Eye Cancer

Eye cancer is a general term used to describe many types of tumors that occur in various parts of the eye. It occurs when cells in or around the eye begin to change and grow uncontrollably, forming a mass called a tumor. A tumor may be benign (noncancerous) or malignant (cancerous, meaning cells can spread to other parts of the body). Cancer that forms in the eyeball is called an intraocular malignancy.

Parts of the eye: The eye is the organ that collects light and sends messages to the brain to form a picture. The three main parts of the eye are:pan>

Eyeball
Orbit (eye socket)
Adnexal (accessory) structures (such as the eyelid and tear glands)

The outer part of the eye is made up of the sclera, retina, and uvea. The sclera is the outer wall of the eyeball. The retina is a thin-layered structure that lines the eyeball and sends information from the eye to the brain. The uvea nourishes the eye. Both the retina and the uvea contain blood vessels. The uvea consists of the following:

Iris: The colored part of the eye that controls the amount of light entering the eye

Ciliary body: Muscular tissue that produces the watery fluid (aqueous humor) in the eye and helps the eye focus

Choroid: The layer of tissue underneath the retina that contains connective tissue and melanocytes and nourishes the inside of the eye; the choroid is the most common site for a tumor.

Types of intraocular cancer: The most common intraocular cancer in adults is uveal metastases, which is cancer that has spread to uvea from another place in the body, called secondary cancer. This article is about primary intraocular cancer, meaning that the tumor started in the eye, not somewhere else in the body.

Melanoma Is the most common type of primary intraocular cancer in adults. It begins when pigmented (colored) cells in the eye called melanocytes grow uncontrollably. Intraocular melanoma is also called uveal melanoma. Medical doctors who specialize in the diseases and function of the eye are called ophthalmologists (or “eye MDs”). These doctors can diagnose and treat intraocular melanoma. Optometrists are another type of eye doctor. They prescribe eyeglasses and contact lenses. They are not medical doctors and are not trained to treat intraocular cancer. Other, less common types of an intraocular tumor include:

Intraocular lymphoma: Is lymphoma that begins in the eyeball. This condition is rare and can be difficult for doctors to diagnose. Many doctors consider intraocular lymphoma to be a type of central nervous system lymphoma. Most intraocular lymphomas are non-Hodgkin lymphoma. Retinoblastoma is a rare form of childhood eye cancer. Hemangioma is a benign vascular tumor of the choroid and retina. In addition, rare tumors of the eye include:

Conjunctival Melanoma: A tumor of the conjunctiva (a membrane that lines the eyelid and eyeball). If this tumor is not treated, it can spread to the lymph nodes. This tumor tends to recur (come back after treatment) on the eye’s surface and looks like dark spots on the eye. Doctors often perform a biopsy (removal of a sample of the tissue for examination under a microscope) on a lesion that appears to be conjunctival melanoma. Eyelid carcinoma (basal or squamous cell) is a variation of skin cancer. This tumor may be surgically removed and is usually not dangerous if it is treated early.

Treatment: The treatment of intraocular melanoma depends on the size and location of the tumor, whether the cancer has spread, and the patient’s overall health. The main goals in treating intraocular melanoma are to reduce the risk of the tumor spreading and to maintain the health and vision of the patient’s eye, if possible. In many cases, a team of doctors may work with the patient to determine the best treatment plan.

This section outlines treatments that are the standard of care (the best treatments available) for this specific type of cancer. Patients are also encouraged to consider clinical trials as a treatment option when making treatment plan decisions. A clinical trial is a research study to test a new treatment to prove it is safe, effective, and possibly better than standard treatment. Your doctor can help you review all treatment options. For more information, see the Clinical Trials section. Descriptions of the most common treatment options for eye cancer are listed below.

Active surveillance/observation: The doctor may recommend this approach if the intraocular melanoma is small or slow-growing and/or if treating the cancer would cause more discomfort than the disease itself, such as for people without any symptoms, older or seriously ill people, or people with a tumor in their only useful eye. The patient is monitored closely, and treatment begins if the tumor shows signs of becoming more aggressive or spreading. This approach may also be called observation, watchful waiting, or watch-and-wait. If the tumor grows bigger than 10 mm in diameter or 2 mm to 3 mm in height (thickness), then the doctor and the patient may decide to proceed with active treatment.

Some people may be worried that the cancer should be treated right away. Because treating the cancer has side effects, some of which can be harmful to the eye, doctors may not want to treat a smaller tumor until it starts to grow or shows high-risk features.

Surgery: A surgical oncologist is a doctor who specializes in treating cancer using surgery. Eye surgery is typically performed by an ophthalmologist. Surgery to the eye is quite common in the treatment of intraocular melanoma. During surgery, the ophthalmologist will remove parts of the affected eye, or even the entire eye, depending on the size and spread of the tumor. Surgical options include:

Iridectomy: Removal of part of the iris

Iridocyclectomy: Removal of part of the iris and ciliary body

Sclerouvectomy/endoresection: Surgery to remove the choroidal tumor while keeping the eye

Enucleation: Removal of the eye

In some cases, surgery may also be used to place a radioactive disc for internal radiation therapy (brachytherapy). More information about radiation therapy is below. The potential side effects of eye surgery are similar to that of any surgery, including a risk of infection, problems from the general anesthesia (the medication used during surgery), and pain. With total removal of the eye, there is a slight risk that the tumor could come back in the orbit. Many patients want to know immediately whether the surgery was successful. However, the success of an operation is hard to tell right away because it may take months before the doctors can determine if all of the cancer cells were removed during surgery. Learn more about cancer surgery.

