Ocular melanoma, or melanoma of the eye, is the most common primary eye tumor in adults and the 2nd most common melanoma (after cutaneous melanoma) with around 2,000 new cases diagnosed each year in the United States. Like other melanomas, it begins in melanocytes – the cells that produce the pigment melanin that colors the skin, hair, and eyes, as well as forms moles. If you have ocular melanoma it is important to know your diagnosis and treatment options. We have provided resources for people diagnosed with ocular melanoma.
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Types of Ocular Melanoma
Different types of melanoma of the eye include:
The uvea is three-layered part of the eye. It is made up of the choroid, iris and ciliary body. Uveal melanoma can form in any of these layers and is named for where it forms:
- Choroidal melanoma begins in the layer of blood vessels – the choroid – beneath the retina. It is the most common type of uveal melanoma. A 2012 article by the American Academy of Ophthalmology discusses the differences between choroidal nevi and choroidal melanoma.
- Iris melanoma occurs in the front, colored part of the eye. Iris melanomas usually grow slowly and do not typically metastasize, or spread, to other parts of the body outside the eye.
- Ciliary melanoma occurs in the back part of the eye – in the ciliary body. Melanomas in the ciliary body tend to grow and metastasize to the liver more quickly than iris melanomas.
The conjunctiva is the clear tissue that covers the white part of the eye, as well as the inside of the eyelids. Conjunctival melanoma is very rare. It often appears as a raised tumor and may contain little or even no pigment. Conjunctival melanoma most commonly occurs in the bulbar conjunctiva – the mucous membrane that covers the outer surface of the eyeball. Unlike other forms of ocular melanoma that spread most often to the liver, when conjunctival melanoma spreads, it most often spreads to the lungs.
Frequency, Incidence & Risk Factors
Melanoma of the skin increased in frequency over the last several decades, while such a trend is less evident with ocular melanoma. About 6 people per 1 million are diagnosed with eye melanoma in the U.S. every year, while invasive melanoma of the skin occurs in approximately 1 in 50 Americans each year. The incidence is similar in other Caucasian populations worldwide. According to a 2017 article published in Clinical Ophthalmology, melanoma of the eye accounts for approximately 3-5% of all melanomas.
A variety of risk factors have been identified, including light eyes, fair skin type, dysplastic nevus syndrome and the BAP1 mutation. The role of sun exposure as a risk factor for ocular melanoma remains unclear.
Melanoma of the eye tends to occur slightly more often in males than in females and overall risk tends to increase with age.