Eye cancer is a general term for many types of tumors that occur in various parts of the eye. It occurs when cells in or around the eye grow abnormally and form 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:

• Eyeball
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• Orbit (eye socket)
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• 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
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• Ciliary body: Muscular tissue that produces the watery fluid (aqueous humor) in the eye and helps the eye focus
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• 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. 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 intraocular tumors 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. Refer to the Cancer.Net Guide to Non-Hodgkin Lymphoma for more information.

Retinoblastoma is a rare form of childhood eye cancer. Information about this cancer can be found in the Cancer.Net Guide to Retinoblastoma, Childhood 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 look 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. Some of these tumors can be surgically removed and are not dangerous if they are treated early. Refer to the Cancer.Net Guide to Eyelid Cancer for more information.

To learn about the cancer terms used in this section, read the Cancer.Net Feature: Cancer Terms to Know: Basic Oncology Terms.

Statistics

In 2008, an estimated 2,390 adults (1,340 men and 1,050 women) in the United States will be diagnosed with primary intraocular cancer. It is estimated that 224 deaths (130 men and 110 women) from this disease will occur this year.

Most new cases (between 1,300 and 1,800) of primary intraocular cancer this year will be melanomas. While the number of new cases and deaths from skin melanoma has been increasing over the past 30 years, the number of new intraocular melanoma cases has remained constant or even slightly decreased during this time. Cancer that has spread to the eye from another place in the body (secondary eye cancer) is more common than primary eye cancer.

The five-year relative survival rate (the percentage of people who survive at least five years after the cancer is detected, excluding those who die from other disease) depends on the size and location of the tumor.

Iris melanoma is rare and does not usually spread. The five-year relative survival rate for people with iris melanoma is about 95%.

Choroidal melanoma is the most common type of intraocular melanoma.

• The five-year relative survival rate for people with small choroidal melanoma is approximately 85%.
  
  
• The five-year relative survival rate for people with medium choroidal melanoma is approximately 68%.
  
  
• The five-year relative survival rate for people with large choroidal melanoma is approximately 50% to 60%.

Ciliary body melanoma is rare. Five-year relative survival rates are hard to determine for this type of melanoma, although it generally has a poorer prognosis (chance of recovery) than choroidal melanoma because it is typically diagnosed at a more advanced stage.

Cancer survival statistics should be interpreted with caution. These estimates are based on data from thousands of cases of this type of cancer in the United States each year, but the actual risk for a particular individual may differ. It is not possible to tell a person how long he or she will live with eye cancer. Because the survival statistics are measured in five-year (or sometimes one-year) intervals, they may not represent advances made in the treatment or diagnosis of this cancer.

Statistics adapted from the American Cancer Society's publication, Cancer Facts & Figures 2008, and the National Cancer Institute.

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A risk factor is anything that increases a person's chance of developing cancer. Some risk factors can be controlled, such as smoking, and some cannot be controlled, such as age and family history. Although risk factors can influence the development of cancer, most do not directly cause cancer. Some people with several risk factors never develop the disease, while others with no known risk factors do. However, knowing your risk factors and communicating with your doctor can help guide you in making more informed lifestyle and health-care choices.

The following factors can raise a person's risk of developing eye cancer:

Age. Most cases of primary intraocular melanoma occur in people over the age of 50, and the average age of diagnosis is 55. It is rare in children and people over the age of 70.

Race. Primary intraocular melanoma is more common in white people and less common in black people.

Gender. Intraocular melanoma affects about equal numbers of men and women.

Individual history. People with the following medical conditions have a higher risk of developing primary intraocular melanoma:

• Ocular or oculodermal melanocytosis (pigmentation of the eye or skin around the eye; it is also called nevus of Ota)



• Nevi, or spots like moles in the eye



• Dysplastic nevus syndrome (a condition marked by multiple flat moles that are irregular in shape or color)

Family history. Intraocular melanoma doesn’t generally run in families, although a couple of rare cases have been reported.

Other. Some studies have suggested that sunlight or certain chemicals may be a risk factor for intraocular melanoma, but the data are not conclusive about this association.

It is recommended that people with a combination of these risk factors see an ophthalmologist for a yearly examination and protect their eyes from ultraviolet (UV) radiation with sunglasses. Anyone who finds unusual moles or other skin growths around the eye or elsewhere on the body should see a dermatologist (a doctor specializing in skin diseases), especially if there is a family history of melanoma.

