Eye Melanoma: Symptoms, Treatment & Prognosis

What Is Eye Melanoma?

Eye melanoma (ocular melanoma) is a cancer that develops in the melanocytes—the pigment-producing cells—of the eye. The most common form, uveal melanoma, affects the choroid, ciliary body, or iris. It is the most common primary intraocular cancer in adults, with an incidence of approximately 5-7 cases per million people annually.

Eye melanoma occurs when the melanocytes within the eye begin to grow uncontrollably and form a tumor. These are the same type of cells that can cause skin melanoma, but the behavior and causes of eye melanoma are distinctly different from its cutaneous counterpart. The uveal tract, which includes the iris (the colored part of the eye), ciliary body (the ring of muscle behind the iris), and choroid (the layer of blood vessels behind the retina), contains most of the melanocytes in the eye and is where the vast majority of ocular melanomas develop.

Understanding the location of the tumor within the eye is crucial because it affects both treatment options and prognosis. Choroidal melanomas, which occur in the back of the eye, are the most common type, accounting for approximately 85-90% of all uveal melanomas. Ciliary body melanomas make up about 5-8% of cases, while iris melanomas, which are visible as dark spots on the colored part of the eye, represent only 3-5% of cases and generally have the best prognosis.

The disease primarily affects adults, with the average age at diagnosis being around 55-60 years. It is extremely rare in children and young adults. While eye melanoma can affect anyone, certain populations have higher incidence rates, particularly those of Northern European descent. The disease occurs with roughly equal frequency in men and women, though some studies suggest a slight male predominance.

How Does Eye Melanoma Differ from Skin Melanoma?

One of the most important distinctions to understand is that eye melanoma is NOT caused by sun exposure or ultraviolet (UV) radiation. This stands in stark contrast to skin melanoma, where sun exposure is a well-established risk factor. Extensive research has failed to establish any causal link between UV exposure and the development of uveal melanoma. This means that wearing sunglasses, while beneficial for overall eye health and preventing other conditions, does not specifically protect against eye melanoma.

The molecular and genetic characteristics of eye melanoma also differ significantly from skin melanoma. Uveal melanoma is characterized by specific genetic mutations, particularly in the genes GNAQ, GNA11, BAP1, SF3B1, and EIF1AX. The BAP1 mutation is particularly important because it is associated with more aggressive disease and higher risk of metastasis. These genetic differences mean that treatments effective for skin melanoma may not work the same way for eye melanoma.

Rare but Serious Condition

While eye melanoma is rare—representing only about 5% of all melanomas—it is the most common primary cancer originating within the eye in adults. In the United States, approximately 2,000-2,500 new cases are diagnosed each year. Despite successful treatment of the primary tumor, approximately half of all patients will eventually develop metastatic disease, most commonly to the liver. This is why ongoing surveillance is so critical, even years after initial treatment.

What Are the Symptoms of Eye Melanoma?

The symptoms of eye melanoma can be subtle and many people have no symptoms at all. When symptoms occur, they may include seeing light flashes in peripheral vision, noticing a dark shadow or curtain in the visual field, blurred or distorted vision where straight lines appear bent, and a growing dark spot on the iris. Eye melanoma typically does not cause pain.

Many cases of eye melanoma are discovered incidentally during routine comprehensive eye examinations. Because the tumor often develops in the back of the eye where it cannot be seen without specialized equipment, patients may be completely unaware of its presence until an ophthalmologist detects it. This underscores the importance of regular eye examinations, particularly for those over 50 years of age.

When symptoms do develop, they typically depend on the location and size of the tumor. Melanomas in the choroid, located at the back of the eye, tend to affect vision as they grow and encroach upon the macula (the central part of the retina responsible for sharp vision) or cause retinal detachment. Iris melanomas may be visible as a dark spot that grows over time, though many iris nevi (freckles) are benign and do not progress to cancer.

