Cancer starts when cells in the body begin to grow out of control. Cells in nearly any part of the body can become cancer, and can spread to other areas of the body. To learn more about how cancers start and spread, see What Is Cancer?
An eye cancer starts in the eye. There are different types of eye cancers. To understand eye cancers, it helps to know something about the parts of the eye and what they do.
The eye has 3 major parts: the eyeball (globe), the orbit, and the adnexal structures.
Doctors often use survival rates as a standard way of discussing a person’s prognosis (outlook). Some people with cancer may want to know the survival statistics for people in similar situations, while others may not find the numbers helpful or may even not want to know them. If you don’t want to know them, stop reading here and skip to the next section.
When discussing cancer survival statistics, doctors often use a number called the 5-year survival rate. The 5-year survival rate refers to the percentage of patients who live at least 5 years after their cancer is diagnosed. Of course, many people live much longer than 5 years (and many are cured).
To get 5-year survival rates, doctors have to look at people who were treated at least 5 years ago. Improvements in treatment since then may result in a better outlook for people now being diagnosed with this cancer. Five-year relative survival rates, such as the numbers below for eye melanoma, assume that some people will die of other causes and compare the observed survival with that expected for people without the cancer. This is a more accurate way to describe the outlook for patients with a particular type and stage of cancer.
Survival rates are often based on previous outcomes of large numbers of people who had the disease, but they can’t predict what will happen in any person’s case. Other factors can also affect a person’s outlook, such as the type of cells in the tumor, the patient’s age and general health, and how well the cancer responds to treatment. Your doctor knows your situation best and can tell you how the numbers below apply to you.
The numbers below come from the National Cancer Institute’s Surveillance, Epidemiology, and End Results (SEER) database, and are based on about 1,500 patients who were diagnosed with melanoma of the eye between 1988 and 2001.
Overall, about 3 out of 4 people with eye melanoma survive for at least 5 years. Survival rates tend to be better for earlier-stage than for later-stage cancers, but accurate survival rates for eye melanomas based on a specific stage are hard to determine because these cancers are fairly rare.
When the cancer is confined to the eye, the 5-year relative survival rate is about 80%. For people with eye melanomas that have spread to distant parts of the body, the 5-year relative survival rate is about 15%.
Because eye lymphoma is rare, accurate survival statistics for this cancer are hard to find. In one study of patients without HIV whose lymphoma was confined to the eye, about half of the patients were still alive 5 years after diagnosis. In many cases the lymphoma has already reached the brain by the time it is found, in which case the outlook is not as good.
Many medical centers around the world are doing research on the causes and treatment of eye cancers. These are challenging diseases to study because they are not common. But each year scientists find out more about what causes them and how to improve treatment.
Learning more about the gene changes that make eye cancer cells different from normal cells will likely play an important role in treating eye melanomas, lymphomas, and other eye cancers in the future.
As we learn about the gene changes in these cancers, we may be able to develop tests to identify people who are more likely to get them and then carefully screen those people.
For example, in recent years, researchers have found that some families have a change (mutation) in the BAP1 gene that makes them more likely to develop melanoma of the eye. While this gene change affects only a small portion of people with eye melanoma, researchers might be able to study it to learn more about how eye melanomas develop.
The genetic changes in tumors may also help predict the likelihood of them spreading. For example, in uveal melanoma, certain genetic changes, such as the loss of one copy of chromosome 3, have been linked to an increased risk of cancer spread.
Recently, researchers have found that patterns of gene expression in tumor cells appear to be an even better way to tell if an eye melanoma is likely to spread. Based on these gene patterns, a little more than half of eye melanomas are shown to be “Class 1” tumors. These cancers have a low risk of spreading. The remaining eye melanomas fall into the “Class 2” category, which have a very high risk of spreading.
Some doctors now offer a test (DecisionDx-UM) for these gene changes, and some patients may want to have them to learn if their cancer is likely to spread. If a patient is found to be at high risk, the doctor might follow them more closely to try to detect cancer spread as early as possible. But other doctors are not as keen on using the test at this time, because we don’t yet have proven ways to prevent the cancer spread or alter the outcome in people who are in the high risk group.
Identifying gene changes in eye cancer cells might also provide specific targets for newer drugs. For example, most eye melanomas have changes in either of 2 related genes, GNAQ or GNA11. The proteins made by these genes are part of the MAPK signaling pathway inside cells that helps them grow. It’s not yet clear if drugs will be able to target these proteins directly, but drugs that target other proteins in the MAPK pathway are now being studied for use against eye melanomas, and some have shown early promising results (see Targeted therapy below).