People with intraocular melanoma often have no symptoms. Many times, an ophthalmologist finds the melanoma during a routine eye examination. The most common symptom is painless loss of vision. Unlike choroidal and ciliary body melanoma, iris melanoma can sometimes be seen because it looks like dark spots on the eye.

People with eye cancer may experience the symptoms described below. Sometimes people with eye cancer do not show any of these symptoms. Or, these symptoms may be similar to symptoms of other medical conditions. If you are concerned about a symptom on this list, please talk with your ophthalmologist.

Having trouble seeing


• Losing part of the field of vision



• Seeing flashes of light



• Seeing spots, squiggly lines, or floating objects (floaters)



• Having a dark spot on the iris

Doctors use many tests to diagnose cancer and determine if it has metastasized (spread). Some tests may also determine which treatments may be the most effective. For most types of cancer, a biopsy is the only way to make a definitive diagnosis of cancer. (However, for eye melanoma, a diagnosis can be made without a biopsy.) The doctor may also suggest other tests that will help make a diagnosis. Imaging tests may be used to find out whether the cancer has metastasized. Your doctor may consider these factors when choosing a diagnostic test:

• Age and medical condition
• The type of cancer
• Severity of symptoms
• Previous test results

The following tests may be used to diagnose eye cancer:

Eye examination. Most cases of melanoma are found during a regular eye examination. The doctor will examine the eye with a lighted instrument called an ophthalmoscope and a slit lamp (a microscope with a light attached to it).

Ultrasound. An ultrasound uses sound waves to create a picture of the eye. It may be used to confirm a diagnosis of intraocular melanoma.

Fluorescein angiography. This procedure takes a picture of the blood vessels in the eye. A fluorescent dye (called fluorescein) is injected into the patient's arm. The dye moves through the body and into the blood vessels in the back of the eye. The doctor then takes several, quick pictures of the eye. Fluorescein angiography may be used to rule out eye problems other than cancer. Indocyanine green angiography is a similar test that uses a different dye called indocyanine green.

Fine needle biopsy. This procedure removes tumor cells from the eye with a needle. This allows the doctor to look at the cells under a microscope. Because doctors can correctly diagnose more than 95% of intraocular melanoma without a biopsy, this procedure is not needed for most people.

Tests for metastases. A tumor in the eye can metastasize through the blood to other parts of the body, most commonly the liver. The doctor may see if the tumor has spread to the liver by testing the levels of liver enzymes in the person's blood or through a computed tomography (CT or CAT) scan (creates a three-dimensional picture of the inside of the body with an x-ray machine) or an ultrasound of the liver. The doctor may order a positron emission tomography (PET scan). In a PET scan, radioactive sugar molecules are injected into the body. Cancer cells absorb sugar more quickly than normal cells, so they light up on the PET scan. PET scans are often used to complement information gathered from CT scan, MRI, and physical examination. The doctor may also recommend a chest x-ray to check if the cancer has spread to the lung.

To learn about the terms used in this section, read the Cancer.Net Feature: Cancer Terms to Know: Newly Diagnosed.

To learn more about what to expect during common diagnostic tests, read Cancer.Net: Tests and Procedures.

Staging is a way of describing a cancer, such as where it is located, if or where it has spread, and if it is affecting the functions of other organs in the body. Doctors use diagnostic tests to determine the cancer's stage, so staging may not be complete until all the tests are finished. Knowing the stage helps the doctor to decide what kind of treatment is best and can help predict a person's prognosis (chance of recovery). For example, people with smaller tumors may not need surgery, while many people with larger tumors will need surgery. There are different stage descriptions for different types of cancer.

One tool that doctors use to describe the stage is the TNM system. This system uses three criteria to judge the stage of the cancer: the tumor itself, the lymph nodes around the tumor, and if the tumor has spread to other parts of the body. The results are combined to determine the stage of cancer for each person. The stage provides a common way of describing the cancer, so doctors can work together to plan the best treatments.

There are four stages of intraocular melanoma:

• A stage I or II cancer is a small to medium-sized tumor that has not spread outside the eye.



• A stage III cancer is a larger tumor that has not spread to the lymph nodes or to other parts of the body.