Visual Symptoms

The most common visual symptoms associated with eye melanoma include:

• Light flashes (photopsia): Seeing flashes of light, particularly in the peripheral vision, can indicate that the tumor is affecting the retina. These flashes may appear as brief sparks or lightning-like streaks and are often more noticeable in dim lighting conditions.
• Visual field defects: A dark shadow, curtain, or area of missing vision may develop as the tumor grows or causes retinal detachment. This shadow typically starts in the peripheral vision and may gradually expand.
• Blurred vision: As the tumor grows, it can push on or distort the retina, causing blurry or distorted vision. Straight lines may appear wavy or bent (metamorphopsia).
• Floaters: While floaters are common and usually harmless, a sudden increase in floaters, especially when accompanied by light flashes, should be evaluated promptly.

Visible Changes to the Eye

Some eye melanomas, particularly those arising in the iris, may be visible to the naked eye. Signs that warrant evaluation include:

• Growing dark spot on the iris: A new brown or black spot on the colored part of the eye that changes in size or shape over time
• Change in pupil shape: The tumor may distort the pupil, causing it to appear irregular
• Heterochromia: A change in the color of part of the iris

When Should You See a Doctor for Eye Changes?

Seek prompt eye care if you experience light flashes in your peripheral vision, a growing dark shadow in your visual field, new floaters especially with flashes, blurred or distorted vision, or notice a growing dark spot on your iris. While these symptoms can have many causes, early evaluation is important. Annual comprehensive eye exams are recommended for adults over 50.

Understanding when to seek medical attention for eye symptoms can be crucial for early detection and treatment. While many eye symptoms are caused by benign conditions, certain warning signs warrant prompt evaluation by an ophthalmologist or optometrist.

The urgency of seeking care depends on the specific symptoms you experience. Some symptoms may develop gradually over weeks or months, while others appear suddenly and require more immediate attention. In general, any sudden change in vision, particularly when accompanied by light flashes or a dark curtain-like shadow in your visual field, should be evaluated within 24-48 hours.

Symptoms Requiring Prompt Evaluation

Contact an eye clinic or schedule an appointment with your ophthalmologist as soon as possible if you experience:

• Persistent light flashes in your peripheral vision that don't go away
• A new or growing dark shadow or curtain in any part of your visual field
• Sudden increase in floaters, especially if accompanied by light flashes
• Noticeable distortion where straight lines appear wavy or bent
• A growing dark spot on the colored part of your eye (iris)

The Importance of Routine Eye Examinations

Because many cases of eye melanoma are discovered during routine examinations before symptoms develop, regular comprehensive eye exams are essential. The American Academy of Ophthalmology recommends that adults without eye conditions or risk factors have a baseline comprehensive eye examination at age 40, with follow-up exams as recommended by their eye care provider. After age 65, annual examinations are generally recommended.

During a comprehensive eye examination, your eye doctor will dilate your pupils to examine the interior of your eye, including the retina and choroid where most ocular melanomas develop. This detailed examination cannot be performed without pupil dilation and is the primary way that eye melanomas are detected before they cause symptoms.

How Is Eye Melanoma Diagnosed?

Eye melanoma is diagnosed through a comprehensive dilated eye examination using specialized instruments including ophthalmoscopy, ocular ultrasound to measure tumor dimensions, and sometimes fluorescein angiography or optical coherence tomography (OCT). Unlike many cancers, biopsy is often not necessary as the tumor can usually be diagnosed based on its appearance and imaging characteristics.

The diagnostic process for eye melanoma typically begins when an ophthalmologist notices a suspicious lesion during a routine eye examination or when a patient presents with concerning symptoms. The diagnosis is usually made based on the clinical appearance of the tumor and specialized imaging, without the need for a tissue biopsy. This is different from most other cancers, where biopsy is essential for diagnosis.

When a suspicious lesion is identified, your general ophthalmologist will typically refer you to an ocular oncologist—a specialist in eye cancers—for further evaluation. The referral process and subsequent examination are usually expedited given the serious nature of the condition.