Immunotherapies are treatments that boost the body’s immune system to try to get it to attack the cancer. Cytokines, monoclonal antibodies, cancer vaccines, and other immunotherapies are among the most promising approaches for treating melanoma and lymphoma. Although most clinical trials of these treatments include people with melanomas of the skin and lymphomas that begin in lymph nodes, results of these studies might help treat people with eye melanomas and lymphomas as well.
One example is ipilimumab (Yervoy), a type of drug called a monoclonal antibody that boosts the overall activity of the immune system. This has been shown to help some people with advanced melanomas of the skin live longer, although it can also have some serious side effects. Some doctors now use it to treat melanomas of the eye as well, although its benefits against this cancer are still being studied in clinical trials.
Newer drugs such as nivolumab and pembrolizumab (Keytruda), which boost the immune response against cancer cells in a slightly different way, have shown even better results against skin melanomas in early studies. These drugs might prove to be useful against eye melanomas as well.
As researchers have learned more about some of the changes in cells that cause them to become cancer, they have begun to develop drugs that target these changes. These new targeted drugs work differently from standard chemo drugs. They might work in some cases when chemo drugs don’t, and they tend to have different (and often less severe) side effects.
Most eye melanomas have changes in the GNAQ or GNA11 genes. Proteins made by these genes are part of the MAPK gene signaling pathway that helps cells grow. Selumetinib is a drug that targets the MEK protein, which is also part of the MAPK pathway. Selumetinib has been shown to slow the growth of advanced eye melanomas in a clinical trial. While it does not cure these cancers, it often shrinks them for a time. For now, this drug is only available through clinical trials.
Other drugs might also be useful in treating cancers with these gene mutations. For example, some early research suggests that sotrastaurin (AEB071), a drug that targets protein kinase C, might be effective against cells with a GNAQ mutation. This is now being studied in clinical trials.
Some newer drugs, such as vemurafenib (Zelboraf®), dabrafenib (Tafinlar®), and trametinib (MekinistTM), target cells with a mutation in the BRAF gene. This mutation is found in about half of patients with skin melanoma, but only in about 5% of patients with eye melanoma. Still, these or similar drugs might help people whose cancer cells have these mutations.
Many targeted drugs are already used to treat other types of cancer. Some of them are now being studied for use against melanoma of the eye as well, including sunitinib (Sutent®), sorafenib (Nexavar®), vorinostat (Zolinza®), and everolimus (Afinitor®).
Other drugs target the blood vessels that tumors need to grow. These are known as anti-angiogenesis drugs. One example is bevacizumab (Avastin®), which is already used to treat some other types of cancer. It may help prevent some radiation side effects, which might help people retain more vision after treatment. This drug is also being studied for use along with chemotherapy in people with advanced eye melanomas.
After someone is diagnosed with eye cancer, doctors will try to figure out if it has spread, and if so, how far. This process is called staging. The stage of a cancer describes how much cancer is in the body. It helps determine how serious the cancer is and how best to treat it. Doctors also use a cancer's stage when talking about survival statistics.
The cancer stage is determined from the results of eye exams, imaging tests (ultrasound, CT or MRI scan, etc.) and other tests, which are described in How Is Melanoma of the Eye Diagnosed? and How Is Lymphoma of the Eye Diagnosed?
A staging system is a standard way for the cancer care team to describe how far a cancer has spread. The most common systems used to describe the stages of eye melanomas are the American Joint Committee on Cancer (AJCC) TNM system and the system used by the Collaborative Ocular Melanoma Study (COMS) group.
Most eye melanomas start in the uvea, which includes the iris, ciliary body, and choroid (see What Is Eye Cancer?). The system below is for these uveal melanomas.
Less often, melanomas can start in other areas in or around the eye, some of which have their own staging systems. Talk to your doctor to learn more about your stage if you have a less common type of eye melanoma.
The system described below is the most recent AJCC system, effective January 2018.
The TNM system is based on 3 key pieces of information:
Numbers or letters appear after T, N, and M to provide more details about each of these factors. Higher numbers or letters mean the cancer is more advanced.
The T categories for iris melanomas are different from the T categories for ciliary body and choroidal melanomas. But the N and M categories are the same for melanomas in all 3 parts of the uvea.
TX: The primary tumor cannot be assessed; information not known.
T0: No evidence of a primary tumor.
T1: Tumor is only in the iris.
T2: Tumor has grown into the ciliary body or choroid (or both).
T3: Tumor has grown into the ciliary body and/or choroid and into the sclera.
T4: Tumor extends outside the eyeball.