• A stage IV cancer can be any size and has spread to the lymph nodes or to other parts of the body.

TNM is an abbreviation for tumor (T), node (N), and metastasis (M). Doctors look at these three factors to determine the stage of cancer:

• How large is the primary tumor, and where is it located? (T, tumor)



• Has the tumor spread to the lymph nodes? (N, node)



• Has the cancer spread to other parts of the body? (M, metastasis)

Some ophthalmologists may not use the TNM system to stage intraocular tumors. However, they still consider the size of the tumor and how it is affecting a person's vision when deciding on a treatment plan.

Specific information about the TNM system is listed below. In eye cancer, T for an iris melanoma is described differently than T for choroidal and ciliary body melanomas. N and M are described the same for iris, choroidal, and ciliary body melanomas.

Tumor. Using the TNM system, the "T" plus a letter and/or number (0 to 4) is used to describe the size and location of the tumor. Some stages are also divided into smaller groups that help describe a patient's condition in more detail. The following classifications are the same for any type of intraocular melanoma:

TX: The primary tumor cannot be evaluated.

T0: There is no tumor in the eye.

Iris melanoma

Iris tumors are classified as T1, T2, T3, and T4.

T1: The tumor is limited to the iris.

T1a: The tumor is in one quadrant (one-fourth) or less of the iris.

T1b: The tumor is in more than one quadrant of the iris.

T1c: The tumor is only in the iris, but there is melanomalytic glaucoma. This means that a buildup of certain cells in the eye blocks the flow of fluid in the eye, causing pressure.

T2: The tumor has joined or grown into the ciliary body and/or choroid.

T2a: The tumor has joined or grown into the ciliary body and/or choroid with melanomalytic glaucoma.

T3: The tumor has joined or grown into the ciliary body and/or choroid and extends to the sclera (outer wall of the eyeball).

T3a: The tumor has joined or grown into the ciliary body and/or choroid and extends to the sclera in association with melanomalytic glaucoma.

T4: The tumor has spread to the outside of the eyeball, the optic nerve, or to the eye socket. This is called extraocular extension.

Ciliary body and choroid melanoma

Tumors in the ciliary body and choroid are also classified as T1, T2, T3, and T4 based on the size of the tumor. The doctor may measure the tumor size in units called optic disc diameters or millimeters (mm). The tumor is measured in width and height (also called thickness).

T1: The tumor is 10 mm or smaller in diameter and 2.5 mm thick or smaller.

T1a: The tumor is 10 mm or smaller and 2.5 mm thick or smaller and has not spread beyond the eyeball.

T1b: The tumor is 10 mm or smaller and 2.5 mm thick or smaller, and there is microscopic spread beyond the eyeball.

T1c: The tumor is 10 mm or smaller and 2.5 mm thick or smaller, and there is visible spread beyond the eyeball (called macroscopic spread).

T2: The tumor is between 10 mm and 16 mm in its greatest diameter and between 2.5 mm and 10 mm thick.

T2a: The tumor is between 10 mm and 16 mm in its greatest diameter and between 2.5 mm and 10 mm thick, and has not spread beyond the eyeball.

T2b: The tumor is between 10 mm and 16 mm in its greatest diameter and between 2.5 mm and 10 mm thick, and there is some microscopic spread beyond the eyeball.

T2c: The tumor is between 10 mm and 16 mm in its greatest diameter and between 2.5 mm and 10 mm thick, and there is some macroscopic spread beyond the eyeball.

T3: The tumor is more than 16 mm in diameter and/or is more than 10 mm thick, but has not spread beyond the eyeball.

T4: The tumor is more than 16 mm in diameter and more than 10 mm thick and has spread to the outside of the eyeball, the optic nerve, or the eye socket. This is called local extraocular extension.

Node. The "N" in the TNM staging system stands for lymph nodes, the tiny, bean-shaped organs that help fight infection. Lymph nodes near the eye are called regional lymph nodes. Lymph nodes in other parts of the body are called distant lymph nodes. N is described the same for iris, ciliary body, and choroidal melanomas.

NX: The regional lymph nodes cannot be assessed.

N0 (N plus zero): There is no regional lymph node metastasis.

N1: There is regional lymph node metastasis.

Distant metastasis. The "M" in the TNM system indicates whether the cancer has spread from the eye to other parts of the body. M is described the same for iris, ciliary body, and choroidal melanomas.

