Eye cancer can occur in any of the structures of the eye. It can start in the eye, called primary intraocular cancers, or result from the spread of cancer in another area of the body, known as secondary intraocular cancers.

Keep reading to learn more about eye cancer, including the causes, symptoms, treatment, and outlook.

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Eye cancer is found in any of the eye’s three major parts.

One part is the eyeball, which is filled with a jelly-like substance called the vitreous humor. The eyeball contains three layers:

  • sclera, which is the outer wall
  • uvea, which provides nourishment for the eye
  • retina, which sends information from the eye to the brain

The uvea is the central opening in the iris and contains the ciliary body, which controls the shape of the lens and focuses the eye, and the choroid, which contains pigmented cells and connective tissue.

Another part of the eye is the orbit. This refers to the tissues and bones surrounding the eyeball.

The third part consists of the adnexal structures, which include the eyelids and tear glands.

Scientists are studying the ways in which changes in the DNA inside genes cause eye cancer. DNA changes can turn on oncogenes, which help cancer cells grow and divide, or turn off tumor suppressor genes, which kill cancer cells or slow down growth. These changes allow healthy cells to grow uncontrollably and form a tumor.

Eye cancer is rare. The American Cancer Society estimates 3,320 new eye cancer cases in 2021 with 400 deaths.

The most common types of primary intraocular cancers vary between adults and children.

In adults, the most common type is melanoma. Intraocular melanoma starts in the pigmented cells that give the eye its color. Uveal melanoma accounts for 85% of all primary intraocular cancers, with 85–90% of tumors occurring in the choroid. Conjunctival melanoma is a less common type in adults and affects the thin, clear covering over the sclera.

The second most common type in adults is non-Hodgkin lymphoma (primary intraocular lymphoma). This usually only occurs in people with immune system conditions.

Orbital and adnexal are other, less common primary eye cancers in adults.

In children, the most common type of primary eye cancer is retinoblastoma. The second most common form is medulloepithelioma, but it is extremely rare.

Secondary intraocular cancers are more common than primary intraocular cancers. For instance, about 90% of ocular melanomas start in the skin. Other common sources of origin are the breasts or lungs. However, skin cancers can still be a primary cancer if they happen on the eyelid.

Many people with eye cancer may have no symptoms until the tumor is more advanced. Signs and symptoms that a person should report to a healthcare professional include:

  • blurry vision or sudden loss of vision
  • floaters, which are specks that move around in the field of vision, or flashes of light
  • visual field loss
  • a growing dark spot on the iris
  • change in size or shape of the pupil
  • change in position of the eyeball
  • bulging of the eye
  • change in the way the eye moves

There is rarely pain with eye cancer unless the tumor is large. These symptoms may be associated with other conditions and may not necessarily indicate cancer.

A healthcare professional may use the following tests to diagnose eye cancer:

  • ophthalmoscopy
  • ultrasound
  • fluorescein angiography

Some risk factors for eye cancer include:

  • Race: The risk of eye melanoma is higher in white people than in People of Color.
  • Eye color: The risk of uveal melanoma is higher in people with light colored eyes, such as blue or green.
  • Older age: Older people are more likely to develop eye cancer.
  • Certain inherited conditions: Conditions such as dysplastic nevus syndrome, oculodermal melanocytosis, and BAP1 cancer syndrome may increase a person’s risk.
  • Eye area moles or freckles: People with different types of moles or freckles on the skin or in the eye may be more likely to develop eye cancer.
  • Family history: Having immediate family with a history of eye cancer can also increase a person’s risk.

More research will help experts understand if certain factors like sunlight exposure, particular occupations, or skin melanoma cause eye cancer.

Experts do not know the exact cause of eye cancer. The risk factors for eye cancer are known, but people who have them are not able to change them.

Although there is no proven link between eye cancer and sunlight, the American Cancer Society recommends wearing UV-protected sunglasses when outside. The best type of sunglasses are wrap-around, with 99–100% UVA and UVB absorption.

Before treatment can begin, a doctor will determine the size of the tumor and whether it has spread to other parts of the eye, lymph nodes, or other parts of the body. This is called staging.

The doctor may also examine the cancer cells under a microscope to determine the type and how likely they are to respond to treatment.

If the tumor is small or slow-growing, the doctor may recommend watchful waiting. Treatment will begin if the tumor reaches a certain size or causes symptoms.

A treatment plan may involve combining several of the following:

  • Surgery: The type and extent of surgery will depend on the location and size of the tumor. The amount of tissue removal can range from just a small portion of the eye structure to complete removal of the eyeball (enucleation). Surgery may lead to loss of some or all vision in the eye.
  • Radiation: Different types of radiation therapy are available. Doctors focus high-energy X-ray beams from a machine close to the cancer, or they may insert a disk with radioactive seeds directly into the eye (brachytherapy or plaque therapy). Radiation is more common than surgery because it can preserve vision and appearance.
  • Laser therapy: Doctors use infrared light to heat and kill the tumor. Doctors typically use this method for small eye cancers because it can cause bleeding, retinal detachment, blockage of eye blood vessels, and eye cancer recurrence.
  • Chemotherapy: This is usually not the first-line treatment for eye cancer unless it has spread to other parts of the body.

Doctors can cure some eye cancers with treatment. In other cases, eye cancer is not treatable, but treatment may keep it from spreading and getting worse.

During follow-up visits, the healthcare team will monitor a person for symptoms and order tests to check for cancer recurrence or spread. They will help manage the side effects of ongoing treatment and ensure that vision remains as clear as possible.

There are no proven ways to prevent eye cancer from recurring or progressing. People can improve their overall health by adopting certain behaviors, such as:

  • not smoking
  • eating well
  • maintaining a healthy weight
  • exercising

The American Cancer Society estimates the 5-year survival rate for localized eye melanoma at 84%. If it metastasizes, or spreads, to distant areas of the body, the rate is 18%. Early detection is crucial for a good outcome.

Many types of cancer support groups are available. Some people may find one-on-one counseling helpful. Numerous resources exist for those who are living with cancer.

Eye cancer can start in any of the structures of the eye. Knowing the signs and symptoms of eye cancer will help people seek prompt diagnosis and treatment.

Several forms of treatment are available. The goal is to preserve vision, if possible, and prevent the cancer from spreading to other parts of the body.

The outlook for eye cancer is good, particularly if it is caught early.