Preparation for the Examination

Before your comprehensive eye examination, there are several things to know and prepare:

• Arrange transportation: Your pupils will be dilated, causing blurry vision and light sensitivity for 4-6 hours. You should not drive after the examination.
• Bring sunglasses: Your eyes will be very sensitive to light after dilation. Sunglasses or a hat with a brim will help.
• Allow sufficient time: A thorough examination may take 2-3 hours including waiting time for dilation to take effect.
• Bring your medical history: Information about any medications you take and your family history of cancer is helpful.

The Examination Process

The diagnostic examination involves several components. First, eye drops are administered to dilate your pupils. This takes about 20-30 minutes to take full effect. The examination itself may feel uncomfortable due to bright lights but is not painful.

Ophthalmoscopy: Using specialized instruments, the doctor examines the interior of your eye, including the retina, choroid, and optic nerve. This allows direct visualization of any tumors in the back of the eye.

Ocular ultrasound (ultrasonography): This imaging technique uses sound waves to measure the size and shape of the tumor. It is particularly useful for determining tumor thickness (height), which is an important factor in treatment planning and prognosis. The ultrasound can also help distinguish melanoma from other types of eye lesions.

Additional imaging: Depending on the findings, additional tests may be performed including fluorescein angiography (using dye to visualize blood vessels), optical coherence tomography (OCT) for detailed cross-sectional imaging, and autofluorescence imaging.

Staging and Metastatic Workup

Once eye melanoma is diagnosed, additional tests are typically performed to determine if the cancer has spread beyond the eye. The liver is the most common site of metastasis, so imaging of the liver is standard:

• Liver ultrasound or MRI: To screen for liver metastases
• Chest X-ray or CT scan: To evaluate the lungs
• Blood tests: Liver function tests may be elevated if metastatic disease is present

The tumor is staged according to the AJCC (American Joint Committee on Cancer) staging system, which considers tumor size, location, and whether there is extraocular extension or metastasis. Staging helps guide treatment decisions and provides prognostic information.

How Is Eye Melanoma Treated?

Eye melanoma is primarily treated with radiation therapy, most commonly using a radioactive plaque (brachytherapy) surgically attached to the eye for several days. This approach successfully treats the tumor while preserving the eye in most cases. Other treatments include external beam radiation, laser therapy, and surgical removal of the eye (enucleation) for very large tumors. Treatment choice depends on tumor size, location, and patient factors.

The treatment of eye melanoma has evolved significantly over the past several decades. The landmark Collaborative Ocular Melanoma Study (COMS), conducted in the 1980s and 1990s, established that radiation therapy provides survival outcomes equivalent to surgical removal of the eye for medium-sized tumors, while allowing many patients to retain their eye and some degree of vision. Today, radiation therapy is the most common treatment approach.

The choice of treatment depends on several factors including the size and location of the tumor, the patient's overall health, the vision in the affected eye and the other eye, and patient preferences. Treatment decisions are typically made collaboratively between the ocular oncologist, radiation oncologist, and patient.

Radiation Therapy with Plaque Brachytherapy

Plaque brachytherapy is the most common treatment for small to medium-sized uveal melanomas. This technique involves surgically placing a small radioactive disc (plaque) on the outside of the eye, directly over the tumor. The plaque delivers concentrated radiation to the tumor while minimizing exposure to surrounding healthy tissues.

The procedure: The surgery to place the plaque is performed under general anesthesia and takes approximately 1-2 hours. The plaque is sutured to the surface of the eye (sclera) in precise alignment with the tumor location. The plaque remains in place for several days (typically 3-7 days depending on the tumor size), during which time the patient stays in the hospital. A second brief surgery removes the plaque when treatment is complete.

During treatment: While the plaque is in place, patients may experience some discomfort or a gritty sensation. They must remain in their hospital room to minimize radiation exposure to others, though they can move around freely within the room. Pain is usually manageable with oral medications.

After treatment: Following plaque removal, patients receive anti-inflammatory eye drops for several weeks. The eye may appear red and feel irritated initially. Vision changes may occur immediately or develop over months to years as the tumor shrinks and radiation effects manifest.

