Uveal Melanoma (Eye Cancer) Diagnosis & Care

This page is for people diagnosed with, or being checked for, uveal melanoma. It covers how the cancer is found, what genetic testing of the tumor tells you, treatment options, and follow-up care.

Uveal melanoma is a cancer of the pigment-making cells inside the eye. The uvea is the middle layer of the eye, and it has three parts. Where the tumor starts affects how it behaves.

• The iris, the colored ring at the front. Iris melanomas are usually small and slow-growing. People often notice them as a dark spot.
• The ciliary body, a ring of tissue behind the iris. Tumors here tend to be larger and more likely to spread.
• The choroid, the blood-vessel layer at the back. Most uveal melanomas begin here.

It is the most common cancer that starts inside the adult eye. It is still rare. The risk is higher in people with fair skin that burns easily, in people with light-colored eyes, and in older people.

It may not cause early symptoms, and it is sometimes found during a regular eye exam. NCI lists blurred vision or a dark spot on the iris among the possible signs.

NCI says a biopsy of the tumor is rarely needed to diagnose intraocular melanoma. Tests that examine the eye, including ultrasound and imaging of the retina and its blood vessels, are used instead.

If a sample is taken, it can be tested in two ways that predict the chance of spread.

Gene expression profiling reports the tumor as class 1 or class 2. Chromosome testing looks at chromosome 3, including loss of one copy (monosomy 3). NCI lists both among the tumor features worth recording for the outlook.

Ask your team what your result would mean for your risk of spread, your follow-up and any trials.

It is also information some people would rather not have. That is a reasonable position. It is worth deciding before the biopsy, not after.

The goal is to control the tumor and, where possible, keep the eye.

Plaque brachytherapy is the most common approach. A small dish holding radioactive seeds is stitched to the outside of the eye, over the tumor. It stays there for several days. A second short operation removes it. NCI says it is the most common form of radiation used for this cancer. In a large trial of medium-sized tumors, 85% of people treated this way kept their eye for 5 years or more.

Charged-particle radiation, such as proton beam, aims radiation from outside the eye. NCI says it can be better used than plaque brachytherapy for larger tumors and tumors close to the center of vision (fovea) or the optic nerve.

Enucleation means removing the eye. NCI says it is done if vision cannot be saved and the tumor is large, has spread to the optic nerve, or causes high pressure inside the eye. An artificial eye can be fitted afterward to match the other eye.

For medium-sized tumors, NCI says plaque brachytherapy gave the same survival as enucleation. So the choice turns on tumor size, location, and what can be saved. Vision in the treated eye can decline over the following years after radiation. That trade-off is worth discussing openly.

Uveal melanoma spreads through the bloodstream. NCI says the first site found is usually the liver.

NCI lists liver function blood tests and imaging such as ultrasound among the tests used to check for spread. Ask your team which checks you need and how often. These appointments can be anxious ones, and it is fair to tell your team that.

Treatment for metastatic uveal melanoma is genuinely difficult.

Tebentafusp was approved based on a randomized trial in which people lived longer than with other drugs (median 21.7 months against 16 months, per FDA). It is approved only for people who carry the HLA-A*02:01 tissue type, so ask whether this has been checked. Tebentafusp is given weekly. The label says the first three infusions are given in a healthcare setting, with monitoring for at least 16 hours afterward, because of a reaction called cytokine release syndrome.

Liver-directed treatments and clinical trials are also part of the conversation. Ask whether a trial is open to you.

• Call 911 or go to an emergency department if you are on tebentafusp and develop a temperature of 100.4°F (38°C) or higher together with light-headedness, trouble breathing, or a spreading rash. That combination can mean cytokine release syndrome, which the drug's label carries a boxed warning about. Do not drive yourself.
• Call 911 or go to an emergency department if you have sudden blurred vision with eye pain, especially if the eye is red, or you suddenly lose sight in an eye, even for a short time, or it feels like a curtain or shade is being drawn across your vision. MedlinePlus calls a red, painful eye with blurred vision a medical emergency. Vision loss after radiation usually comes on slowly over years, so a fast change is not part of the expected pattern. Do not drive yourself.
• Call your care team within a day or two if you notice new jaundice, ongoing pain under the right ribs, or unexplained weight loss between your liver surveillance scans. Report these rather than waiting for the next scheduled appointment.

• DailyMed label: KIMMTRAK (tebentafusp-tebn) — boxed warning on cytokine release syndrome
• FDA Drug Trials Snapshot: KIMMTRAK (tebentafusp-tebn)
• NCI drug information: tebentafusp-tebn
• NCI: Intraocular (Uveal) Melanoma Treatment (PDQ®) Health Professional Version
• MedlinePlus: Vision problems