MX: Distant metastasis cannot be assessed.

M0 (M plus zero): There is no distant metastasis.

M1: There is metastasis to other parts of the body.

Cancer stage grouping

Doctors assign the stage of the cancer by combining the T, N, and M classifications.

Stage I: The tumor diameter is 10 mm or smaller, the tumor's thickness is 2.5 mm or smaller, and the tumor has not spread to the regional lymph nodes or other areas in the body (T1, T1a, T1b, or T1c; N0, M0).

Stage II: The tumor is between 10 mm and 16 mm in diameter, is between 2.5 mm and 10 mm thick, and has not spread to the regional lymph nodes or other areas of the body (T2, T2a, or T2b; N0, M0).

Stage III: The tumor is more than 16 mm in diameter and/or is more than 10 mm thick. It may or may not have spread beyond the eyeball, but has not spread to the regional lymph nodes or to other areas of the body (T3 or T4; N0, M0).

Stage IV: The tumor has spread to either the regional lymph nodes and/or other areas of the body, regardless of how large the tumor is (any T, N1, M0; or any T, any N, M1).

Recurrent: This is cancer that has come back after treatment. It may return in the eye or in another part of the body.

Histopathology and grading

After a biopsy or when the tumor is surgically removed, doctors may look at the kinds of cells that are in the tumor. This is called histopathology, and there are three types of histopathology patterns that may be present in the tumor:

• Spindle cell melanoma (the cells are longer and tapered at the ends)



• Epithelioid melanoma (the cells are oval-shaped)



• Mixed cell melanoma (both spindle and epithelioid)

Generally, a tumor made up of spindle cells has a better prognosis than a tumor made up of epithelioid cells. The tumor is given a grade to describe the composition of its cells. A lower grade generally indicates a better prognosis than a higher grade.

GX: The grade cannot be assessed

G1: A spindle cell melanoma

G2: A mixed cell melanoma

G3: An epithelioid melanoma

Used with permission of the American Joint Committee on Cancer (AJCC), Chicago, Illinois. The original source for this material is the AJCC Cancer Staging Manual, Sixth Edition (2002) published by Springer-Verlag New York, www.springer-ny.com.

The treatment of intraocular melanoma depends on the size and location of the tumor, whether the cancer has spread, and the person’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 eye, if possible. In many cases, a team of doctors may work with the patient to determine the best treatment plan.

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 cancer is monitored closely, and treatment begins if the tumor shows signs of becoming more aggressive or spreading. This approach may also be called active surveillance, 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 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 demonstrate growth or have high-risk features.

Surgery

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.

• Iridectomy: Removal of part of the iris
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• Iridocyclectomy: Removal of part of the iris and ciliary body
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• Sclerouvectomy/endoresection: Surgery to remove the choroidal tumor while keeping the eye
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• 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.

Having an eye removed

Sometimes the only choice a doctor has in treating intraocular melanoma is to remove the eye. This means that the person will lose all of their vision in that 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.

Radiation therapy

Radiation therapy, which may also be called radiotherapy, is the use of high-energy x-rays or other particles to kill cancer cells. There are different types of radiation therapy.

• Proton-beam or charged-particle radiation therapy directs high-energy particles to the tumor, which reduces the possibility of damage to nearby tissue.
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• Brachytherapy is a procedure where the ophthalmologist places a radioactive disc (sometimes called a plaque) near the tumor. This may also be called plaque therapy.
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• Traditional external-beam radiation therapy delivers x-rays from outside the body to the tumor and may be given after enucleation or as a palliative treatment (treatment that improves a person's quality of life).

Radiation therapy can 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 person'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 can be surgically removed.
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• 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
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• Radiation optic neuropathy: Optic nerve damage
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• Neovascular 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.

Laser therapy

This procedure uses heat in the form of a laser to shrink smaller tumors. 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

Iris melanoma

Iris melanoma is not generally treated unless the tumor begins to grow. The following are examples of common treatment options:

• Observation
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• Surgery-iridectomy
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• Radiation therapy (charged particle or brachytherapy)
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• 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:

• Observation
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• Radiation therapy (charged particle or brachytherapy)
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• Laser therapy
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• Surgical resection and/or enucleation

Careful observation is common, 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 medium-sized choroidal tumors. The following are treatment options for a medium-sized tumor:

• Radiation therapy (charged particle or brachytherapy)
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• Surgery to remove the tumor
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• Enucleation
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• 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 these types of tumors.

