:

• Intraocular (Eye) Melanoma Home Page
• Computed Tomography (CT) Scans and Cancer
• Targeted Cancer Therapies
• Lasers to Treat Cancer

For more childhood cancer information and other general cancer resources, visit:

• About Cancer
• Childhood Cancers
• CureSearch for Children's Cancer
• Late Effects of Treatment for Childhood Cancer
• Adolescents and Young Adults with Cancer
• Children with Cancer: A Guide for Parents
• Cancer in Children and Adolescents
• Staging
• Coping with Cancer
• Questions to Ask Your Doctor about Cancer
• For Survivors and Caregivers

About This PDQ Summary

About PDQ

Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish.

PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government's center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH.

Purpose of This Summary

This PDQ cancer information summary has current information about the treatment of childhood intraocular (uveal) melanoma. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care.

Reviewers and Updates

Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.

The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Pediatric Treatment Editorial Board.

Clinical Trial Information

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.

Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).

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The best way to cite this PDQ summary is:

PDQ® Pediatric Treatment Editorial Board. PDQ Childhood Intraocular (Uveal) Melanoma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/eye/patient/child-intraocular-melanoma-treatment-pdq. Accessed <MM/DD/YYYY>.

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Last Revised: 2021-08-24

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