Uveal Melanoma

Uveal melanoma, sometimes called ocular melanoma, is a type of cancer that affects the uvea, the middle layer of the eye that contains blood vessels, pigment cells (melanocytes), and provides nutrients and oxygen to the retina. It is a rare form of melanoma derived from the melanocytes in the uvea, accounting for only about 5% of all cases of melanoma in the United States.

Uveal melanoma can develop in different parts of the uvea, including the iris, ciliary body, and choroid. Choroidal melanoma is the most common site (90% of all uveal melanomas) followed by ciliary body (6%) and iris (4%) melanomas.

The rate new cases of uveal melanomas have remained fairly consistent over the last three decades across the world.

What are the signs and symptoms of uveal melanoma?

Uveal melanoma may not cause any symptoms in its early stages, and it may be detected during a routine eye exam with pupil dilation, which is the best way to screen for uveal melanoma. However, as the tumor grows, it can cause a variety of symptoms, including:

• Blurred vision or loss of vision in one eye
• Dark spots on the iris (the colored part of the eye) - A change in the shape or size of the pupil
• A bulging of the eye
• Flashes of light or floating spots in the vision
• Pain or redness in the eye

It's important to note that these symptoms can also be caused by other eye conditions, so it's important to see an eye doctor if you experience any changes in your vision or eye health. If uveal melanoma is suspected, your doctor may perform additional tests, such as an ultrasound or biopsy, to confirm the diagnosis. Early detection and treatment of uveal melanoma can improve the chances of a successful outcome.

How common is uveal melanoma?

Uveal melanoma is a rare form of melanoma, accounting for only about 5% of all cases. About -5000 cases of uveal melanoma are diagnosed each year in the United States.  

How serious is uveal melanoma?

Uveal melanoma is a serious condition that requires prompt medical attention. While it is a rare form of melanoma, if undiagnosed, it can be aggressive and can spread to other parts of the body, such as the liver (most common organ for uveal melanoma to spread to), lungs, brain, kidney, and bones. In fact, up to 50% of patients with uveal melanoma will develop metastatic disease, which can be life-threatening.

The prognosis for uveal melanoma depends on a number of factors, including the size and location of the tumor, the extent of its spread, and the patient's overall health. In general, smaller tumors that have not spread beyond the eye have a better prognosis than larger tumors or those that have spread to other parts of the body.

Who gets uveal melanoma?

Uveal melanoma is a rare type of cancer that can affect anyone, but it is more common in certain groups of people. Some of the factors that may increase the risk of developing uveal melanoma include:

• Gender: Men have a slightly higher chance of getting uveal melanoma than women.
• Age: Uveal melanoma is most commonly diagnosed in people over the age of 50 and is rarely found in children.
• Race: The risk of uveal melanoma is lowest in Blacks followed by increased rates in Asians, then Hispanics, then non-Hispanics, respectively.
• Physical features: Uveal melanoma is more common in people with light-colored eyes, such as blue or green eyes, and fair skin.
• Genetics: Some inherited genetic mutations have been linked to an increased risk of uveal melanoma which can be diagnosed at a younger age, including mutations in the BAP1 gene.
• Exposure to ultraviolet (UV) radiation: While UV radiation from the sun is a known risk factor for skin cancer, it is not clear if it plays a role in the development of uveal melanoma.

It's important to note that many people who develop uveal melanoma do not have any known risk factors, and the exact cause of the disease is not fully understood. If you are concerned about your risk of developing uveal melanoma, talk to your healthcare provider. They can help you understand your risk factors and recommend appropriate screening tests or preventive measures.

What causes uveal melanoma?

The exact cause of uveal melanoma is not fully understood, but it is believed to be related to mutations that occur in the pigment cells of the eye. These mutations can cause the cells to grow and divide uncontrollably, leading to the development of a uveal tumor. The mutations found in uveal melanoma are distinct from those found in other melanoma subtypes.

How is uveal melanoma treated?

The treatment of uveal melanoma depends on several factors, including the size and location of the tumor, as well as the patient's overall health. Treatment options may include:

• Radiation therapy: This is the most common treatment for uveal melanoma. Radiation therapy uses different types of high-energy beams of radiation to destroy cancer cells. It can be delivered externally (external beam radiation therapy) or internally (brachytherapy).
• Surgery: Surgery may be an option for small tumors that have not spread beyond the eye. The goal of surgery is to remove the tumor and a small amount of surrounding tissue.
• Laser therapy: Laser therapy uses a high-energy beam of light to destroy cancer cells. It may be used to treat small tumors that are located in the front of the eye.
• Chemotherapy: Chemotherapy is not commonly used to treat uveal melanoma, but it may be an option for advanced cases that have spread to other parts of the body.
• Immunotherapy: Immunotherapy is a type of treatment that uses the body's immune system to fight cancer. It may be an option for advanced cases of uveal melanoma.

In 2022, a new type of Immunotherapy called, Tebentafusp (Kimmtrak), was approved by the FDA to treat uveal melanoma that cannot be removed or has spread to other parts of the body. It works by bridging together tumor and immune T cells, which helps the body’s immune system to locate and kill the uveal melanoma cells. Learn more about Tebentefusp and who is eligible to receive it.

Your healthcare provider will work with you to determine the best treatment plan for your individual needs. It's important to discuss the potential benefits and risks of each treatment option, as well as any potential side effects, with your healthcare provider.

For patients facing rare melanoma subtypes, including those with uveal melanoma, it’s important to see a doctor who specializes in treating patients with your specific type of melanoma. Learn more about choosing your doctor and getting a second opinion for uveal melanoma.