What are the different types of rare eye cancer?

Share on Pinterest

According to the United Kingdom’s National Health Service (NHS), eye cancer is rare. It states that doctors in the U.K. diagnose around 850 cases per year.

In the United States, the American Cancer Society predicted that there would be about 3,490 new eye cancer cases in 2023.

Primary eye cancers, which begin in or around the eye, are rare. It is more common to have secondary eye cancer, which has spread to the eye from another area of the body.

All types of primary eye cancer are relatively rare. Types of eye cancer include:

• Intraocular melanoma: This is the most common type of eye cancer. Intraocular melanoma usually develops in the uvea, the middle layer of the eyeball. This is called uveal melanoma. Less commonly, melanoma develops in the conjunctiva of the eye. This is called conjunctival melanoma.
• Intraocular lymphoma: Intraocular lymphoma develops in the retina and the vitreous, which is the jelly-like substance in the center of the eye.
• Retinoblastoma: Retinoblastoma is the most common type of eye cancer affecting children, but it is still rare.
• Medulloepithelioma: Medulloepithelioma is an eye cancer that usually affects children ages 2–10 years.
• Primary acquired melanosis of the conjunctiva: This is the most common type of precancerous change in cells of the eye.
• Rhabdomyosarcoma: Rhabdomyosarcoma is a soft tissue cancer that can affect children. It may cause crossed eyes, bulging of the eyes, or vision problems.

Rare eye cancers that can affect the conjunctiva and eyelids include the following:

• squamous cell carcinoma
• basal cell carcinoma
• sebaceous carcinoma

Symptoms may vary depending on the type of eye cancer.

Ocular melanoma

Symptoms of ocular melanoma include:

• vision problems, such as blurry vision or sudden vision changes
• eye floaters
• partial loss of the field of sight
• a growing dark spot on the colored part of the eye
• an unusual change in pupil size or shape
• a change of position of the eyeball in the socket or changes in how the eye moves
• eye bulging

Intraocular lymphoma

Symptoms of intraocular lymphoma may affect one or both eyes and include:

• blurry vision
• loss of vision
• eye floaters
• swelling or redness in the eye
• increased sensitivity to light
• eye pain, in rare cases

Retinoblastoma

Symptoms of retinoblastoma include:

• a pupil that appears white when light shines on it
• the eyes appearing to look in different directions
• redness or swelling of the eye

The ACS states that a person’s risk of developing eye cancer increases with age. According to its statistics, eye cancer also appears to be more common in males and white people.

The American Academy of Ophthalmology suggests that the risk factors for intraocular lymphoma — the most common type of eye cancer — include:

• AIDS
• autoimmune disease, such as rheumatoid arthritis
• other medical conditions affecting the immune system
• medications that stop the body from rejecting an organ transplant

Genetics can also play a role in eye cancers. For example, it is possible to inherit a gene that causes retinoblastoma.

A doctor may use a variety of imaging techniques to diagnose eye cancer, such as:

• ultrasound
• optical coherence tomography, which involves using light waves to create images of the retina
• CT and MRI scans
• fluorescein angiography, which involves using a special dye to view the blood vessels in the eye

An eye doctor may also use certain instruments to examine the eye, such as:

• A slit lamp: This is a type of microscope that uses light to look at the inside of the eye.
• An ophthalmoscope: This instrument has a light and a magnifying lens to look at the back of the eye, which includes the retina and optic nerve.
• A gonioscope: This is a specialized lens that a doctor will place on the eye’s surface to check the front of the eye.

A doctor may also take a biopsy of part of the eye for examination under a microscope to confirm findings. This can help identify any atypical or cancerous cells.

Treatment for eye cancer may depend on various factors, such as:

• the location and size of the tumor
• if the cancer has spread
• if or how the tumor is growing
• any complications

Treatment options may include:

• Watchful waiting: If people have eye cancer with no symptoms, doctors may observe the condition to see if it progresses before starting treatment.
• Local resection: This involves surgery to remove the tumor and the small area of surrounding healthy tissue.
• Immunotherapy: Immunotherapy supports the immune system to destroy cancer cells. However, this is not a common treatment for eye cancer.
• Targeted therapy: In some cases, people may receive targeted drugs to treat eye cancer. Targeted therapy specifically identifies and destroys cancer cells.
• Chemotherapy: People may have chemotherapy alone or alongside radiation therapy to destroy cancer cells in the body.
• Radiation therapy: Radiation therapy is a common treatment for eye cancer. It uses high doses of radiation to destroy cancer cells.
• Enucleation: This surgical procedure removes the eyeball and a section of the optic nerve and replaces it with an artificial eye. This treatment is less common now than it once was.

A person’s outlook may depend on the type of eye cancer they have and its stage, as well as other factors such as their age and overall health.

The ACS provides the following 5-year relative survival rates for people with a diagnosis of ocular melanoma during the years 2012–2018:

Over 9 out of 10 cases of retinoblastoma in children in the U.S. will have a cure. However, an individual’s outlook may be less favorable if the cancer spreads beyond the eye.

People with extraocular lymphoma, which develops outside of the eyeball, may have a better outlook than those with intraocular lymphoma, which develops inside the eyeball.

Eye cancers are rare. Types of eye cancer include melanoma, lymphoma, and retinoblastoma. A doctor may diagnose eye cancer through various tests and scans that involve examining all areas of the eye.

Treatment may depend on the type and stage of eye cancer. Options may include radiation therapy, chemotherapy, or surgery.