All types of eye cancer are rare. The most common types of eye cancer in adults are melanoma and lymphoma. The most common type in children is retinoblastoma.

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Some types of eye cancer have a good outlook and may be treatable without removal of your eye. But eye cancers that have spread to distant areas of your body can be life threatening. If it’s not diagnosed and treated early, eye cancer can lead to blindness.

Read on to learn more about the most common types of eye cancer, including their symptoms, treatment options, and risk factors.

Ocular melanoma is the most common type of tumor that can start in the eyes of adults. The most common type of ocular melanoma is uveal melanoma, which develops in about 6–8 people per million in Western countries each year.

In about half of people with uveal melanoma, the cancer spreads to distant tissues within 10–15 years. Almost all people with uveal melanoma that spreads will eventually die from this cancer.

Symptoms of ocular melanoma

Symptoms of ocular melanoma may not appear for many years. When symptoms happen, they may include:

Treatment options for ocular melanoma

Treatment options for ocular melanoma include:

  • a “watch and wait” approach to see how the cancer changes
  • radiation therapy (often internal radiation)
  • surgery to remove the affected tissue or your entire eye
  • laser and heat therapies

The Food and Drug Administration approved the medication tebentafusp-tebn (Kimmtrak) in 2022 to treat uveal melanoma that can’t be removed surgically in people with a specific mutation in the HLA gene.

Risk factors for ocular melanoma

Your chance of developing ocular melanoma is higher if you have:

  • light skin (white people are 8–10 times more likely than Black people to develop ocular melanoma)
  • an atypical mole on your skin
  • light eye color
  • a mole on your iris
  • freckles
  • a history of:
    • working in sunlight
    • tanning
    • taking part in other outdoor activities in sunlight

Lymphoma is a cancer that develops in a type of white blood cells called lymphocytes. Most lymphomas occur in lymph nodes, but about 30% develop outside your lymph nodes, spleen, or bone marrow.

Eye lymphoma is extremely rare, making up 1–2% of lymphomas outside the lymph nodes. Most cases are a subtype called non-Hodgkin’s lymphoma.

One 2023 study suggests that survival rates for lymphoma of the eye range from 54.3% to 96.9%, depending on the person’s age and ethnicity and the location of the tumor.

Symptoms of eye lymphoma

Possible symptoms include:

Treatment options for eye lymphoma

Treatment options for eye lymphoma include:

Risk factors for eye lymphoma

People with compromised immune systems have an increased risk of developing eye lymphoma. It usually develops in adults in their 40s or 50s and affects women about twice as often as men.

Retinoblastoma is the most common primary eye cancer in childhood. It starts in the retina.

Retinoblastoma tends to have a good outlook, with a survival rate of more than 95% in developed countries.

Symptoms of retinoblastoma

Symptoms of retinoblastoma can include:

  • leukocoria, which is a whitish pupillary reflex seen in 60% of people with retinoblastoma
  • crossed eyes (strabismus)
  • eye pain and redness
  • eye inflammation
  • a visible growth
  • decreased vision
  • trouble moving your eye

Treatment options for retinoblastoma

Treatment options for retinoblastoma include:

  • chemotherapy
  • radiation therapy
  • surgery to remove your eye
  • transpupillary thermal therapy
  • cryotherapy

Risk factors for retinoblastoma

The risk of developing retinoblastoma is highest in children who are under 3 years old and have a family history of this cancer. More research is needed to identify additional risk factors.

Medulloepithelioma is a cancer that usually develops on your eye’s ciliary body, which produces the fluid inside your eye. Up to 90% of tumors appear before age 10. If the affected eye is removed, this cancer has a 5-year survival of 90–95%.

Because this cancer is so rare, it’s not clear how many people it affects.

Symptoms of medulloepithelioma

Small tumors rarely cause symptoms. Usually, this cancer is not diagnosed until the tumor is large enough to be seen through the pupil. Symptoms might include:

  • vision loss
  • pupillary reflex
  • pain
  • a visible mass
  • eye redness

Treatment options for medulloepithelioma

Treatment options include:

  • eye removal (enucleation), which is standard treatment for large tumors
  • surgery to remove the tumor
  • cryotherapy
  • radiation therapy

Risk factors for medulloepithelioma

Medulloepithelioma seems to occur most often in people between the ages of 2 and 10 years.

Squamous cell carcinoma of the conjunctiva affects about 1 in 5 million people per year in the United Kingdom and 1 in 53,000 in Brisbane, Australia. It develops in the thin, clear membrane that covers your eye white and inner eyelid.

Symptoms of squamous cell carcinoma of the conjunctiva

Symptoms may include:

  • a fleshy mass or round lump
  • irritation
  • redness
  • a feeling that something is in your eye
  • itchiness
  • reduced visual acuity

Treatment options for squamous cell carcinoma of the conjunctiva

Treatment options include:

  • chemotherapy
  • immunotherapy
  • radiation therapy
  • surgery
  • cryotherapy
  • eye removal

Risk factors for squamous cell carcinoma of the conjunctiva

Risk factors include:

Other types of eye cancer include:

  • Metastatic eye cancer: This happens when cancer spreads to your eye from another part of your body.
  • Squamous cell carcinoma of the cornea: This cancer develops in the cells that cover your iris and pupil.
  • Malignant neoplasm of the caruncle: This cancer develops in the caruncle, the pink spot in your inner eye that produces oil.
  • Adenocarcinoma of the retinal pigment: This cancer develops in glandular cells that nourish your retina.

Your eye doctor can perform a comprehensive eye exam to look for structural problems with your eye. If they suspect cancer, they will likely send you to a specialist for additional testing.

You may undergo tests such as:

When to consult a doctor

It’s important to schedule an appointment with your eye doctor if you develop vision changes or any possible symptoms of eye cancer without a known cause. Your symptoms may be due to something else, but if you do have cancer, an early diagnosis gives you the best chance of preserving your vision.

What is the most common form of eye cancer?

The most common eye cancers in adults are melanoma and non-Hodgkin’s lymphoma. The most common types in children are retinoblastoma and medulloepithelioma.

What is an aggressive form of eye cancer?

The most common type of melanoma that can develop in your eye is uveal melanoma. A rare type called conjunctival melanoma tends to be very aggressive.

What are the first signs of eye cancer?

Some of the first symptoms of eye cancer are:

  • a lump on or around your eye
  • changes to your vision
  • a dark spot on your eye
  • eye bulging

All types of eye cancer are rare. The most common type in adults is melanoma, and the most common type in children is retinoblastoma.

Eye cancer doesn’t always cause symptoms, and when symptoms do appear, they usually have many potential causes. It’s important to visit your doctor if you notice any changes to your vision, bulging of your eye, or other potential warning signs of a serious eye problem.