Oncologist-approved cancer information from the American Society of Clinical Oncology

Eye Cancer


Last Updated: February 06, 2012

This section has been reviewed and approved by the Cancer.Net Editorial Board,  04/10

Overview

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:

  • 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.

Find out more about basic cancer terms used in this section.

Statistics

This year, an estimated 2,610 adults (1,310 men and 1,300 women) in the United States will be diagnosed with primary intraocular cancer. It is estimated that 270 deaths (120 men and 150 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 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 people with 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 eye cancer.

Statistics adapted from the American Cancer Society's publication, Cancer Facts & Figures 2012, and the National Cancer Institute.

Medical Illustrations

Eye Cancer

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Risk Factors

A risk factor is anything that increases a person’s chance of developing cancer. 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 them to your doctor may help you make 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 factors. 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.

Symptoms

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.

People with eye cancer may experience the following symptoms or signs. Sometimes people with eye cancer do not show any of these symptoms. Or, these symptoms may be caused by a medical condition that is not cancer. If you are concerned about a symptom or sign on this list, please talk with your doctor.

  • 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. Unlike choroidal and ciliary body melanoma, iris melanoma can sometimes be seen because it looks like dark spots on the eye.

Diagnosis

Doctors use many tests to diagnose cancer and find out 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 often 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 suspected

  • Severity of symptoms

  • Previous test results

In addition to a physical examination, 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.

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 and risk of metastases. A tumor that starts in the eye can spread 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 or an ultrasound of the liver. A CT scan creates a three-dimensional picture of the inside of the body with an x-ray machine. A computer then combines these images into a detailed, cross-sectional view that shows any abnormalities or tumors. Sometimes, a contrast medium (special dye) is injected into a patient’s vein to provide better detail. The doctor may also order a positron emission tomography (PET) scan. A PET scan is a way to create pictures of organs and tissues inside the body. A small amount of a radioactive substance is injected into a patient’s body. This substance is absorbed mainly by organs and tissues that use the most energy. Because cancer tends to use energy actively, it absorbs more of the radioactive substance. A scanner then detects this substance to produce images of the inside of the body. The doctor may also recommend a chest x-ray to check if the cancer has spread to the lung.

Another test is available that helps predict whether a choroidal melanoma or ciliary body melanoma is likely to spread to distant sites in the body. Doctors examine cells from the tumor from either a biopsy or enucleation (removal of the eye) and examine abnormal genes and proteins in the cells by a process called gene expression profiling to classify the tumor as either a class I (at high risk for metastasis) or class II (at low risk). Talk with your doctor about the risks and benefits of having this test.

Learn more about what to expect when having common tests, procedures, and scans.

Find out more about common terms used during a diagnosis of cancer.

Staging

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 patient's prognosis (chance of recovery). 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.

In addition to staging, doctors may use other information to help figure out prognosis and the risk of the cancer spreading. These findings may also be included on the pathology report and include:

  • Alterations to the cancer cell’s chromosomes (a long strand of genes); for example, one copy of chromosome three, called monosomy 3, can indicate a higher risk of the cancer spreading

  • Gene expression profiles (as described in Diagnosis); these tests classify a tumor into class I (at lower risk for metastasis) and class II (at higher risk for metastasis)

  • Other characteristics of the cancer cells, such as the grade (see below for more details)

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 an intraocular tumor. 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 the tumor in even 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

An iris tumor is classified as T1, T2, T3, and T4. Some stages are divided into smaller groups that help describe the tumor in even more detail.

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.

T4a: The tumor has spread is less than 5 millimeters (mm) outside of the eye.

T4b: The tumor has spread more than 5 mm outside of the eye

Ciliary body and choroid melanoma

A tumor in the ciliary body and choroid is also classified as T1, T2, T3, and T4 based on the size of the tumor, which is measured in optic disc diameters or millimeters (mm). The tumor is measured for both width and height (also called thickness). A tumor is given a classification according to the table below, based on its width and height.

Size Category Classification Table for Ciliary Body and Choroid Melanoma

Thickness (mm)

Category

 

 

 

 

 

 

Thicker than 15 mm

 

 

 

 

4

4

4

12.1 to 15.0

 

 

 

3

3

4

4

9.1 to 12.0

 

3

3

3

3

3

4

6.1 to 9.0

2

2

2

2

3

3

4

3.1 to 6.0

1

1

1

2

2

3

4

Less than 3.0

1

1

1

1

2

2

4

Largest basal diameter (mm)

Less than 3.0 mm

3.1 to 6.0 mm

6.1 to 9.0 mm

9.1 to 12.0 mm

12.1 to 15.0 mm

15.1 to 18.0 mm

Larger than 18.0 mm

Used with permission of the American Joint Committee on Cancer (AJCC), Chicago, Illinois.

T1: The tumor is size category 1.

T1a: The tumor is size category 1 and does not involve the ciliary body or other parts of the eye.

T1b: The tumor is a category 1 and involves the ciliary body.

T1c: The tumor is size category 1 that does not involve the ciliary body. But, there is a very small area (5 mm or less in diameter) of visible spread beyond the eyeball (called extraocular spread).