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.

Recurrent intraocular melanoma

Recurrent uveal 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 remove 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.

Metastatic intraocular melanoma

This is melanoma that has spread from the eye to other parts of the body. 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.

To learn about the terms used in this section, read the Cancer.Net Feature: Cancer Terms to Know: During Treatment.

Doctors are always looking for better ways to treat patients with eye cancer. A clinical trial is a way to test a new treatment in order to prove that it is safe, effective, and possibly better than a standard treatment. Patients who participate in clinical trials are among the first to receive new treatments before they are widely available. However, there is no guarantee that the new treatment will be safe, effective, or better than a standard treatment. One clinical trial that has shaped how intraocular melanoma is treated is COMS.

Patients decide to participate in clinical trials for many reasons. For some patients, a clinical trial is the best treatment option available. Because standard treatments are not perfect, patients are often willing to face the added uncertainty of a clinical trial in the hope of a better result. Other patients volunteer for clinical trials because they know that the only way to make progress in treating eye cancer is to find new therapies. Even if they do not benefit directly from the clinical trial, their participation may benefit future patients with eye cancer.

To join a clinical trial, patients must complete a learning process known as informed consent. During informed consent, the doctor should list all of the patient's options, so the person understands how the new treatment differs from the standard treatment. The doctor must also list all of the risks of the new treatment, which may or may not be different from the risks of standard treatment. Finally, the doctor must explain what will be required of each patient in order to participate in the clinical trial, including the number of doctor visits, tests, and the schedule of treatment. Learn more about clinical trials, including patient safety, phases of a clinical trial, deciding to participate in a clinical trial, questions to ask the research team, and links to find cancer clinical trials.

Cancer and cancer treatment can cause a variety of side effects; some are easily controlled, and others require specialized care. Below are some of the side effects that are common to eye cancer and its treatments. For more detailed information on managing these and other side effects of cancer and cancer treatment, visit the Cancer.Net Managing Side Effects section.

Infection. An infection occurs when harmful bacteria, viruses, or fungi (such as yeast) invade the body and the immune system is not able to destroy them quickly enough. Patients with cancer are more likely to develop infections because both cancer and cancer treatments (particularly chemotherapy and radiation therapy to the bones or extensive areas of the body) can weaken the immune system. Symptoms of infection include fever (temperature of 100.5°F or higher); chills or sweating; sore throat or sores in the mouth; abdominal pain; pain or burning when urinating or frequent urination; diarrhea or sores around the anus; cough or breathlessness; redness, swelling, or pain, particularly around a cut or wound; and unusual vaginal discharge or itching.

Pain. Depending on the stage of disease, 30% to 75% of all patients experience pain from cancer. About 85% to 95% of cancer pain can be treated successfully. Pain can make other aspects of cancer seem worse, such as fatigue (tiredness), weakness, sleep disturbance, and confusion. Pain can come from the tumor itself or may be a result of cancer treatment. Pain from a tumor can be a result of the tumor growing and spreading to the bones or other organs and putting pressure on and damaging nerves. Pain from surgery is normal and may persist for months or years. Pain may develop after radiation therapy and go away on its own. It can also develop months or years after treatment.

Loss of vision. Visual loss can happen immediately or gradually in the eye that was treated. Talk with your ophthalmologist if you start to experience any visual changes.

Dry eye. A dry eye can also be a complication of treatment. Some treatment options include over the counter eye drops, prescription eye drops such as cyclosporine ophthalmic (Restasis), and plugs that can be placed in the tear ducts. Talk with your ophthalmologist about how to manage this and other side effects.

After treatment for eye cancer ends, talk with your doctor about developing a follow-up care plan. This plan may include regular physical examinations and/or medical tests to monitor your recovery in the coming months and years. The follow-up care plan for people with intraocular melanoma will depend on the treatment they received. People who have had radiation therapy or have had no treatment, but are being carefully observed, should have the tumor monitored closely, about every three to six months. Patients who have had the eye surgically removed should receive a yearly eye examination, and doctors should monitor the orbit closely for the next two to three years.

