Intraocular melanoma

Other Names:

Melanoma of the Uvea; Uveal melanoma

Intraocular melanoma is a cancer of the pigment-producing cells (melanocytes) in the middle layer of the eye, called the uveal tract.

The uveal tract has 3 main parts: (1) the choroid (the tissue layer filled with blood vessels); (2) the ciliary body (the ring of muscle tissue that changes the size of the pupil and the shape of the lens); and (3) the iris (the colored part of the eye). Most cases (90%) of intraocular melanoma develop in the choroid, called choroidal melanoma; the ciliary body is less commonly a site of origin, and the iris is the least common. Each manifests with different clinical features and symptoms.

Malignant melanoma of the choroid.

Variably pigmented, mushroom-shaped choroidal tumor has ruptured the Bruch membrane and grown into the subretinal space.

Risk factors

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.

Signs or symptoms

Intraocular melanoma may not cause early signs or symptoms. It is sometimes found during a regular eye exam when the doctor dilates the pupil and looks into the eye. Signs and symptoms may be caused by intraocular melanoma or by other conditions. Check with your doctor if you have any of the following:

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.

For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. 80%-99% of people have these symptoms

• Choroidal melanoma
• Visual loss(Loss of vision)

30%-79% of people have these symptoms

• Ciliary body melanoma
• Iris melanoma
• Retinal detachment(Detached retina)

5%-29% of people have these symptoms

• Abnormal visual accommodation
• Abnormality of refraction
• Inferior lens subluxation
• Metamorphopsia
• Mydriasis(Dilated pupil)
• Ocular hypertension(High eye pressure)
• Photopsia
• Vitreous hemorrhage
• Zonular cataract

1%-4% of people have these symptoms

• Inflammatory abnormality of the eye
• Ocular pain(Eye pain)

Diagnosis

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

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 and goes into the bloodstream. 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.

Indocyanine green angiography: A procedure to look at blood vessels in the choroid layer of the eye. A green dye (indocyanine green) is injected into a blood vessel in the arm and goes into the bloodstream. 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.

Ocular coherence tomography: An imaging test that uses light waves to take cross-section pictures of the retina, and sometimes the choroid, to see if there is swelling or fluid beneath the retina.

A biopsy is the removal of cells or tissues so they can be viewed under a microscope to check for signs of cancer. Rarely, a biopsy of the tumor is needed to diagnose intraocular melanoma.

Prognosis

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.

Treatment

There are different types of treatments for patients with intraocular melanoma. Five types of standard treatment are used:

Surgery:

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 signs or 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.

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.

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.

The medication(s) listed below have been approved by the Food and Drug Administration (FDA) as orphan products for treatment of this condition.

• Aldesleukin (Brand name: Proleukin) Treatment of adults with metastatic melanoma.

NIH genetic and rare disease info

• NIH info on Intraocular melanoma

Intraocular melanoma is a rare disease.

Intraocular melanoma Resources
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