Ciliary body and choroidal melanomas are divided into 4 main T categories (T1 to T4), based on the diameter (width) and the thickness of the tumor. T1 tumors are the smallest; T4 tumors are the largest. Each of these categories is then broken down further, based on how far the tumor has grown.
TX: The primary tumor cannot be assessed; information not known.
T0: No evidence of a primary tumor.
T1 tumors:
T2 tumors:
T3 tumors:
T4 tumors:
T4e: The tumor can be any size. It is growing outside the eyeball and the part of the tumor that is outside the eyeball is greater than 5 mm across.
NX: Lymph nodes cannot be assessed.
N0: Cancer has not spread to nearby lymph nodes.
N1: Cancer has spread to nearby lymph nodes, or it has spread as small cancer deposits in other parts of the eye.
M0: Cancer has not spread to distant parts of the body.
M1: Cancer has spread to distant parts of the body.
To assign an overall stage, the T, N, and M categories are combined in a process called stage grouping. The stages are described by Roman numerals from I (the least advanced) to IV (the most advanced). Some stages are further divided with letters.
Stage I |
T1a, N0, M0 |
Stage IIA |
T1b to T1d, N0, M0 |
Stage IIB |
T2b or T3a, N0, M0 |
Stage IIIA |
T2c or T2d, N0, M0 |
Stage IIIB |
T3d, N0, M0 |
Stage IIIC |
T4d or T4e, N0, M0 |
Stage IV |
Any T, N1, M0 |
This staging system for uveal melanoma can be very complex. If you’re interested in learning more about it and how it might apply to your cancer, ask your doctor to explain it to you in a way you understand.
The TNM system is very detailed, but in practice many doctors use the simpler staging system devised by the COMS group, which has done most of the clinical research on how to treat intraocular melanoma. This system divides eye melanomas into small, medium, and large:
Intraocular lymphoma does not have its own staging system. These cancers may be staged using the system for other non-Hodgkin lymphomas, which is described in Non-Hodgkin Lymphoma Stages.
Unlike eye melanomas, the size of the tumor is usually not a major factor in determining the treatment options for eye lymphomas. Instead, treatment options are generally based on the type of lymphoma, as well as on whether the lymphoma is limited to the eye or is also in other areas of the body.
The American Cancer Society’s estimates for eye cancer in the United States for 2018 are:
Primary eye cancers can occur at any age, but the risk for most types increases as people get older. The rate of eye melanomas has been fairly stable over the past few decades. Cancers that spread to the eye from another part of the body (secondary eye cancers) are actually more common than primary eye cancers.
Most cancers of the eye and orbit in adults are melanomas, with lymphomas being the next most common. Both of these cancers start more often in other parts of the body. More than 9 out of 10 melanomas start in the skin, while most lymphomas begin in lymph nodes.
For statistics on survival, see Eye cancer survival rates.
Visit the American Cancer Society’s Cancer Statistics Center for more key statistics.
A risk factor is anything that affects your chance of getting a disease such as cancer. Different cancers have different risk factors. Some risk factors, like smoking, can be changed. Others, like a person’s age or family history, can’t be changed.
But having a known risk factor, or even several risk factors, does not mean that you will get the disease. And many people who get the disease may have few or no known risk factors.
The risk of intraocular melanoma is much higher in whites than in African Americans or Asian Americans.
People with light colored eyes are somewhat more likely to develop melanoma of the eye than are people with brown eyes.
Eye melanomas can occur at any age, but the risk goes up as people get older. Eye melanoma is slightly more common in men than in women.
People with dysplastic nevus syndrome, who have many abnormal moles on the skin, are at increased risk of skin melanoma. They also seem to have a higher risk of developing melanoma of the eye.
People with abnormal brown spots on the uvea (known as oculodermal melanocytosis or nevus of Ota) also have an increased risk of developing eye melanoma.
BAP1 cancer syndrome is a rare inherited condition in which family members are at increased risk for eye melanoma, as well as melanoma of the skin and some other cancers. This condition is caused by an inherited mutation (change) in the BAP1 gene.
Eye melanomas can run in some families who do not have these conditions, but this is very rare.
Sun exposure: Too much exposure to sunlight (or sunlamps), a known risk factor for melanoma of the skin, has also been proposed as a possible risk factor for melanoma of the eye, but this has not been proven.
Certain occupations: Some studies have suggested that welders, farmers, fishermen, chemical workers, and laundry workers may have a higher risk of eye melanoma, but none of these links has been proven conclusively.
The only known risk factor for primary lymphoma of the eye is having a weakened immune system. Examples include people with AIDS and people who take anti-rejection drugs after organ or tissue transplants.