External Beam Radiation

For some tumors, particularly those near the optic nerve or in challenging locations, external beam radiation may be preferred. Proton beam radiation is a specialized form that delivers highly focused radiation with minimal damage to surrounding structures. This treatment is available at specialized centers with proton therapy facilities.

Laser Therapy

Laser treatments may be used as adjunct therapy along with radiation, or for very small tumors. Transpupillary thermotherapy (TTT) uses heat from a laser to destroy tumor cells. Laser treatment alone is generally reserved for small tumors and may be combined with other therapies.

Surgical Removal of the Eye (Enucleation)

While most eye melanomas can be treated with radiation, surgical removal of the eye (enucleation) may be necessary for very large tumors, tumors that have grown extensively, or when the eye has already lost vision. This procedure involves removing the entire eyeball while preserving the eye muscles and surrounding structures.

After enucleation: Patients receive a temporary conformer (plastic placeholder) initially, and are fitted for a custom ocular prosthesis (artificial eye) approximately 4-6 weeks after surgery. Modern prostheses are remarkably realistic and move naturally with the remaining eye muscles. Most patients adapt well and can return to normal activities, though depth perception is affected when one eye is removed.

Recovery and Side Effects

Recovery time varies depending on the treatment received. After plaque brachytherapy, most patients can return to light activities within 2 weeks, though some take longer. After enucleation, physical recovery is typically 2-4 weeks, with prosthesis fitting following several weeks later.

Side effects of radiation treatment may include:

• Dry eye and irritation
• Cataracts (often developing months to years after treatment)
• Radiation retinopathy (damage to the retina)
• Optic neuropathy (damage to the optic nerve)
• Vision loss (varies from mild to severe depending on tumor location)
• Need for additional treatments if complications develop

What Is Life Like with an Eye Prosthesis?

Most people who receive an ocular prosthesis (artificial eye) adapt well and can lead normal lives. Modern prostheses are custom-made to match the other eye and move naturally with the remaining eye muscles. Care involves daily cleaning and periodic professional polishing. Driving with one eye is typically permitted after adaptation, though depth perception is affected.

For patients who require enucleation, receiving an ocular prosthesis is an important part of the recovery process. Understanding what to expect can help reduce anxiety and facilitate adjustment to this significant change.

Approximately 4-6 weeks after surgery, once the socket has healed sufficiently, patients are fitted for a custom prosthesis by an ocularist—a specialist who designs, fabricates, and fits artificial eyes. The fitting process involves taking impressions of the socket and carefully matching the color and appearance of the natural eye. The prosthesis is hand-painted to replicate the iris color, blood vessel patterns, and other details of the patient's natural eye.

Types of Prostheses

Ocular prostheses may be made from acrylic (plastic) or glass, each with advantages:

• Acrylic prostheses: More durable and resistant to breakage. Require professional polishing every 6-12 months. May need replacement every 5-7 years.
• Glass prostheses: Lighter weight and maintain polish longer. More fragile and need replacement every 2-3 years. Not available in all countries.

Daily Life with a Prosthesis

Most patients adapt remarkably well to their prosthesis. The artificial eye moves in coordination with the natural eye due to the preserved eye muscles, providing a natural appearance. Daily care is straightforward, involving removal for cleaning and overnight storage in saline solution.

Activities and lifestyle considerations include:

• Driving: Most patients can drive after adapting to monocular (one-eye) vision. Requirements vary by jurisdiction, and driving assessment may be required.
• Sports and activities: Most activities can be continued with appropriate eye protection for the remaining natural eye.
• Swimming: Swimming is generally safe, though some prefer to use goggles to prevent the prosthesis from dislodging.
• Work: Most occupations can be continued, with accommodations as needed for jobs requiring depth perception.

Why Is Lifelong Monitoring Essential?

Lifelong follow-up monitoring is essential because eye melanoma can spread (metastasize) to other parts of the body, particularly the liver, even years after successful treatment of the primary tumor. Approximately 50% of patients eventually develop metastatic disease. Regular liver imaging and clinical examinations allow early detection of metastases when treatment options may be more effective.

