Intraocular Melanoma

Overview

What is intraocular melanoma?

Intraocular melanoma is a rare cancer that forms inside the eye. It causes melanocytes (cells that produce pigment, or color) to grow out of control. The disease can lead to vision changes or loss.

Intraocular melanoma usually affects the middle part of the eye called the uvea. The uvea is between the sclera (outer white part of the eye) and the retina (inner part of the eye that processes light and controls vision).

Intraocular melanoma is also called uveal melanoma. It can form in any of the three parts of the uvea:

  • Iris (colored part of the eye).
  • Ciliary body (ring of muscle fibers behind the iris).
  • Choroid (tissue with blood vessels that bring oxygen and nutrients to the eye).

This type of eye cancer usually starts in the choroid. It can metastasize (spread) from the uvea to other parts of the body, usually the liver.

Intraocular melanoma affects the same types of cells as skin melanoma, but they’re different conditions.

Does intraocular melanoma cause vision loss or blindness?

Some vision loss is common with intraocular melanoma. The amount of vision loss depends on the size of the melanoma and how long you have had it. Long-standing eye melanomas may cause complete loss of vision.

Who is at risk of getting intraocular melanoma?

The following risk factors make you more likely to develop eye melanoma:

  • Being white.
  • Light-colored eyes or skin.
  • Being between 70 and 80 years old.
  • Family history of uveal melanoma (rare).

How common is intraocular melanoma?

Intraocular melanoma is the most common type of eye cancer in adults. But the condition is very rare. Healthcare providers diagnose about 2,500 people in the U.S. with intraocular melanoma each year.

Symptoms and Causes

What causes intraocular melanoma?

Genes, which are made of DNA, give instruction to cells about how to multiply. But if a gene mutates (changes), it might allow cells to multiply out of control. That leads to cancer.

With intraocular melanoma, mutated DNA give the wrong instructions to melanocytes in the eye. The melanocytes grow and form tumors.

What are the symptoms of intraocular melanoma?

In some cases, intraocular melanoma may not cause symptoms. Or symptoms may be difficult to spot since the cancer is in part of the eye that isn’t visible.

When symptoms do occur, they can include:

  • Blind spots or a reduced field of vision.
  • Blurred or low vision.
  • Changes to the position of the eye in the eye socket.
  • Changes to the size or shape of the pupil.
  • Dark spot on the iris.
  • Displaced eye lens (specific to melanoma in the ciliary body).
  • Double vision.
  • Eye pain.
  • Floaters (spots) or flashes of light in your field of vision.
  • Retinal detachment (specific to melanoma in the choroid).

Diagnosis and Tests

How is intraocular melanoma diagnosed?

Your healthcare provider evaluates your symptoms and reviews your medical history. During an eye exam, your provider checks your vision and looks at the inside of your eye. They may dilate (enlarge) your pupils with special eye drops. This helps your provider see structures at the back of your eye.

Other tests may include:

  • Angiography to check for blocked or leaking blood vessels in the choroid.
  • Ultrasound to measure the size of the structures inside the eye and check for tumors.

If your provider diagnoses you with eye melanoma, they may do more imaging or blood tests. These tests help your provider stage the cancer. Staging is the process of finding out if the cancer has spread from your eye to other areas of your body.

Management and Treatment

How is intraocular melanoma treated?

If an eye tumor isn’t causing symptoms or vision loss, and is small in size (less than 2.5 mm in thickness) you may not need treatment right away. Your healthcare provider may suggest “watchful waiting” and monitor your condition for tumor growth prior to recommending treatment.

If you do need treatment, the most common treatments are radiation therapy or surgery. Your treatment plan depends on:

  • Location of the tumor.
  • Size and thickness of the tumor.
  • Whether the cancer has spread to other areas of the body.
  • Your age and health.

How does surgery treat intraocular melanoma?

There are several types of surgery for intraocular melanoma:

  • External resection to remove the tumor and some of the surrounding healthy tissue from outside-in.
  • Enucleation to remove the eye and part of the optic nerve.

Some degree of vision loss is a risk with each type of surgery. You may choose to have a prosthetic (artificial) eye after enucleation or exenteration, but this won’t restore vision in that eye.

How does radiation therapy treat intraocular melanoma?

  • Radiation therapy uses powerful doses of radiation to destroy tumors. Depending on the size and location of your tumor, your healthcare provider may recommend:
  • External radiation therapy, which points beams of radiation at the tumor from outside the body. Special techniques such as proton beam radiation target the tumor. This minimizes damage to nearby eye and brain tissues.
  • Internal radiation therapy, which implants seeds of radiation inside the eye, near the tumor. Providers call this technique radioactive plaque therapy. The seeds are inside a metal plaque (disk) to protect other parts of the eye.

Are there other treatments for intraocular melanoma?

Other treatments for intraocular melanoma include:

  • Transpupillary thermal therapy (TTT), which uses heat from a laser to destroy the tumor.
  • Endoresection, wherein the tumor is removed from within the eye.

Prevention

How can I prevent intraocular melanoma?

Most risk factors for intraocular melanoma, such as age and race, aren’t controllable. But you can reduce your risk by visiting an ophthalmologist (a healthcare provider specializing in eye care) for regular eye exams.

Outlook / Prognosis

What is the outlook for people with intraocular melanoma?

Research shows that intraocular melanoma spreads to other parts of the body in about 40% to 50% of cases. In about 90% of those cases, the cancer spreads to the liver. Choroid melanoma and ciliary body melanoma are more likely to spread than iris melanoma. Like all cancers, the outlook is better when healthcare providers catch and treat the tumor early.

Living With

What should I do if I have intraocular melanoma?

Stay in communication with your healthcare provider and monitor the intraocular melanoma closely. You may need periodic liver imaging exams to find out if the cancer has spread.

What should I ask my healthcare provider?

If you have intraocular melanoma, you may want to ask your provider:

  • What is the size of the tumor?
  • What treatments are right for me?
  • Will treatment affect my vision?
  • Are there clinical trials I can take part in?
  • Can the cancer come back after treatment?

A note from Cleveland Clinic

Intraocular melanoma is cancer inside the eye. It affects cells in the uvea, or the middle part of the eye. Symptoms can include vision loss or changes to the shape and appearance of the eye. Intraocular melanoma can spread to other parts of the body such as the liver. The most common treatments are radiation therapy and surgery.

Last reviewed by a Cleveland Clinic medical professional on 04/23/2021.

References

  • American Cancer Society. Can Eye Cancer Be Prevented? Damtao, BE and Singh AD. Uveal tumors. Clinical Ophthalmic Oncology. Springer- nature, New York, NY 2019. Accessed 4/18/2021.
  • National Cancer Institute. . Accessed 4/18/2021. Intraocular (Eye) Melanoma (https://www.cancer.gov/types/eye)
  • National Organization for Rare Disorders (NORD). . Accessed 4/18/2021. Ocular Melanoma (https://rarediseases.org/rare-diseases/ocular-melanoma/)
  • Aronow ME, Topham AK, Singh AD. Uveal Melanoma: 5-Year Update on Incidence, Treatment, and Survival (SEER 1973-2013). Ocul Oncol Pathol. 2018 Apr;4(3):145-151. Accessed 4/18/2021.

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