Eye Cancer

What is eye cancer?

Eye cancer includes several rare types of cancers that begin in your eye, including your eyeball and the structures surrounding your eyeball. Eye cancer starts when cells multiply out of control and form a tumor. Tumors can be benign (noncancerous) or malignant (cancerous). Unlike benign tumors, malignant tumors can grow and the cancer can spread throughout your body.

Diagnosing and treating eye cancers early can often prevent the spread.

What are the types of eye cancer?

Healthcare providers categorize eye cancers based on where cancer starts, its location in your eye and the types of cells.

Intraocular melanomas

Intraocular melanoma arises from cells called melanocytes, the same type of cell involved in the most serious form of skin cancer (melanoma). Most eye cancers are melanomas. Most form in the middle part of your eye (uvea). They’re called uveal melanomas. They include:

• Iris melanoma: Forms in the colored part of your eye, or iris. It often produces a dark, growing spot that stands out against your iris. They tend to grow slowly.
• Ciliary body melanoma: Forms in the muscles that adjust your eyeball lens so you can see objects near and far. The ciliary body is behind your iris.
• Choroidal melanoma: Forms in the layer of your eyeball that keeps your retina (in the back of your eye) and the front of your eye nourished with blood. The choroid is the most common site for eye melanoma to form.

Melanomas sometimes form in the conjunctiva, the membrane that covers the front part of your eyeball. They’re called conjunctival melanomas. They’re incredibly rare. Like uveal melanomas, they tend to spread and are aggressive.

Eyelid and orbital cancer

Orbital and adnexal cancer form in the tissues close to your eyeball. Orbital cancers form in your orbit, or the tissues, muscles and nerves that move your eyeball. Adnexal cancer forms in supporting tissues, including your eyelids and tear glands. Healthcare providers classify them according to the type of cell that transforms into cancer.

Most are:

• Squamous cell carcinoma: Forms from the squamous cells in your top layer of skin.
• Basal cell carcinoma: Forms from the basal cells in your top layer of skin.
• Rhabdomyosarcoma: Forms inside muscle tissue.

Retinoblastoma

Retinoblastoma is a malignant tumor arising from the retina in the back of your eye. They’re most common in children under age five.

Intraocular lymphoma

Intraocular lymphoma is a rare form of B-cell lymphoma. It forms in white blood cells called lymphocytes. It’s most common in people who are older than 50 or who have weakened immune systems. Many people with this form of eye cancer also have primary central nervous system lymphoma (PCNSL). PCNSL is a cancer that may affect various parts of your central nervous system, including your brain, spinal cord and spinal fluid.

How common is eye cancer?

Eye cancer is extremely rare. Only about 3,400 people in the United States receive an eye cancer diagnosis each year. It’s more common for cancers to start in other parts of your body and spread to your eye. Because they don’t start in your eye, providers don’t consider these cancers eye cancer.

What is the most common form of eye cancer?

Intraocular melanomas are by far the most common form of eye cancer. Most start in the middle part of your eye in a structure called the choroid. Approximately 2,500 people in the United States receive this diagnosis each year.

What are the symptoms of eye cancer?

Many people with eye cancer don’t experience symptoms unless a tumor is growing in a location that interferes with how their eye works. Experiencing symptoms doesn’t mean you have eye cancer. Many benign (noncancerous) eye conditions share symptoms with eye cancer. See a healthcare provider to know for sure.

The most common symptom of eye cancer is painless vision loss. Other vision problems that may be signs of eye cancer include:

• Blurry vision.
• Vision loss (either partial or total).
• Seeing flashes of light, squiggly lines or spots (floaters).

Other signs and symptoms include:

• A bulging eye.
• Eye irritation that doesn’t improve.
• A dark spot in your iris that gets bigger.
• A growing lump on your eyelid or in your eyeball.
• Changes in your eyeball’s positioning in the socket and how it moves.

What are the first signs of eye cancer?

Most people don’t learn they have eye cancer until a healthcare provider, like an optometrist or ophthalmologist, notices something suspicious during an eye exam. For example, enlarged blood vessels in your eye or a dark spot may signal eye cancer or another eye condition. You’ll need tests to be sure.

What causes eye cancer?

As with cancers in general, eye cancer occurs when cells begin to divide and multiply out of control, eventually forming a mass called a tumor. Pieces of the tumor can break off and spread to your lymph nodes and bloodstream. The cancer cells can travel to other parts of your body via your bloodstream and lymphatic system, causing new tumors to form in other organs. When this happens, healthcare providers say that your cancer has “spread” or “metastasized.” It’s a sign of a more advanced disease.

Scientists are still researching to understand what causes otherwise healthy cells to become cancer cells.

What are the risk factors for eye cancer?

