Eye Cancer (Intraocular Lymphoma)


General Information

What is intraocular melanoma?

Intraocular melanoma, a rare cancer, is a disease in which cancer (malignant) cells are found in the part of the eye called the uvea. The uvea contains cells called melanocytes, which contain color. When these cells become cancerous, the cancer is called a melanoma. The uvea includes the iris (the colored part of the eye), the ciliary body (a muscle in the eye), and the choroid (a layer of tissue in the back of the eye). The iris opens and closes to change the amount of light entering the eye. The ciliary body changes the shape of the lens inside the eye so it can focus. The choroid layer is next to the retina, the part of the eye that makes a picture.

If there is melanoma that starts in the iris, it may look like a dark spot on the iris. If melanoma is in the ciliary body or the choroid, a person may have blurry vision or may have no symptoms, and the cancer may grow before it is noticed. Intraocular melanoma is usually found during a routine eye examination, when a doctor looks inside the eye with special lights and instruments.

The chance of recovery (prognosis) depends on the size and cell type of the cancer, where the cancer is in the eye, and whether the cancer has spread.

Stages of Intraocular Melanoma

Once intraocular melanoma is found (diagnosed), more tests will be done to find out exactly what kind of tumor the patient has and whether cancer cells have spread to other parts of the body. This is called staging. A doctor needs to know the stage to plan treatment. Intraocular melanoma is staged based on the area of the eye where the tumor is found and the size of the tumor.

Iris
Intraocular melanomas of the iris occur in the front colored part of the eye. Iris melanomas usually grow slowly and do not usually spread to other parts of the body.

Ciliary body/choroid, small size
Intraocular melanomas of the ciliary body and/or choroid occur in the back part of the eye. They are grouped by the size of the tumor. Small size ciliary body or choroid melanoma is 2 to 3 millimeters or less thick.

Ciliary body/choroid, medium/large size
Intraocular melanomas of the ciliary body and/or choroid occur in the back part of the eye. They are grouped by the size of the tumor. Medium/large size ciliary body or choroid melanoma is more than 2 to 3 millimeters thick.

Extraocular extension
The melanoma has spread outside the eye, to the nerve behind the eye (the optic nerve), or to the eye socket.

Recurrent
Recurrent disease means that the cancer has come back (recurred) after it has been treated.

Treatment Option Overview

How intraocular melanoma is treated:

There are treatments for all patients with intraocular melanoma. In some cases a doctor may watch the patient carefully without treatment until the cancer begins to grow. When treatment is given, three types of treatment are commonly used:

-Surgery (taking out the cancer)
-Radiation therapy (using high-dose x-rays or other high-energy rays to kill cancer cells)
-Photocoagulation (destroying blood vessels that feed the tumor)

Surgery is the most common treatment of intraocular melanoma. A doctor may remove the cancer using one of the following operations:

-Iridectomy removes only parts of the iris.
-Iridotrabeculectomy removes parts of the iris and the supporting tissues around the cornea, the clear layer covering the front of the eye.
-Iridocyclectomy removes parts of the iris and the ciliary body.
-Choroidectomy removes parts of the choroid.
-Enucleation removes the entire eye.

Radiation therapy uses x-rays or other high-energy rays to kill cancer cells and shrink tumors. Radiation may come from a machine outside the body (external-beam radiation therapy) or from putting materials that contain radiation (radioisotopes) in the area where the cancer cells are found (internal radiation therapy). In intraocular melanoma, internal radiation may be put next to the eye using small implants called plaques. Radiation can be used alone or in combination with surgery.

Photocoagulation is a treatment that uses a tiny beam of light, usually from a laser, to destroy blood vessels and kill the tumor.

Treatment By Stage

The choice of treatment depends on where the cancer is in the eye, how far it has spread, and the patient's general health and age.

Standard treatment may be considered because of its effectiveness in patients in past studies, or participation in a clinical trial may be considered. Not all patients are cured with standard therapy and some standard treatments may have more side effects than are desired. For these reasons, clinical trials are designed to find the best ways to treat cancer patients and are based on the most up-to-date information. A large clinical trial is ongoing in many parts of the country for patients with intraocular melanoma. To learn more about clinical trials, call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237); TTY at 1-800-332-8615.

Iris Melanoma
If the tumor is small, there are no symptoms, and the tumor is not growing, treatment may not be needed. If the tumor begins to grow or if there are symptoms, treatment may be one of the following:

1.Surgery to remove parts of the iris (iridectomy).
2.Surgery to remove parts of the iris and the supporting tissues around the cornea (iridotrabeculectomy).
3.Surgery to remove parts of the iris and the ciliary body.
4.Surgery to remove the eye (enucleation).

Ciliary Body and Choroid Melanoma, Small Size
If the tumor is small, there are no symptoms, and the tumor is not growing, treatment may not be needed. If the tumor begins to grow, or if there are symptoms, treatment may be one of the following:

1.Internal radiation therapy.
2.External beam radiation therapy.
3.Surgery to remove the tumor and part of the iris or choroid (iridocyclectomy or choroidectomy).
4.Surgery to remove the eye (enucleation).
5.External beam radiation therapy followed by enucleation.

Ciliary Body and Choroid Melanoma, Medium/Large Size
If the tumor is not growing, treatment may not be needed. If treatment is needed, it may be one of the following:

1.Internal radiation therapy.
2.External-beam radiation therapy.
3.Surgery to remove the tumor and part of the iris or choroid (iridocyclectomy or choroidectomy).
4.Surgery to remove the eye (enucleation).
5.External beam radiation therapy followed by enucleation.
6.A clinical trial. A large trial is in progress in many parts of the country comparing standard treatments. Clinical trials are also testing new treatments.

Extraocular Extension Melanoma
Treatment may be one of the following:

1.Surgery to remove the eye and other tissues in the eye socket (orbital exenteration) with or without radiation therapy.
2.Surgery to remove the eye (enucleation) with or without radiation therapy.

Recurrent Intraocular Melanoma
Treatment will depend on the treatment the patient received before, the patient's age and health, where the cancer came back, and how far the cancer has spread. The patient may want to take part in a clinical trial.






The information on this page was obtained from the National Cancer Institute. The National Cancer Institute provides accurate, up-to-date information on many types of cancer, information on clinical trials, resources for people dealing with cancer, and information for researchers and health professionals.

The National Cancer Institute is in no way affiliated with the Mary Stolfa Cancer Foundation.

The information on this web site is provided for general information only. It is not intended as medical advice, and should not be relied upon as a substitute for consultations with qualified health professionals who are familiar with your individual medical needs. The MSCF disclaims all obligations and liabilities for damages arising from the use or attempted use of the information, including but not limited to direct, indirect, special, and consequential damages, attorneys' and experts' fees and court costs. Any use of the information will be at the risk of the user.





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