The exact cause of most eye cancers is not known. But scientists have found that the disease is linked with some other conditions, which are described in “ What are the risk factors for eye cancer?” A great deal of research is being done to learn more about the causes.
Scientists are learning how certain changes in the DNA inside cells can cause the cells to become cancerous. DNA is the chemical in each of our cells that makes up our genes, the instructions for how our cells function. We usually look like our parents because they are the source of our DNA. But DNA can also influence our risk for developing certain diseases, such as some kinds of cancer.
Some genes control when our cells grow, divide into new cells, and die. Genes that help cells grow, divide, or stay alive are called oncogenes. Genes that slow down cell division or cause cells to die at the right time are called tumor suppressor genes. Cancers can be caused by DNA changes that turn on oncogenes or turn off tumor suppressor genes.
Some people with cancer have DNA changes they inherited from a parent that increase their risk for the disease. For example, some people inherit a change (mutation) in the BAP1 tumor suppressor gene, which increases their risk of eye melanoma and some other cancers. When the BAP1 gene is mutated, it doesn’t work normally, which can allow cells with this change to grow out of control.
Most DNA changes linked to cancer are acquired during life rather than inherited before birth. For example, recent research has shown that about 4 out of 5 eye melanomas have changes in either of 2 related genes, GNA11 or GNAQ, which appear to be oncogenes. Other, as of yet unknown, gene changes are probably needed for these cancers to develop as well.
Scientists are studying these and other DNA changes to learn more about them and how they might lead to eye cancer. But it is still not exactly clear what causes these changes to occur in some people and not others.
We do not yet know what causes most cancers of the eye, so it is not yet possible to prevent them.
We know there is a link between sunlight and melanomas of the skin, and there are things you can do that might reduce your risk of these cancers, including limiting your exposure to intense sunlight, covering up with protective hats and clothing, and using sunscreen.
The American Cancer Society also recommends wearing UV-protected sunglasses when outside in strong sunlight. Wrap-around sunglasses with 99% to 100% UVA and UVB absorption provide the best protection for the eyes and the surrounding skin. This might help reduce the risk of developing cancers of the skin around the eyes. The link between sunlight and eye melanomas is not proven, but some doctors think that sunglasses might also reduce eye melanoma risk.
Many people with eye lymphoma have no clear risk factors for this disease. For now, the best way to limit the risk of eye lymphoma is to try to avoid infection with HIV, the virus that causes AIDS.
Eye cancer is uncommon, and there are no widely recommended screening tests for this cancer in people at average risk. (Screening is testing for a disease like cancer in people without any symptoms.) Still, some eye cancers can be found early.
Some doctors may recommend yearly eye exams for those at higher risk of eye melanoma, such as people with dysplastic nevus syndrome. Regular eye exams are an important part of everyone’s health care, even if they have no symptoms. Often melanomas of the eye are found during a routine eye exam. When the doctor looks through the pupil at the back of the eye, he or she may see a dark spot that might be an early melanoma.
Many doctors feel that most melanomas start from a nevus (mole), which is a benign (non-cancerous) tumor of pigment cells. If an eye nevus is present, it should be looked at regularly by an ophthalmologist (a doctor who specializes in eye diseases). People who notice a dark spot on the colored part of their eye (the iris) should have a doctor look at it, especially if it is getting bigger.
If eye cancer keeps growing or comes back after one kind of treatment, it may be possible to try another treatment plan that might still cure the cancer, or at least keep it under control enough to help you live longer and feel better. Clinical trials also might offer chances to try newer treatments that could be helpful. But when a person has tried many different treatments and the cancer is still growing, even newer treatments might no longer be helpful. If this happens, it’s important to weigh the possible limited benefits of trying a new treatment against the possible downsides, including treatment side effects. Everyone has their own way of looking at this.
This is likely to be the hardest part of your battle with cancer – when you have been through many treatments and nothing’s working anymore. Your doctor might offer you new options, but at some point you may need to consider that treatment is not likely to improve your health or change your outcome or survival.
If you want to continue to get treatment for as long as you can, you need to think about the odds of treatment having any benefit and how this compares to the possible risks and side effects. Your doctor can estimate how likely it is the cancer will respond to treatment you’re considering. For instance, the doctor may say that more treatment might have about a 1 in 100 chance of working. Some people are still tempted to try this. But it is important to have realistic expectations if you do choose this plan.
No matter what you decide to do, it’s important that you feel as good as you can. Make sure you are asking for and getting treatment for any symptoms you might have, such as nausea or pain. This type of treatment is called palliative care.
