Uveal Melanoma: Causes, Symptoms, and Treatment Options

Uveal melanoma is a rare but serious form of eye cancer that develops in the uvea, the middle layer of the eye. This condition can lead to various symptoms, including blurry vision, floaters, and in some cases, vision loss. Early diagnosis and treatment are crucial for a better prognosis. This guide explores the causes, symptoms, treatment options, and overall outlook for individuals with uveal melanoma.

What is Uveal Melanoma?

Uveal melanoma is the most common type of primary eye cancer in adults, originating in the eye rather than spreading from other areas. Although it’s rare, with about 1 in 200,000 adults diagnosed annually, it’s the most prevalent melanoma of the eye.

The uvea consists of three parts:

• Ciliary body: A ring of muscle that adjusts the lens for focusing.

• Choroid: A layer rich in blood vessels that nourishes the eye.

• Iris: The colored part of the eye.

Approximately 90% of cases start in the choroid, while the remaining occur in the iris or ciliary body.

Symptoms of Uveal Melanoma

In its early stages, uveal melanoma may not present noticeable symptoms. However, as it progresses, common symptoms include:

• Blurry or double vision

• Vision distortion or loss

• Floaters (small spots in your vision)

• Flashing lights

• Eye irritation or pain

• Redness or bulging of the eye

• Changes in pupil shape

• Dark or growing spot on the iris

When to Seek Medical Attention

It is essential to consult an eye doctor if you experience any changes in vision or symptoms such as:

• Persistent blurry vision

• Distorted vision

• Eye pain or discomfort

Early detection can significantly improve outcomes.

Causes and Risk Factors

The exact causes of uveal melanoma are not fully understood, but a combination of genetic and environmental factors is believed to contribute. Identified risk factors include:

• Light-colored eyes and fair skin

• Dysplastic nevus syndrome (atypical mole syndrome)

• Atypical moles or eye freckles

• Family history of BAP1 cancer predisposition syndrome

• Age (most cases occur in individuals aged 60 and older)

While ultraviolet (UV) light exposure is a known risk factor for skin melanoma, its role in uveal melanoma remains unclear.

Who is Most Affected?

• Uveal melanoma predominantly affects Caucasian individuals, with nearly 98% of cases diagnosed in this group. It is 8 to 10 times more common in Caucasians than in people of African descent.

Treatment Options for Uveal Melanoma

Treatment depends on the tumor's size, location, and whether the cancer has spread. Common options include:

1. Radiation Therapy

• Plaque brachytherapy: A small disc containing radioactive material is placed near the tumor.

• External beam radiation: Focused radiation targets the tumor from outside the body.

2. Laser Therapies

• Transpupillary thermotherapy: A laser heats and destroys cancer cells.

• Laser photocoagulation: Destroys blood vessels feeding the tumor.

3. Surgery

• Removal of the tumor or, in severe cases, the entire eye (enucleation) to prevent further spread.

4. Immunotherapy

• Drugs such as tebentafusp-tebn or darovasertib are used for advanced or metastatic melanoma.

5. Conservative Management

• In select cases, doctors may opt to monitor the cancer closely without immediate treatment.

Is Uveal Melanoma Curable?

While no definitive cure exists, many patients achieve remission with early treatment. Remission means the disease is no longer active and symptoms have subsided. Long-term outcomes depend on factors like tumor size, location, and whether the cancer has spread.

Prevention and Eye Health

Though preventing uveal melanoma isn’t always possible, certain measures can support overall eye health:

• Wear UV-protective sunglasses outdoors.

• Schedule regular eye exams to catch potential issues early.

Prognosis and Survival Rates

Uveal melanoma has the best prognosis when detected early. Survival rates vary based on the stage at diagnosis:

Metastatic Spread

Approximately 50% of uveal melanoma cases metastasize, commonly to the liver. This significantly worsens the outlook.

Tumors in the Iris

Melanoma in the iris is often detected early and rarely spreads, resulting in a better prognosis.

Takeaway

Uveal melanoma is a rare but serious form of cancer that requires prompt diagnosis and treatment. Regular eye exams can help detect changes early, improving treatment outcomes. If you’re experiencing symptoms like blurry vision or dark spots on your iris, seek medical advice promptly.

By staying informed and proactive, you can better manage your eye health and overall well-being.