All people treated for intraocular melanoma need to be monitored for evidence of cancer that may have spread to other areas of the body. This process may include a physical examination, blood work, and imaging tests, such as chest x-rays, ultrasound tests, or CT scans every six to 12 months. Your doctor can work with you to decide on an acceptable follow-up care plan.

A cancer diagnosis causes people to cope with a number of issues, and the same is true once cancer treatment has ended. Some people with eye cancer may experience depression. If you think that you or a family member may be experiencing depression, it is important to ask for help because it can be treated. Read more about Mental Health and Cancer and Managing Side Effects: Depression.

Some people may have difficulties coping with the fact that the cancer could come back, even years later, or spread to other parts of the body. This fear is especially true in people who keep their eye. Read more about Coping With Fear of Recurrence. Other people may fear loss of vision. Learn more about common issues cancer survivors face on Cancer.Net: Survivorship.

To learn about the terms used in this section, read the Cancer.Net Feature: Cancer Terms to Know: After Treatment.

Research involving more advanced diagnostic procedures and treatments for eye cancer is ongoing. The following advancements may still be under investigation in clinical trials and may not be approved or available at this current time. Always discuss all diagnostic and treatment options with your doctor.

Genetics. As with other types of cancer, researchers are studying the genes and proteins that may be involved in the development of intraocular melanoma. These studies may eventually lead to better treatment options. Researchers are also looking for markers, or proteins in the blood, that may tell the doctor if the tumor has spread to other parts of the body.

Improving radiation therapy. Doctors are finding ways to make radiation therapy safer by reducing the side effects to the eye. These methods include adjusting the dose of radiation and finding ways to direct radiation only at the tumor. Doctors are also experimenting with sandwich therapy, which is a combination of laser therapy and radiation disc therapy.

Biologic therapy. Biologic therapy is also called immunotherapy. This treatment helps restore or stimulate the body’s immune system. Several types of biologic therapies are being tested in clinical trials in people with advanced intraocular melanoma.

• Cancer vaccines are experimental treatments that stimulate the person's own immune system to fight cancer. In one study, a vaccine that has been tested in people with cutaneous (skin) melanoma is being tested in people with intraocular melanoma. This vaccine is an adjuvant therapy, meaning it is given with other types of therapy. It may help the person's immune system fight cancer and prevent the tumor's spread to other areas of the body. Sometimes the vaccine is given with an immune system stimulant, such as GM-CSF (sargramostim).



• Monoclonal antibodies specifically target and kill cancer cells.

Chemotherapy. Chemotherapy is the use of drugs to kill cancer cells. New chemotherapeutic agents are being tested in patients with late-stage melanoma. Sometimes this is combined with new drugs that help shut off the blood supply to the tumor, called anti-angiogenesis drugs.

Treating liver metastases. Because intraocular melanoma frequently metastasizes to the liver, many people need treatment to the liver. A technique called chemoembolization allows doctors to separate the blood supply to the liver from the rest of the body and deliver chemotherapy directly to the liver.

Regular communication with your doctor is important in making informed decisions about your health care. Consider asking the following questions of your doctor.

General questions:

• What is my diagnosis?



• What does this mean?



• Do I need treatment right away?



• What are my treatment options?



• What treatment do you recommend?



• What is the goal of this treatment?



• What clinical trials are open to me?

For patients who need surgery:

• What type of surgery do you recommend? Why?



• What are the side effects of my surgery?



• Will I need to stay in the hospital for this surgery? For how long?



• Will I have problems with my vision afterwards, either immediately or in the long term?

For patients who need an eye removed:

• How do I adjust to using one eye?



• How long will it take me to recover?



• How soon can I get a prosthesis (artificial eye)?



• When do I get a permanent prosthesis?



• How do I care for my prosthesis?

For patients who need radiation therapy:

• What kind of radiation therapy will I receive?



• What does the preparation for this treatment involve?



• What is the likelihood of having radiation damage to my eye?



• What is the risk of vision loss?



• What other side effects can I expect from this treatment?



• What can be done to treat the side effects?

After treatment:

• What are the chances that the cancer will return?



• What follow-up tests do I need, and how often do I need them?

The Eye Cancer Network
Eye Care Foundation
115 E. 61st Street
New York, NY  10021
Phone: 212-832-8170
www.eyecancer.com

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