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 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. The uvea contains cells called melanocytes, which contain color. When these cells become cancerous, the cancer is called a melanoma.
Intraocular melanoma occurs most often in people who are middle aged. 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.
Once intraocular melanoma is found (diagnosed), more tests will be done to find out exactly what kind of tumor the patient has and whether cancercells 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.
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.
Intraocular melanomas of the ciliary body occur in the back part of the eye.
Intraocular melanomas of the choroid occur in the back part of the eye. They are grouped by the size of the tumor.
Small size choroidal melanoma is 3 millimeters or less in thickness.
Medium and large size choroidal melanomas are more than 3 millimeters in thickness.
The melanoma has spread outside the eye, to the nerve behind the eye (the optic nerve), to the eye socket, or to other parts of the body.
Recurrent disease means that the cancer has come back (recurred) after it has been 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-dosex-rays or other high-energy rays to kill cancer cells)
Laser therapy (using an intensely powerful beam of light to destroy the tumor or 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.
Orbital exenteration removes the eye and other tissues within the eye socket.
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.
Laser therapy is a treatment that uses an intensely powerful beam of light to destroy the tumor. The laser may be used with a special dye that is injected into the tumor, causing the laser to heat the dye and kill the tumor. This is called thermotherapy. Photocoagulation is another treatment that uses a laser to destroy the blood vessels that feed the tumor, killing the tumor indirectly.
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.
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:
Surgery to remove the tumor.
Surgery to remove the eye (enucleation).
Internal radiation therapy using small implants (plaques).
Treatment may be one of the following:
External-beam radiation therapy.
Surgery to remove the tumor.
Surgery to remove the eye (enucleation).
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:
Laser therapy (photocoagulation or thermotherapy).
Surgery to remove the tumor and part of the iris or choroid (iridocyclectomy or choroidectomy).
For medium sized choroidal melanomas, treatment may be one of the following:
Internal radiation therapy followed by laser therapy (coagulation or thermotherapy).
For large choroidal melanomas, treatment may be one of the following:
External-beam radiation therapy followed by enucleation.
For extraocular extension, treatment will most likely involve surgery to remove the eye and other tissues in the eye socket (orbital exenteration) with or without radiation therapy.
There is no systemic treatment for metastaticintraocular 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.
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