Understanding Eye Cancer: Types, Symptoms, Treatment

There are several different types of eye cancer. Let’s explore them now.

Intraocular melanoma

Intraocular melanoma is the most common type of eye cancer in adults. It begins in pigmented cells called melanocytes. You can develop intraocular melanoma in the following areas:

• Uvea: The uvea is a richly vascularized layer of tissue in your eye that includes the colored iris, the ciliary body, and the choroid. “Richly vascularized” means the tissue has an abundance of blood vessels. Intraocular melanoma typically occurs in the uvea, most often in the choroid.
• Conjunctiva: The conjunctiva is the moist tissue that covers the white of the eyeball and the inner part of your eyelids. Conjunctival melanoma usually arises from abnormal pigmented patches in the conjunctiva.

Even though intraocular melanoma is a common type of eye cancer, it’s uncommon overall. Melanoma most commonly affects the skin.

Intraocular lymphoma

Intraocular lymphoma is a rare type of lymphoma that affects the eye. The sites where this type of lymphoma is most likely to develop include the uvea and the vitreous humor inside of your eyeball.

In about 80% of people, intraocular lymphoma affects both eyes. Many people that develop this type of lymphoma also have lymphoma that’s affecting their brain, called central nervous system lymphoma.

Retinoblastoma

Retinoblastoma is a type of eye cancer that starts in the retina. The retina is the part of your eye that converts light into nerve impulses that your brain can use to make images. It can occur in one eye or both eyes.

Retinoblastoma is the most common type of eye cancer in children. It mainly affects children under the age of 5, although it can also happen in older children and adults in rare situations.

Intraocular medulloepithelioma

Medulloepithelioma is a rare type of eye cancer that’s more common in children. It’s most often diagnosed between the ages of 2 and 10, and it typically only affects one eye.

Medulloepithelioma starts in the ciliary body, which is a part of the uvea. A healthy ciliary body adjusts the shape of your eye’s lens and makes clear aqueous humor fluid that fills the front portion of the eye.

Squamous cell carcinoma

Squamous cell carcinoma can also affect the eye, although this is rare. When this type of cancer starts in the eye, it develops in the conjunctiva.

While it’s rare overall, squamous cell carcinoma is the most common type of cancer that affects the conjunctiva. Typically, it only occurs in one eye.

The symptoms of eye cancer can vary greatly based on the specific type of eye cancer. The table below helps to break down the potential symptoms of each type.

Generally speaking, eye cancer happens when cells in the eye begin to grow uncontrollably. This happens due to genetic changes that may be inherited from your parents or may be acquired during your lifetime.

The risk factors for each time of eye cancer are different. The table below can help to give you a better idea of who’s more at risk of each type of eye cancer.

The diagnostic process for eye cancer often starts with a visit to an eye doctor. After reviewing your medical history, the doctor will do an eye exam that includes the following:

• checking your vision
• evaluating the movement of your eyes
• using tools like an ophthalmoscope or a slit lamp to look at the inside of your eye

If the eye exam suggests eye cancer, additional imaging tests may be ordered to get a better look at your eyes and surrounding tissues. Tests may include:

• ultrasound, which uses sound waves to make images of the eye
• optical coherence tomography, which uses a noninvasive ultrasound device to provide a real-time, cross-sectional view of the retina
• fluorescein angiography, which uses a fluorescent dye to help a doctor look for abnormal blood vessel growth or other conditions affecting the eye
• CT scan, which makes cross-sectional images of your body by taking a series of X-rays
• MRI scan, which uses strong magnets and radio waves to create images

For some eye cancers, a biopsy is needed to confirm the diagnosis. This can be done painlessly with a thin hollow needle that draws out a sample of cancer cells (fine needle aspiration) or by surgically removing part of the tumor (incisional biopsy).

The treatment that’s recommended for eye cancer depends on many factors. These include:

• the specific type of eye cancer
• the stage of the cancer
• the size and location of the cancer
• whether one or both eyes are affected
• how likely it is that vision will be preserved
• your age and overall health
• your personal preference, or in the case of infants or young children, the preference of a parent or guardian

Some of the types of treatment that may be recommended include:

• Radiation therapy: Radiation therapy uses ionizing radiation to kill cancer cells. For eye cancer, it can be delivered in two ways:
  • Plaque therapy: A thin piece of irradiated metal is temporarily sewn to the eye overlaying the base of the tumor to precisely deliver a large dose of radiation.
  • External beam radiation: You receive directed radiation from a source outside of your body.
• Laser therapy: Laser therapy uses laser energy to kill cancer cells. It may also be used as an additional treatment after plaque therapy.
• Cryotherapy: Cryotherapy uses extreme cold to kill cancer cells.
• Chemotherapy: Chemotherapy uses drugs that disrupt the growth and division of cancer cells. Chemotherapy for eye cancer may be selectively administered to blood vessels using a guided catheter to enter the eye, or it may be slowly injected through the conventional intravenous (IV) route.
• Surgery: Surgery for eye cancer can involve removing only the tumor or the entire eye.

The outlook for a person with eye cancer can depend on many factors. One of these is the type of eye cancer that you have.

For example, the outlook for people with retinoblastoma is typically very good. Overall survival rates are often more than 95%.

There’s a poorer outlook for people with other types of eye cancer. For example, the overall 5-year relative survival rate for intraocular melanoma is 82%, based on people who received a diagnosis of melanoma of the eye in the United States between 2011 and 2017. Survival rates drop further if the tumor has already spread beyond the eye.

Research from 2022 found that the 5-year survival rate for intraocular lymphoma was 74.2%.

Other factors that affect eye cancer outlook include:

• the extent of the cancer, such as if it’s still localized to the eye or has spread outside of the eye
• the size of the tumor
• the tumor cell type and the classification of tumor tissue as seen under the microscope (which can help determine whether the tumor is likely to be benign or malignant)
• the parts of the eye affected by the cancer
• whether one or both eyes are affected
• your age and overall health

There are several types of eye cancer. Intraocular melanoma and retinoblastoma are the most common types of eye cancer in adults and children, respectively. However, eye cancer as a whole is still quite rare.

The outlook for eye cancer is generally best when it’s still small and is only in the eye. To get a better idea of your individual outlook, including whether your vision will be impacted, have an open conversation with a care team.

The symptoms of eye cancer can vary greatly based on the type of eye cancer. See an eye doctor if you develop concerning eye symptoms. They can do tests to help figure out what may be causing them.