Ocular cancer, or eye cancer, is a category of cancerous cells that start and grow in various part of the eye. Not only can these growths damage your vision, but they can also spread to other parts of the body.

The category includes several kinds of cancers: intraocular cancers, adnexal cancers, orbital cancers and secondary cancers.

Most eye cancers in adults are secondary or metastatic, meaning that they started elsewhere in the body before invading eye tissues.

Types of Eye Cancers

Intraocular cancers

Intraocular cancers start inside the eye and can affect tissues in these three parts of the eyeball:

  • Sclera: This is the fibrous tissue covering the eye and which gives the eyeball its white color.
  • Uvea: This is the layer of tissue in the middle of the eye under the sclera.
  • Retina: This is the light-sensitive tissue lining the back of your eyeball.

eyelash closeup

Adnexal cancers

Adnexal cancers mostly affect the eyelids, tear glands or other support structures around the eyeball.

Orbital cancers

These tumors develop in the tissues surrounding the eyeball or eye socket, such as muscles and nerves.

Secondary cancers

Cancerous eye growths are classified as secondary if they spread from other body organs, such as the breasts, to the eye. These tumors travel through the bloodstream and often end up in the choroid layer.

Types of Cancers in the Eye

Also called intraocular cancers, types of cancerous growths in the eye include:

  • Eye melanoma
  • The iris
  • The choroid
  • Ciliary body melanoma
  • Conjunctival melanoma
  • Lymphoma of the eye
  • Retinoblastoma

Eye Melanoma

Melanoma is the abnormal growth of color-pigment-producing cells in the skin. Eye melanoma occurs when these cancerous cells form in your eyeball.

While cancer can affect any eyeball tissue, most eye melanomas start in the uvea. These cancers may affect any of the following areas:

The Iris

Iris melanoma affects the front colored part of the eye surrounding the pupil. It’s usually smaller than the other intraocular melanomas and barely spreads to other body organs.

The Choroid

The choroid comprises connective tissue and blood vessels (capillaries) that nourish the eye. This part of the eyeball has the same type of melanin-producing cells as the skin, which is why most intraocular melanomas begin here.

Choroidal cancer has a higher probability of spreading to distant body organs compared to the other uveal melanomas.

Ciliary Body Melanoma

This subtype of uveal melanoma develops in the ciliary body, which adjusts the shape of the lens when you’re focusing.

Conjunctival Melanoma

Conjunctival melanoma is a rare type of intraocular cancer that affects the clear layer of tissue lining the eyeballs.

Lymphoma of the Eye

Also called primary intraocular lymphoma (PIOL), this tumor mostly affects people with a weak immune system. It can cause damage inside the eye in parts like the optic nerve or retina at the back.

Retinoblastoma

Retinoblastoma occurs when malignant nerve cells in the retina develop and multiply abnormally in the eye. This intraocular cancer affects young children and may be triggered by a genetic mutation.

Besides damaging the retina, the cancerous cells can spread through the blood stream to other body organs.

Symptoms and Early Detection

Eye melanoma has no early outward signs, so you may not initially realize you’re sick. However, eye exams can help detect any damage early on if you have this cancer.

Typical eye melanoma symptoms that you may experience in advanced stages of the condition include:

  • Small dark shapes appearing across your field of view (floaters)
  • Dark areas in your side vision
  • Hazy vision in one eye
  • A dark fleck on the iris
  • Your pupil may look different

Causes

Evidence points to erratic behavior of the pigment cells responsible for your skin and eye color. As per their DNA, these cells shouldn’t naturally multiply out of control.

A genetic mutation occurs, causing the cells to grow and form a melanoma in the eye. What triggers the DNA change is still an active area of research.

For some patients with melanoma of the conjunctiva, extended sun exposure may be a predisposing factor. Other people are born predisposed to eye cancer because they have blue or green eyes.

Stages of Eye Cancers

How far your eye cancer has advanced determines your best treatment options and the outlook. Staging is the system your doctor uses to figure this out after running the necessary tests.

Eye melanomas develop through four main stages. The first is the smallest and easiest to treat. (It is also the most difficult stage in which to find a cancer.) The last stage is the most advanced and severe.

Here are some of the scenarios your doctor will be evaluating depending on the stage of your eye tumor:

Stage I

Tumor forms in the choroid (size is category 1)

Stage II

  • Malignancy has grown to size category 2 and has invaded the ciliary body or
  • Malignancy has grown to size category 3 but hasn’t spread beyond the choroid layer

Stage III

Some possibilities early in stage IIIA are:

Initially, the growth has penetrated the white outer coating of the eyeball (sclera) and may have reached the ciliary body. The section of the tumor outside the eyeballs is 5 mm thick or smaller.

There are several stage IIIC scenarios, including:

  • The cancer is category 4 in size and has invaded the ciliary body. The section of the tumor outside the eyeball is not thicker than 5 mm or
  • Cancer formed outside the eyeball is thicker than 5 mm, regardless of its overall size

Stage IV

At this point, tumor size isn’t a key factor as the cancerous cells have travelled to other parts of the body. It may have spread to other eye structures separate from the original site.