T1d: Thetumor is a size category 1 that involves the ciliary body with extraocular spread less than 5 mm.

T2: The tumor is size category 2.

T2a: The tumor is size category 2 and does not involve the ciliary body or other parts of the eye.

T2b: The tumor is size category 2 and involves the ciliary body.

T2c: The tumor is size category 2 that does not involve the ciliary body. But, there is a very small area (5 mm or less in diameter) of visible spread beyond the eyeball.

T2d: The tumor is size category 2 that involves the ciliary body with extraocular spread less than 5 mm.

T3: The tumor is size category 3.

T3a: The tumor is size category 3 and does not involve the ciliary body or other parts of the eye.

T3b: The tumor is size category 3 and involves the ciliary body.

T3c: The tumor is size category 3 that does not involve the ciliary body. But, there is a very small area (5 mm or less in diameter) of visible spread beyond the eyeball.

T3d: The tumor is size category 3 that involves the ciliary body with extraocular spread less than 5 mm.

T4: The tumor is size category 4.

T4a: The tumor is size category 4 and does not involve the ciliary body or other parts of the eye.

T4b: The tumor is size category 4 and involves the ciliary body.

T4c: The tumor is size category 4 that does not involve the ciliary body. But, there is a very small area (5 mm or less in diameter) of visible spread beyond the eyeball.

T4d: The tumor is size category 4 that involves the ciliary body with extraocular spread less than 5 mm.

T4e: The tumor is any size category with extraocular spread of more than 5 mm in diameter.

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 melanomas of the iris, ciliary body, and choroid.

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.

M1a: There is metastasis to other parts of the body and the largest metastasis is 3 centimeters (cm) or less in diameter.

M1b: There is metastasis to other parts of the body and the largest metastasis is between 3.1 cm and 8 cm in diameter.

M1c: There is metastasis to other parts of the body and the largest metastasis is larger than 8.1 cm in diameter.

Cancer stage grouping

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

Stage I: The tumor is size category 1 and does not involve the ciliary body or other parts of the eye, nor has it spread to the regional lymph nodes or to other areas of the body (T1a, N0, M0).

Stage IIA: The tumor is either a size category 1 that may or may not involve the ciliary body, with or without extraocular extension, or it is a size category 2 that does not involve the ciliary body. There is no spread to the regional lymph nodes or to other areas of the body (T1b, T1c, T1d, or T2a; N0, M0).

Stage IIB: The tumor is either a size category 2 that involves the ciliary body but has not spread beyond the eyeball, or it is a size category 3 that has not spread to the ciliary body or eyeball. It has not spread to the regional lymph nodes or to other areas of the body (T2b or T3a; N0, M0).

Stage IIIA: Stage IIIA describes any one of these conditions:

  • A tumor of size category 2 with extraocular spread to a diameter of 5 mm or less, with or without ciliary body involvement that has not spread to the lymph nodes or to other parts of the body (T2c or T2d, N0, M0)

  • A tumor of size category 3 that may or may not involve the ciliary body, with or without extraocular spread to a diameter of 5 mm or less, but hasn’t spread to the lymph nodes or to other parts of the body (T3b or T3c, N0, M0)

  • A tumor of size category 4 that does not involve the ciliary body and has not spread to the lymph nodes or to other parts of the body (T4a, N0. M0).

Stage IIIB: Stage IIIB describes any one of these conditions:

  • The tumor is either a size category 3 with ciliary body involvement and extraocular spread that has not spread to the lymph nodes or to other parts of the body (T3d, N0, M0)

  • The tumor is a size category 4 with or without ciliary body involvement that may or may have spread outside the eyeball. It has not spread to the regional lymph nodes or to other areas of the body (T4b or T4c, N0, M0).

Stage IIIC: The tumor is a size category 4 that involves the ciliary body and has spread outside the eyeball. However, it has not spread to the regional lymph nodes or to other areas of the body (T4d or T4e; N0, M0).

Stage IV: This stage describes a tumor of any size that has spread to the lymph nodes and/or to other parts of the body outside of the eye (any T, N1, M0; or, any T, any N, M1).

Recurrent: Recurrent cancer 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 types 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 (G) to describe the composition of its cells. A lower grade generally indicates a better prognosis than a higher grade.

GX: The grade cannot be evaluated

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, Seventh Edition (2010) published by Springer-Verlag New York, www.cancerstaging.net

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

  • 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. 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.

Find out more about common terms used during cancer treatment.

Clinical Trials Resources

Doctors and scientists are always looking for better ways to treat patients with eye cancer. A clinical trial is a way to test a new treatment to prove that it is safe, effective, and possibly better than a standard treatment. The clinical trial may be evaluating a new drug, a new combination of existing treatments, a new approach to radiation therapy or surgery, or a new method of treatment or prevention. 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.

For instance, one clinical trial that has shaped how intraocular melanoma is treated is called 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 finding new drugs and other therapies is the only way to make progress in treating eye cancer. Even if they do not benefit directly from the clinical trial, their participation may benefit future patients with eye cancer.