Having an eye removed: Sometimes the only choice a doctor has in treating intraocular melanoma is to remove the eye. Because of this visual loss, a person with one eye may have trouble with depth perception. Most people adjust to these differences. Many people worry about what they will look like when they have an eye removed. The cosmetic surgery available today usually yields good cosmetic results. To fill the area left by the missing eye, the person is fitted for a prosthesis (artifical eye). The prothesis will look and behave almost the same as a natural eye. For example, the artifical eye will move along with the person’s remaining eye, just not as much as a natural eye moves. Family members may be able to tell that the eye is not real, but it is unlikely that strangers will know. If enucleation is required, talk with your doctor about a prosthesis; it may take many weeks for patients to receive the prosthesis. Also, ask about support services that may be available to you to help adjust to the loss of an eye. Learn more about rehabilitation.

Radiation therapy: Radiation therapy is the use of high-energy x-rays or other particles to kill cancer cells. A doctor who specializes in giving radiation therapy to treat cancer is a radiation oncologist. A radiation therapy regimen (schedule) usually consists of a specific number of treatments given over a specific time.

The most common type of radiation treatment is called external-beam radiation therapy, which is radiation given from a machine outside the body. Traditional external-beam radiation therapy may be given after enucleation or as a palliative treatment (treatment that improves a person’s quality of life). When radiation treatment is given using implants, it is called internal radiation therapy, brachytherapy, or plaque therapy. For this treatment, the ophthalmologist places a radioactive disc (sometimes called a plaque) near the tumor.

Proton therapy (also called proton beam therapy) is a type of external-beam radiation therapy that uses protons rather than x-rays. At high energy, protons can destroy cancer cells. Learn more about proton therapy. Radiation therapy may result in a variety of side effects, so it is important to talk with your ophthalmologist about what to expect. The extent of side effects depends on the type and dose of radiation therapy the person receives, where the tumor is located, and the patient’s general health. The side effects may not show up right away.

Cataracts are very common. A cataract is when the lens of the eye becomes cloudy. People with cataracts may have cloudy or foggy vision, have trouble seeing at night, or have problems with glare from the sun or bright lights. If the cataract is causing major problems with a person’s eyesight, it may be surgically removed.

Loss of eyelashes and/or a dry eye can occur with external-beam radiation therapy and proton-beam radiation therapy.

The following side effects are less common and can cause a loss of vision:

Radiation retinopathy: The development of abnormal blood vessels in the retina

Radiation optic neuropathy: Optic nerve damage

Nonvascular glaucoma: A painful condition where new blood vessels develop and block the outflow of fluid from the eye

If there is significant damage to the eye from radiation therapy, the eye may need to be removed. Learn more about radiation therapy.

Laser therapy: This procedure uses heat in the form of a laser to shrink a smaller tumor. It may also be called thermotherapy or transpupillary thermotherapy (TTT). This treatment potentially has fewer side effects than surgery or radiation therapy. Laser therapy may also be combined with radiation therapy.

Treatment by disease and stage: Below is an outline of the common treatments used depending on the type and stage of the eye cancer. In addition to standard treatments, patients are encouraged to talk with their doctors about clinical trials that are open to them, no matter the type or stage of the tumor.

Iris melanoma: Iris melanoma is not generally actively treated unless the tumor begins to grow, although there can be exceptions. The following are examples of common treatment options:

Active surveillance/observation

Surgery—iridectomy

Radiation therapy (charged-particle therapy, such as proton therapy, or brachytherapy)

Enucleation, if the tumor is too large to remove or it spreads beyond the eye

Small choroidal and ciliary body tumor: The following are some treatment options for a small choroidal or ciliary body tumor:

Active surveillance/observation

Radiation therapy (charged-particle therapy, such as proton therapy, or brachytherapy)

Laser therapy

Surgical resection (removal of the tumor) and/or enucleation

Careful observation is a common treatment plan, although patients and their doctors may choose another option depending upon the location of the tumor or if the tumor begins to grow

Medium choroidal and ciliary body tumor: The two most common treatment options for medium-sized choroidal and ciliary body melanoma are radiation therapy and enucleation. It is believed that there is no difference in survival rates between these two treatment methods for a medium-sized choroidal tumor. The following are treatment options for a medium-sized tumor:

Radiation therapy (charged-particle therapy, such as proton therapy, or brachytherapy)

Surgery to remove the tumor

Enucleation

Enrolling in a clinical trial

In addition, the combination of laser therapy and radiation therapy (sometimes called “sandwich therapy”) is being used more frequently to treat this type of tumor.

Large choroidal and ciliary body tumor: For a large tumor, enucleation is the usual treatment. Results of the Collaborative Ocular Melanoma Study (COMS) revealed that patients had similar survival rates whether they received radiation therapy before enucleation or had their eye removed with no prior radiation treatment. Enrolling in a clinical trial may be another option for people with large choroidal and ciliary body tumors, as is additional radiation brachytherapy.

Recurrent intraocular melanoma: Recurrent melanoma is melanoma that has come back after previous treatment. Enucleation is one of the main treatments if a person’s eye has not yet been removed. A person with recurrent intraocular melanoma may also consider enrolling in a clinical trial.

Extraocular extension melanoma: If the tumor has spread to the outside of the eye, optic nerve, or eye socket, the doctor may recommend removal of the eye. Or, the doctor may perform a modified enucleation, which is the removal of the eyeball and adjacent structures. In some cases, the doctor may decide to remove the entire eye and the adjacent structures in a process called an exenteration. If the spread is small, some doctors will try to save the eye by removing the outer part of the tumor and treating the eye with radiation therapy. Talk with your doctor about possible treatment options.

Metastatic intraocular melanoma: This is melanoma that has spread from the eye to other parts of the body, such as the liver. Treatment options include treating the disease in the affected organ or enrolling in a clinical trial. Palliative treatment can also be used to reduce pain, control symptoms, and make a person more comfortable.