Also, you have stage IV cancer if it’s spread from the eye to other body organs like the lungs or brain.

Diagnosing Eye Cancer

To get a conclusive report on your condition, you may require multiple eye cancer tests and exams. These will help your doctor determine the type and severity of eye damage.

Common diagnostic procedures for eye tumors include:

  • Ultrasound exams
  • Fluorescein angiography
  • Dilated pupil exams

Ultrasound Exam

This exam helps your doctor determine the size of your eye tumor. It begins with the administration of eye-numbing drops to make you comfortable.

Next, your doctor will gently place a special ultrasound-emitting device on your eye to look into the melanoma. The feedback the instrument receives is processed into images that help evaluate the tumor.

Fluorescein Angiography

If your cancer has caused any damage in the eye, fluorescein angiography may help reveal it. With this procedure, a dye is injected into your arm to circulate into blood vessels in your eye.

The dye will leak if there are blood vessel leaks in the eye. Circulation is restricted if any blood vessels are closed off. Technicians use a special camera to detect leaks or blockages.

Dilated Pupil Exams

Dilated pupil exams are standard procedures for most eye checkups. With your pupil enlarged, your doctor gets a clearer view of the inside of your eye.

Special instruments can be used to observe parts like the retina and the optic nerves. Common eye exams that involve dilating the pupil include:

Ophthalmoscopy

Your doctor will use a special magnifying lens to examine tissues at the back of your eye. The test can reveal problems affecting tissues like the optic nerve and retina.

Slit-Lamp Exam

Also known as biomicroscopy, a slit-lamp exam helps your doctor detect abnormalities inside your eye. The procedure combines a strong beam of light and microscope to help observe the eye in detail.

Your doctor may recommend multiple dilated pupil exams to closely track any changes in the size of your eye tumor over time. After a series eye tests, it’s easier to tell whether your growth is cancerous or harmless.

Lab Tests (Biopsies)

A biopsy is a standard eye cancer test performed in the lab. It involves removing a sample of cells from the growth.

The cells are tested for cancer in the lab.

Treatment

Intervention can begin once your doctor has seen where your tumor is and what damaged has occurred.

You may have one cancer treatment or a combination of treatments depending on your condition. The further the malignancy has advanced within the eye or beyond, the fewer are your options.

Types of eye cancer treatment include:

  • Surgery
  • Radiography
  • Laser therapy
  • Chemotherapy

Surgery

Surgeons have a set of options they can use to remove cancer from in and around the eye.

Resection

This method is a good option for cancerous growth in its early stages. It involves cutting the tumor out of your eye.

Eye Removal

Sometimes, cancer causes too much irreversible damage that eye removal is necessary to protect the patient. After the procedure, you may get a custom-fit prosthetic eye to boost your facial appearance.

Radiotherapy

Radiation energy, such as high-dose x-rays, can destroy cancerous cells or prevent them from multiplying. Like surgery, it’s a viable therapy for early-stage eye melanoma.

Laser Therapy

Photocoagulation is the most common type of laser therapy for eye cancer. With this treatment, your doctor uses special equipment to aim laser pulses at the tumor site in your eye.

The light beams “burn” and destroy eye tissues supplying the melanoma. Only a few pulses are required to eliminate small tumors.

Chemotherapy

Chemotherapy may be an option if you have conjunctival melanoma. In that scenario, your doctor will administer powerful eye drops to destroy cancerous cells on the surface of your eye.

Outcomes

Your care team can help you make informed decisions about cancer therapy by candidly discussing scenario outcomes. Statistically, treatment success rates are higher with melanomas that haven’t invaded outside of the eyeball.

The outlook gets worse as the cancer advances. It’s a lot different in stage four when multiple parts of the body require treatment.

As your doctor explains your choices and prognosis, you may ask them about possible effects on your vision. Invasive procedures like surgery can affect otherwise healthy eye tissue, damaging your vision.

Chemotherapy and laser therapy have potential side effects, too. Talk about these with your cancer care team to get the safest and most effective treatment plan possible.

What Doctor to See

You should see an eye doctor, specifically an ophthalmologist, if you have eye melanoma. Book an appointment right away once you experience vision changes like floaters or blind spots.

Your ophthalmologist will run the necessary eye tests to give you an accurate diagnosis. They’re the right specialist to see considering that ophthalmologic surgery is often the first-line treatment for eye melanomas.

References

Eye Cancer. (March 2020). American Academy of Ophthalmology.

Intraocular (Uveal) Melanoma Treatment (PDQ) – Patient Version. (July 2021). National Institutes of Health.

What Is Eye Lymphoma? (May 2021). American Academy of Ophthalmology.

What Is Ocular Melanoma? (January 2022). American Academy of Ophthalmology.

Eye Cancer Survival Rates. American Cancer Society.

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