Sometimes people have concerns that, by participating in a clinical trial, they may receive no treatment by being given a placebo or a “sugar pill”. The use of placebos in cancer clinical trials is rare. When a placebo is used in a study, it is done with the full knowledge of the participants. Find out more about placebos in cancer clinical trials.

To join a clinical trial, patients must participate in a 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.

For specific topics being studied for eye cancer, learn more in the Current Research section.

Side Effects

Cancer and its treatment can cause a variety of side effects. However, doctors have made major strides in recent years in reducing pain, nausea and vomiting, and other physical side effects of cancer treatments. Many treatments used today are less intensive but as effective as treatments used in the past. Doctors also have many ways to provide relief to patients when such side effects do occur.

Fear of treatment side effects is common after a diagnosis of cancer, but it may be helpful to know that preventing and controlling side effects is a major focus of your health care team. Before treatment begins, talk with your doctor about possible side effects of the specific treatments you will be receiving. The specific side effects that can occur depend on a variety of factors, including the type of cancer, its location, the individual treatment plan (including the length and dosage of treatment), and the person’s overall health.

Ask your doctor which side effects are most likely to happen (and which are not), when side effects are likely to occur, and how they will be addressed by the health-care team if they do happen. Also, be sure to communicate with the doctor about side effects you experience during and after treatment. Learn more about the most common side effects of cancer and different treatments, along with ways to prevent or control them.

In addition to physical side effects, you may experience psychosocial (emotional and social) effects as well. Learn more about the importance of addressing such needs, including concerns about managing the cost of your medical care.

Learn more about late effects or long-term side effects by reading the After Treatment section or talk with your doctor.

After Treatment

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 decided to choose active surveillance 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 to watch for any sign that the cancer 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, CT scans, or PET scans, every six to 12 months. Your doctor can work with you to decide on an appropriate follow-up care plan. ASCO offers cancer treatment summary forms to help keep track of the cancer treatment you received and develop a survivorship care plan once treatment is completed.

People recovering from eye cancer are encouraged to follow established guidelines for good health, such as maintaining a healthy weight, not smoking, eating a balanced diet, and having recommended cancer screening tests. Talk with your doctor to develop a plan that is best for your needs. Moderate physical activity can help rebuild your strength and energy level. Your doctor can help you create an appropriate exercise plan based upon your needs, physical abilities, and fitness level. Learn more about healthy living after cancer.

Find out more about common terms used after cancer treatment is complete.

Current Research

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

Tumor biology/targeted therapy for melanoma. Researchers are studying the genes and proteins that may be involved in the development of intraocular melanoma. Targeted therapy is a treatment that targets the cancer’s specific genes, proteins, or the tissue environment that contributes to cancer growth and survival.

Recent studies show that not all tumors have the same targets. Many research studies are taking place now to find out more about specific molecular targets and new treatments directed at them. Learn more about targeted treatments.

To learn more about possible targeted treatments for specific subtypes, use the Targeted Therapy Finder – Melanoma tool.

Researchers are also looking for markers, or specific substances in the blood, that may tell the doctor if the tumor has spread to other parts of the body. Researchers are also performing biopsies on the tumors to help predict whether a specific tumor is likely to 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 better ways to direct radiation only at the tumor.

Biologic therapy. Biologic therapy is also called immunotherapy, which is designed to boost the body’s natural defenses to fight cancer. It uses materials either made by the body or in a laboratory to bolster, target, or restore immune system function. 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 after 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. Systemic chemotherapy is delivered through the bloodstream, targeting cancer cells throughout the body. Chemotherapy is given by a medical oncologist, a doctor who specializes in treating cancer with medication. Some people may receive chemotherapy in their doctor’s office; others may go to the hospital. A chemotherapy regimen (schedule) usually consists of a specific number of cycles given over a specific time.

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

To find clinical trials specific to your diagnosis, talk with your doctor or search online clinical trial databases now.

Questions to Ask the Doctor

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 type of eye cancer do I have?

  • Where exactly is the tumor located?

  • What stage is the cancer? What does this mean?

  • Can you explain my pathology report (laboratory test results) to me?

  • Do I need treatment right away?

  • What are my treatment options?

  • What clinical trials are open to me?

  • Who will be part of my health care team, and what does each member do?

  • Who will be coordinating my overall treatment and follow-up care?

  • What treatment do you recommend? Why?

  • What is the goal of this treatment?

  • What are the possible side effects of this treatment, both in the short term and the long term?

  • How will this treatment affect my daily life? Will I be able to work, exercise, and perform my usual activities?

  • If I’m worried about managing the costs related to my cancer care, who can help me with these concerns?

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 only having one eye?

  • How long will it take me to recover physically?

  • What rehabilitation services are available?

  • 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?

  • What support services are available to me? To my family?

Patient Information Resources

In addition to Cancer.Net, there are other sources of information about this type of cancer available online. Cancer.Net maintains a list of national, not-for-profit organizations that may be helpful in finding additional information, services, and support. As always, be sure to talk with your doctor about questions you may have about information you find about this disease.

View organizations that offer information on this specific type of cancer.