Unlike many cancers where the risk of recurrence diminishes over time, the metastatic risk from eye melanoma persists for many years—even decades—after treatment. This unique characteristic of uveal melanoma makes ongoing surveillance critically important throughout the patient's lifetime.

The liver is by far the most common site of metastasis, accounting for approximately 90% of metastatic cases. This hepatotropism (tendency to spread to the liver) is a distinctive feature of uveal melanoma. Other potential sites of spread include the lungs, bones, and skin, though these are less common.

Monitoring Schedule

A typical surveillance schedule includes:

• Eye examinations: Regular examinations of the treated eye to monitor for local tumor control and treatment complications
• Liver imaging: Ultrasound, CT, or MRI of the liver every 6-12 months, depending on risk factors
• Blood tests: Periodic liver function tests
• Physical examination: Regular assessment by your oncology team

The frequency and type of monitoring may be adjusted based on genetic testing results. Tumors with certain high-risk features, such as BAP1 mutations or monosomy 3, may warrant more intensive surveillance.

What Happens If Eye Melanoma Spreads?

If eye melanoma spreads (metastasizes), it most commonly affects the liver. While metastatic uveal melanoma cannot currently be cured, treatments can slow progression and extend life. Options include liver-directed therapies (hepatic artery infusion, embolization), surgical resection of isolated metastases, immunotherapy, and targeted therapies. Clinical trials are actively investigating new treatment approaches.

When eye melanoma metastasizes, treatment goals shift toward controlling the disease, maintaining quality of life, and extending survival. While the prognosis for metastatic uveal melanoma is generally poor compared to the primary disease, significant advances in treatment options offer hope for improved outcomes.

Treatment approaches for metastatic disease may include:

• Surgical resection: If metastases are limited and accessible, surgical removal may be considered
• Liver-directed therapies: Hepatic artery infusion chemotherapy, chemoembolization, or radioembolization can deliver treatment directly to liver tumors
• Immunotherapy: Checkpoint inhibitors and other immunotherapies are being studied, though response rates differ from skin melanoma
• Targeted therapy: Drugs targeting specific molecular pathways are under investigation
• Clinical trials: Many patients benefit from participation in clinical trials testing new treatments

When curative treatment is not possible, palliative care focuses on symptom management, pain control, and maintaining quality of life. Palliative care can be provided alongside other treatments and is tailored to individual patient needs and preferences.

How Can You Cope with a Cancer Diagnosis?

Receiving a cancer diagnosis is emotionally challenging, and feelings of shock, fear, anxiety, and sadness are normal. Support resources include your medical team, hospital social workers, counselors, cancer support groups, and organizations like the Ocular Melanoma Foundation. Many people find that understanding their condition, connecting with others, and maintaining normal activities helps in coping.

Being diagnosed with eye melanoma brings many emotions and concerns. The rarity of the disease can make it feel isolating—most people have never heard of ocular melanoma. Understanding what you're facing and connecting with resources can help you navigate this challenging time.

Common reactions to diagnosis include shock, disbelief, fear about treatment and prognosis, concerns about vision loss, anxiety about the future, and grief for the life you expected. All of these feelings are valid and normal responses to a serious diagnosis.

Sources of Support

Multiple resources are available to help patients and families cope:

• Your medical team: Doctors, nurses, and social workers can address your questions and concerns
• Hospital support services: Many cancer centers offer counseling, support groups, and patient navigation services
• Patient organizations: The Ocular Melanoma Foundation and similar organizations provide education, support communities, and resources
• Online communities: Connecting with others who have eye melanoma can reduce isolation and provide practical insights
• Mental health professionals: Psychologists and counselors specializing in cancer can help develop coping strategies

Communicating with Family

Sharing your diagnosis with family members, including children, requires thoughtful consideration. Being honest and age-appropriate in your explanations helps maintain trust and allows family members to support each other. Children, in particular, benefit from honest information shared in terms they can understand, and from reassurance that their needs will continue to be met.

Many people find that involving loved ones in medical appointments, discussing concerns openly, and allowing others to help provides comfort during a difficult time.