Researchers have identified several risk factors that may increase your likelihood of developing eye cancer:

• Age: Providers diagnose most eye cancers in people over 50. The exception is retinoblastoma, which affects children under age 5.
• Skin color: You’re more likely to get eye cancer if you’re white and if you have pale skin.
• Eye color: People with light eyes (blue, green) are more likely to get eye cancer than people with dark eyes (brown).
• Inherited conditions: Dysplastic nevus syndrome, an inherited condition that involves having several atypical-looking moles, can increase your risk of some eye cancers. BAP1 tumor predisposition syndrome can increase your risk of multiple cancers, including uveal melanoma.
• Sunning and tanning: It’s possible that exposure to UV rays from the sun or tanning beds can increase your risk of intraocular melanoma. Medical researchers need to conduct more research to know for sure.

How is eye cancer diagnosed?

An eye disease specialist (ophthalmologist) or an ocular oncologist diagnoses eye cancer. They may perform a variety of procedures to rule out other, more common eye conditions before arriving at a cancer diagnosis.

Eye exam

During an eye exam, a healthcare provider examines your eye closely, looking for signs of cancer. They may look for dark spots and enlarged blood vessels. They may check to see if your eyeball is moving as it should. They may use special tools to see the structures in your eye more clearly.

• Ophthalmoscope: An ophthalmoscope is a handheld instrument that contains a light and multiple lenses. It allows healthcare providers to view structures in the back of your eyeball, like your retina.
• Slit lamp: A slit lamp is a device that sits on a platform and table. It uses a light source and special lenses, like a microscope, to see detailed views of the front and back of your eyeball.

Imaging

The results of imaging procedures and the information from your eye exam are often enough to diagnose eye cancer. Common imaging procedures include:

• Ultrasound: Ultrasounds use sound waves to create images of the inside of your eyeball. Ultrasounds show how big a tumor is and its location. They’re especially useful in diagnosing intraocular melanomas.
• Fluorescein angiography: During this procedure, a healthcare provider injects dye into your bloodstream that makes your blood vessels show up more clearly during imaging. Once the dye has had time to reach the blood vessels in your eyeball, your provider uses a special camera that shows how blood is flowing in your eyeball.

You may need additional imaging procedures if your provider suspects the cancer’s spread. Imaging procedures that can show if cancer’s spread to parts outside your eye include:

• Ultrasound.
• Chest X-ray.
• CT scan.
• MRI.
• PET scan.

Biopsy

During a biopsy, a healthcare provider removes a sample of tissue from the tumor and tests it for cancer cells. Healthcare providers can identify most eye cancers with a physical exam and imaging. Still, a biopsy can provide information about the makeup of your cancer cells, including genetic mutations (changes) that make them unique. Your provider can use this information to determine characteristics about your cancer, like how aggressive it is. It can also show if you’re eligible for certain treatments.

• Fine needle aspiration biopsy: A tiny needle removes a sample of fluid from your eye to test for cancer cells.
• Incisional biopsy: A provider removes part of the tumor and tests the tissue for cancer cells.
• Excisional biopsy: A provider removes the entire tumor and tests the tissue for cancer cells.

How is eye cancer staged?

Cancer staging helps providers determine how advanced cancer is. They use this information to plan treatments and gauge your prognosis, or the likely outcome of your condition.

There are two common staging systems for eye cancer:

American Joint Committee on Cancer (AJCC) TNM staging system

Providers stage cancer by assessing various factors.

• T: The tumor’s size and whether it’s grown into nearby parts of your eye.
• N: Whether the tumor’s spread to the lymph nodes in your ear and neck.
• M: Whether the cancer’s spread to other organs (usually your liver).

They consider this information together to assign eye cancer a stage between I and IV, with I being the least advanced and IV meaning the cancer’s more advanced.

Collaborative Ocular Melanoma Study (COMS) staging system

Another common system stages cancer based on tumor size. Size influences the type of treatments that will likely work best. Measurements are in millimeters (mm).

• Small: Between 1 mm and 2.5 mm in height and 5 mm to 16 mm in width.
• Medium: Between 2.5 mm and 10 mm in height and 16 mm or less in width.
• Large: Bigger than 10 millimeters in height and 16 millimeters in width.

What additional tests will be done to diagnose eye cancer?

Your provider may recommend liver imaging scans if they suspect your cancer’s spread. Your liver is the most common place for eye cancer to spread outside of your eye.

How is eye cancer treated?

For slow-growing tumors or if the diagnosis isn’t certain, your provider may recommend monitoring your condition and delaying treatment — especially if treatment risks outweigh the benefits. For example, you may want to delay treatment if treating an area could cause vision loss.

Radiation therapy

Radiation therapy is one of the most common treatments for eye cancer.