Palliative care helps relieve symptoms, but it is not expected to cure the disease. It can be given along with cancer treatment, or can even be cancer treatment. The difference is its purpose – the main goal of palliative care is to improve the quality of your life, or help you feel as good as you can for as long as you can. Sometimes this means using drugs to help with symptoms like pain or nausea. Sometimes, though, the treatments used to control your symptoms are the same as those used to treat cancer. For instance, radiation might be used to help relieve pain caused by a large tumor. Or chemo might be used to help shrink a tumor and keep it from blocking the bowels. But this is not the same as treatment to try to cure the cancer.
At some point, you may benefit from hospice care. This is special care that treats the person rather than the disease; it focuses on quality rather than length of life. Most of the time, it is given at home. Your cancer may be causing problems that need to be managed, and hospice focuses on your comfort. You should know that while getting hospice care often means the end of treatments such as chemo and radiation, it doesn’t mean you can’t have treatment for the problems caused by your cancer or other health conditions. In hospice the focus of your care is on living life as fully as possible and feeling as well as you can at this difficult time. You can learn more about hospice in Hospice Care.
Staying hopeful is important, too. Your hope for a cure may not be as bright, but there is still hope for good times with family and friends — times that are filled with happiness and meaning. Pausing at this time in your cancer treatment gives you a chance to refocus on the most important things in your life. Now is the time to do some things you’ve always wanted to do and to stop doing the things you no longer want to do. Though the cancer may be beyond your control, there are still choices you can make.
You can learn more about the changes that occur when treatment to cure the cancer stops working, and about planning ahead for yourself and your family, in Nearing the End of Life and Advance Directives.
For many people with eye cancer, treatment can remove or destroy the cancer. Completing treatment can be both stressful and exciting. You may be relieved to finish treatment, but find it hard not to worry about cancer the growing or coming back. (When cancer comes back after treatment, it is called a recurrence.) This is a very common concern in people who have had cancer.
It may take a while before your fears lessen. But it may help to know that many cancer survivors have learned to accept this uncertainty and are living full lives. See Living With Uncertainty: The Fear of Cancer Recurrence for more about this.
For other people, the eye cancer may never go away completely. These people might get regular treatments with chemotherapy, radiation therapy, or other therapies to help keep the cancer in check for as long as possible. Learning to live with cancer as a more of a chronic disease can be difficult and very stressful. It has its own type of uncertainty. See When Cancer Doesn’t Go Away for more about this.
If you have completed treatment, your doctors will still want to watch you closely. It’s very important to keep all follow-up appointments. During these visits, your doctors will ask about symptoms, examine you, and may order certain tests.
Follow-up is needed to check for cancer recurrence or spread, as well as possible side effects of certain treatments. This is a good time for you to ask your health care team any questions you need answered and to discuss any concerns you might have.
Almost any cancer treatment can have side effects. Some might last for a few weeks or months, but others can last the rest of your life. Don’t hesitate to tell your cancer care team about any symptoms or side effects that bother you so they can help you manage them.
Your doctor will most likely want to see you fairly often (every couple of months or so) at first. The time between visits may get longer if you are not having any problems. During these doctor visits, you might get:
If cancer does recur at some point, further treatment will depend on where the cancer is, what treatments you’ve had before, and your health. For more information on how recurrent cancer is treated, see “ Treating uveal (eye) melanoma by location and size” For more general information on dealing with a recurrence, see When Your Cancer Comes Back: Cancer Recurrence.
Treatments for eye cancers such as surgery, radiation therapy, and laser therapy can cause side effects. Your doctors will check your treated eye for complications and may recommend medicines or operations to help control side effects and help to keep your vision as clear as possible. For example, radiation therapy might cause cataracts to form or injure muscles around the eye, resulting in blurred or double vision. In either case, surgery may help with these problems.
Follow-up exams and tests are also important for people who have had an eye removed, because melanomas can still sometimes recur in the area around the eye or in distant parts of the body.
Physical exams are usually done about every 3 months for the first few years after treatment. Other tests might include lumbar punctures (spinal taps) to look for lymphoma cells in the cerebrospinal fluid and MRI scans of the brain to look for recurrence or metastasis.
At some point after your treatment, you might be seeing a new doctor who doesn’t know about your medical history. It’s important to be able to give the details of your diagnosis and treatment. Gathering these details during or soon after treatment may be easier than trying to get them at some point in the future. Make sure you have this information handy (and always keep copies for yourself):
It is also very important to keep health insurance. Tests and doctor visits cost a lot, and even though no one wants to think of their cancer coming back, this could happen.