Ocular Melanoma

Ocular melanoma is a type of melanoma, which is cancer that impacts the melanocytes. The melanocytes help the body produce pigment.

The eye is made up of three main layers of tissue, as detailed below.

The sclera: This is the outermost layer of the eye, sometimes called the white of the eye.

The uvea: This is the layer underneath the sclera. It’s made up of the iris, which is the colored part of the eye, the muscle around the iris and the choroid, which is another pigmented layer of tissue behind the retina.

The retina: This is the innermost layer of tissue in the eye. It’s the part of the eye that is connected to the brain and is essential to eyesight.

Ocular melanoma may occur in any of these layers of tissue if melanocytes begin to behave abnormally and form a tumor. In rare instances, it may also occur in the eye socket or other tissue surrounding the eyeball.

Some people with ocular melanoma, the most common type of eye cancer, don’t experience any symptoms. Others may not notice any symptoms before they are diagnosed with the condition. In many cases, the first person to see signs of eye cancer may be a patient’s eye doctor. Some of the more common symptoms of ocular melanoma include:

• Problems seeing out of one eye
• Changes to eyesight, including blurred vision, problems with peripheral vision or “seeing double”
• Dark spots on either the white of the eye (sclera) or the iris
• Seeing flashes of light
• Soreness or irritation in the eye
• A feeling of pressure inside the eye
• Seeing wavy lines or specks (sometimes called “floaters”) in the eye
• Changes in how the pupil or eyeball are shaped

Doctors don’t always know the exact reason a patient develops ocular melanoma. However, there are some factors that may increase the risk for developing ocular melanoma. These risk factors include:

• Being exposed to natural sunlight or other sources of ultraviolet light (like tanning beds) over long periods of time
• Having pale skin or a light eye color, like blue or green
• Having a family history of melanoma or a genetic condition that causes moles or unusual growths on the skin
• Having a mole on or around the eye
• Being over the age of 50

The first signs of ocular melanoma are often detected by a patient’s eye doctor during a routine eye exam. To fully and accurately diagnose the disease, patients may also undergo tests performed by doctors who specialize in cancers that affect the eyes, including those listed below.

An ultrasound of the eye: This may be used to create an image of the inside of the eyeball. This allows doctors to see the location and size of the cancer.

Optical coherence tomography (OCT): This is a type of imaging that scans the inside of the eye and produces highly detailed pictures. OCT may help determine a tumor’s size and location.

Fluorescein angiography: This is a procedure that uses a kind of dye to show whether there are any blockages or other issues with the blood vessels in the eye.

Fundus autofluorescence: This test uses an advanced camera to detect any signs of damage inside the eye, which may be signs of cancer.

A biopsy: This procedure may be recommended for patients with conjunctival melanoma. This involves removing the suspected tumor from the outside of the eye, after which it is examined in a lab.

A cancer’s stage refers to how large a tumor is and how far it has spread from its original location. Ocular melanoma may be diagnosed using the TNM system, which is the standard way to stage many types of cancer. This system measures the size of a tumor (T), whether it has spread to any lymph nodes (N) and whether it has spread, or metastasized (M), to other parts of the body.

Cancer doctors may also use the Collaborative Ocular Melanoma Study (COMS) system, which is much simpler, using only three stages, as listed below.

Small: This refers to any ocular melanoma that is 1-3 millimeters (mm tall and 5-16 mm wide.

Medium: This refers to any ocular melanoma that is 3.1-8 mm tall and less than 16 mm wide.

Large: This refers to any ocular melanoma that is more than 8 mm tall and more than 16 mm wide.

The most common treatments for ocular melanoma include radiation therapy and surgery. However, every patient — and every cancer diagnosis — is different. A patient’s cancer care team may recommend one or multiple treatment options, listed below, depending on the type of ocular melanoma a patient has, what size the tumor is and their overall health.

Watchful waiting (or observation): This may be recommended for very small ocular melanomas. This involves monitoring the tumor every 2 to 4 months for any changes in its size or how it appears.

Radiation therapy: This is sometimes used to destroy cancer cells or shrink a tumor, either on its own or in combination with other treatment options. Radiation therapy for ocular melanoma may be delivered from outside the body using powerful laser beams or from within the body, by placing a small disk (known as a plaque) containing radioactive material at the site of the tumor for a short period of time.

Surgery: This may be used to remove certain kinds of ocular melanoma. However, it poses some risk of complications, depending on where the cancer is located in the eye.

Laser therapy: This is an option that uses extreme heat to target and destroy cancer cells. It is most often used in combination with radiation therapy.

Drugs: Certain medications, primarily targeted therapies and immunotherapies, may be recommended for more advanced ocular melanoma or cancer that has spread to other parts of the body.

Who Treats Ocular Melanoma?

Patients with ocular melanoma are cared for by a multidisciplinary cancer care team with expertise in eye health, melanoma and a variety of advanced treatment options. This team may include:

• Melanoma experts, physicians who specialize in cancers that affect the melanocytes in the body — cells that are found in the skin, as well as some kinds of soft tissues like the eye
• Surgical oncologists with expertise in treating melanomas of the eye
• Radiation oncologists, who are doctors with specialist training in the latest external radiation techniques and internal radiation therapies like brachytherap
• Medical oncologists, who are experts in how to use cancer medicines including innovative immunotherapies
• Supportive care staff, who provide access to services that offer physical and emotional support during and after cancer treatment
• Trained oncology nurses, who care for patients during treatment
• Dermatologists with expertise in the early detection and management of melanoma, which can develop in other parts of the body such as the skin in patients with ocular melanoma

Ocular melanoma may spread, or metastasize, to other parts of the body. Metastatic ocular melanoma affects between 40% and 50% of people diagnosed with the condition. By far the most common place that ocular melanoma spreads to is the liver. It may also spread to the bones, lungs, skin or soft tissues in the body.

What Happens When Ocular Melanoma Spreads to the Liver?

If ocular melanoma has spread to the liver, a patient’s cancer care team will work with to create a treatment plan that is personalized to his or her unique diagnosis and health goals.

Studies have shown that surgery may be an effective way to treat ocular melanoma that has metastasized to the liver. A newer treatment option for patients whose ocular melanoma has spread to the liver but cannot be removed surgically is the Hepzato Kit^™, which allows doctors to deliver a particular chemotherapy drug directly to a tumor, with the goal of shrinking or destroying it.

Based on data from the National Cancer Institute’s Surveillance, Epidemiology, and End Results (SEER) Program, the overall five-year relative survival rate for ocular melanoma is 81%. This means that more than 8 out of 10 people diagnosed with ocular melanoma survive at least five years after their initial diagnosis, compared to people without that cancer type. However, survival rates differ based on how advanced the cancer is at diagnosis.

The five-year relative survival rate for localized ocular melanoma (which has not spread from its original location) is 85%.

The five-year relative survival rate for regional ocular melanoma (which has spread from its original location to nearby parts of the body) is 67%.

The five-year relative survival rate for distant ocular melanoma (which has spread to distant parts of the body) is 16%.

Patients should always talk to the care team to get a personalized prognosis based on such factors as their cancer stage, overall health and other factors. Early detection is dramatically correlated with improved survival.