• Brachytherapy: Brachytherapy, or internal radiation therapy, is the most common treatment for eye melanomas. For treatment, your provider will implant a tiny disc near the tumor that releases radiation to kill cancer cells.
• External beam radiation therapy (EBRT): With EBRT, a machine that never touches your body directs radiation toward a tumor. Techniques include stereotactic surgery, which directs high doses of radiation toward your tumor in one treatment session. Proton beam radiation therapy is another option. This is a newer form of radiation therapy that delivers precise, high doses of radiation toward tumors. As the equipment is very expensive to maintain, it isn’t available everywhere.

Surgery

Surgery is a common treatment option, especially for small tumors that haven’t spread beyond your eyeball. Procedures include:

• Iridectomy: Removes part of your iris. Providers commonly use this procedure when treating small melanomas.
• Iridocyclectomy: Removes part of your iris and ciliary body. Providers commonly use this procedure when treating small melanomas.
• Transscleral resection: Removes melanomas in your choroid or ciliary body.
• Enucleation: Removes your eyeball. You may need this surgery for large tumors or when there’s no way to preserve your vision with treatment. Afterward, you’ll get an artificial eyeball that matches your remaining eye. Your healthcare team will work with you closely to fit you with a replacement that looks and moves like your eye.
• Orbital exenteration: Removes your entire eyeball and some of the surrounding tissue. Your provider may recommend this procedure if the cancer’s spread into structures surrounding your eyeball. As with enucleation, you’ll get an artificial eyeball implant afterward.

Laser therapy

Laser therapy uses heat to destroy eye cancer. The most common type is transpupillary thermotherapy (TTT). During the procedure, infrared light delivers concentrated heat toward the tumor, destroying cancer cells. Providers may use this on its own or after brachytherapy to prevent cancer from returning (recurring).

Immunotherapy

Immunotherapy treatments help your immune system identify and destroy cancer cells more effectively. In certain instances, providers use the immunotherapy drug tebentafusp to treat uveal melanoma. Immunotherapy is a common treatment for cancer that’s spread or that providers can’t surgically remove.

Targeted therapy

Targeted therapy drugs target specific weaknesses in cancer cells, destroying them. You may be eligible for targeted therapy treatments if cancer cells contain a BRAF gene mutation (change). Currently, this mutation is more common in skin melanomas, but this treatment may be beneficial in people with eye melanomas, too.

Chemotherapy

Chemotherapy isn’t a common treatment for eye cancer, but your healthcare provider may recommend it if your cancer hasn’t responded to other treatments or if it spreads to other areas.

What are possible treatment side effects?

Side effects depend on the type of treatment your provider recommends. As these treatments target your eye, it’s possible that you’ll experience vision changes. One of the most significant risks is partial or complete vision loss. These risks depend on multiple factors that you should discuss with your provider.

How can I prevent eye cancer?

There’s no way to prevent eye cancer. Still, you can improve your prognosis by getting screened if you know that you’re in a high-risk group for getting eye cancer. For example, you may consider regular exams if you have BAP1 tumor predisposition syndrome. If you have a family history of retinoblastoma and have a child, it’s a good idea to get them regular eye exams to screen for cancer.

Is cancer of the eye curable?

Your prognosis, or likely treatment outcome, depends on many factors, including the tumor’s size, location and how much it’s spread. For example, brachytherapy eliminates 95% of small and medium intraocular melanomas. Eye cancer may not be curable. However, its growth within your eyeball can be contained.

Ask your healthcare provider about your prognosis based on your specific type of eye cancer.

What is the survival rate of eye cancer?

Survival rates communicate information about how many people with a certain cancer diagnosis are alive five years after their diagnosis when compared to people without the same diagnosis. The survival rates for the most common form of eye cancer, intraocular melanoma, are excellent when a provider diagnoses and treats the cancer when it’s still in your eyeball. The survival rates aren’t as good if the cancer’s spread to other organs.

Fortunately, providers diagnose and treat most cancers before they’ve metastasized.

What questions should I ask my doctor?

Questions you may ask include:

• What type of eye cancer do I have?
• What stage is my cancer?
• What treatment options would you recommend?
• What are potential treatment side effects?
• Will I be able to keep my vision following treatment?
• How likely is it that treatment will eliminate my cancer completely?

A note from Cleveland Clinic

An eye cancer diagnosis can mean many things depending on the type of cancer you have, its location in your eye and whether it’s spread. With the most common types of eye cancer, treatment success depends on early diagnosis. This is why regular eye exams are so important. As most eye cancers don’t cause symptoms in the early stages, irregular findings during an eye exam are usually the first sign of eye cancer. Follow your vision care provider’s guidance on how often you need regular eye exams to detect vision problems and other conditions that may affect your eyes.