Causes and Risk Factors of Ocular Melanoma

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Ocular (eye) melanoma is a rare cancer that occurs when the cells that produce pigment (color) in the eyes divide and multiply too rapidly. This produces a lump of tissue known as a tumor.

It's not clear exactly why ocular melanoma occurs, but some factors may increase the risk of it happening, such as age, race, and family history. This article discusses the causes and risk factors that could increase your chance of developing ocular melanoma.

Close up of young woman's blue eyes, freckles


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Types of Ocular Melanoma

Eye melanoma most commonly develops in the cells of the middle layer of the eye (uvea). This is referred to as uveal melanoma. Ocular melanoma can also occur on the outermost layer on the front of the eye (conjunctiva), in the socket that surrounds the eyeball, and on the eyelid, though these types of eye melanoma are very rare.

Common Causes

A risk factor is anything that increases the chance of developing cancer. It's worth noting, however, that some people with several risk factors never develop cancer, while others with no known risk factors do. 

The following factors may raise a person’s risk of developing eye melanoma:

  • Age: Eye melanomas can occur at any age, but the risk goes up as people get older. The incidence of ocular melanoma appears to increase with age, with a peak in people ages 60 to 70.
  • Race: Ocular melanoma is more common in White people. Rates of this type of cancer are eight to 10 times higher among White people compared with Black people.
  • Eye color: People with light-colored eyes are somewhat more likely to develop uveal melanoma of the eye than are people with darker eye and skin color.
  • Gender: Ocular melanoma affects slightly more men than women.
  • Moles: Different types of moles (nevi) in the eye or on the skin have been associated with an increased risk of uveal eye melanoma. Nevus or nevi are growths or marks on tissue such as the skin that are usually discolored and sometimes raised. They are sometimes described as an “eye freckle.” People with atypical (unusual looking) moles are up to 10 times more likely to develop ocular melanoma than the average population.

Cancer of the eye is fairly rare. The American Cancer Society estimates that 3,320 new cancers of the eye and orbit (the bony socket of the eye) will be diagnosed in the United States in 2021.

Genetics

People with certain genetic (inherited) conditions appear to have a higher risk of developing ocular melanoma:

  • People with abnormal brown spots on the middle layer of the eye (known as oculodermal melanocytosis or nevus of Ota) have an increased risk of developing eye melanoma. The estimate of lifetime risk for a White patient with oculodermal melanocytosis to develop eye melanoma is 1 in 400.
  • People with dysplastic nevus syndrome, who have many abnormal moles on the skin, are at increased risk of skin melanoma. They also seem to have a higher risk of developing melanoma of the eye.
  • Ocular melanoma can run in families, although it is uncommon. BAP1 cancer syndrome is a rare inherited condition in which family members are at increased risk for eye melanoma, as well as melanoma of the skin, malignant mesotheliomakidney cancer, and others. This condition is caused by an inherited mutation (change) in the BAP1 gene.
  • An eye condition, known as primary acquired melanosis (PAM), where the melanocytes in the eye grow too much, is a risk factor for conjunctival melanoma. About 60% of conjunctival melanomas arise from PAM.

People with a combination of these risk factors may benefit from seeing an ophthalmologist (specialist eye doctor) for a yearly examination. Anyone who finds unusual moles or other skin growths around the eye or elsewhere on the body should see a dermatologist, a doctor specializing in skin diseases. 

Lifestyle Risk Factors

Melanoma of the skin has been linked to exposure to ultraviolet (UV) rays from the sun (or tanning beds). Some researchers have proposed UV rays as a possible risk factor for uveal or conjunctival melanoma of the eye, but studies so far have shown mixed results. More research is needed in this area.

Some patients with uveal eye melanoma have a history of melanoma of the skin, but it is still not known if having skin melanoma increases your risk of eye melanoma.

Summary

Ocular melanoma is rare, and scientists are still investigating the causes of the cancer. Researchers do know that age, race, eye color, and certain inherited conditions may contribute to your risk of developing eye cancer. Regular eye checkups are advised to spot any potential cancer lesions early.

A Word From Verywell

If you fall into several of the risk categories for eye melanoma, schedule regular checkups with a specialist eye doctor to put your mind at ease. Rest assured that eye melanoma is fairly rare and any issues you have with your eyes will likely be down to noncancerous causes.

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4 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read our editorial process to learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
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