What Oliver Sacks's Story Can Help Us Understand About Eye (Uveal) Melanoma

What he wrote, and what he said

Oliver Sacks was a neurologist and the author of Awakenings and The Man Who Mistook His Wife for a Hat. NPR reported that in a February 2015 opinion piece he announced that what had started as a melanoma in his eye had spread to his liver, and that he did not have long to live. He died on August 30, 2015, at 82.

In an interview published by TED, he described the diagnosis in his own words. "I was absolutely terrified with this melanoma at first. I didn't even know one could have ocular melanomas, let alone that they were much more benign than other sorts." He described the moment of diagnosis this way: "the surgeon brought out a model of an eye and he put in it something that looked like a little, shriveled, black cauliflower."

That is his public record. This page does not go past it.

A different cancer from skin melanoma

Melanoma of the uveal tract is rare, but NCI calls it the most common cancer that starts inside the adult eye.

The uvea is the pigmented middle layer of the eye. It has three parts: the iris at the front, the ciliary body behind it, and the choroid lining the back. Most uveal melanomas start in the choroid. Iris melanomas are least common, tend to be smaller and slower, and rarely spread.

The US incidence is about 4.3 new cases per million people a year, higher in men at 4.9 than in women at 3.7. The rate peaks near age 70. Our page on uveal melanoma covers diagnosis and care.

Risk factors NCI names are White race and ethnicity, light eye color, fair skin, and the ability to tan. Sunlight is the obvious suspect, and the evidence does not support it. NCI reports that studies of sun exposure have found only weak links or contradictory results.

Why it is usually silent

NCI states that most uveal melanomas are completely without symptoms at first.

As a tumor grows, what it does depends on where it sits. An iris melanoma can distort the pupil. A ciliary body melanoma can blur vision. A choroidal melanoma can sharply reduce vision, usually because the retina has detached behind it.

A small one cannot be told apart from a mole. NCI says small uveal lesions are watched for growth before melanoma is diagnosed at all. Features that raise suspicion include orange pigment on the surface, fluid under the retina, a thickness over 2 mm, and low internal reflectivity on ultrasound. In one series of 2,514 people with choroidal moles, 8.6% had become melanoma by five years and 17.3% by fifteen.

What treatment involves

For a medium choroidal melanoma, NCI says eye-sparing radiation is preferred for most people. That means plaque brachytherapy, a small radioactive disc stitched to the outside of the eye over the tumor, or an external beam. Other options include removing part of the eye wall, and adding laser or heat treatment to the plaque.

Enucleation, removal of the eye, remains standard for large choroidal melanomas, and for tumors causing severe glaucoma or growing into the optic nerve.

The liver problem

This is the part of Sacks's story that generalizes.

NCI reports that spread is present in only 2% to 3% of people when the eye tumor is first found. The uvea has a rich blood supply and no lymphatic drainage. So when the disease does spread, it travels in the bloodstream, not through lymph nodes. Sentinel node biopsy is not part of staging here.

And the first site is usually the liver. In the Collaborative Ocular Melanoma Study, among people who developed metastases, the liver was the only detectable site in 46%, and the liver plus other sites in a further 43%. Lung, bone, and tissue under the skin are the other common destinations.

That is why follow-up after eye treatment centers on the liver, and why years can pass between the eye and the liver. Our page on metastatic cancer explains what spread means generally.

The outlook

NCI names the strongest prognostic factors. Cell type, tumor size, where the front edge of the tumor sits, how much of the ciliary body is involved, and whether it has grown outside the eye. Spindle-A cell tumors carry the best outlook and epithelioid cell tumors the worst, though most are a mixture.

The five-year mortality after metastasis from ciliary body or choroidal melanoma is about 30%, compared with 2% to 3% for iris melanomas.

NCI's summary of treatment once the disease has spread is blunt. No effective systemic treatment has been identified, and trials are an option. One drug has since been approved in the United States. Tebentafusp is licensed for adults with unresectable or metastatic uveal melanoma who carry the HLA-A*02:01 tissue type. It carries a boxed warning for cytokine release syndrome, and in its trial 94% of participants had liver metastases.

These are group figures. They do not describe any one person.

When to get checked

There is no screening test for eye melanoma, and NCI has no evidence-based screening or prevention information for it. A routine dilated eye examination, where an optometrist or ophthalmologist looks at the back of the eye, is how many are found — often in someone with no symptoms at all.

Book an eye examination without waiting if you notice:

• A dark spot on the iris that is growing
• A change in the shape of the pupil
• Blurred vision in one eye that does not correct with glasses
• A shadow, curtain, or missing patch in one part of your vision

Most have other causes, including retinal detachment, which is itself an emergency. They all need an eye examined rather than a wait.

What this does not mean

• NPR reported the melanoma and the liver spread. Nothing about his stage, cell type, or treatment was made public here, and this page does not infer it.
• Eye melanoma is not skin melanoma. Both start in pigment cells, but the risk factors, spread pattern, and treatments differ.
• Sun exposure is a clear risk factor for skin melanoma. NCI says the evidence does not support it for uveal melanoma.
• The mortality figures above describe groups treated over past decades. They are not a forecast for anyone.

Sources

• NPR, Oliver Sacks, Renowned Neurologist And Author, Dies At 82 — https://www.npr.org/sections/thetwo-way/2015/08/30/436013382/oliver-sacks-renowned-neurologist-and-author-dies-at-82
• TED Blog, Remembering Oliver Sacks — https://blog.ted.com/remembering-oliver-sacks/
• NCI PDQ, Intraocular (Uveal) Melanoma Treatment (Health Professional Version) — https://www.cancer.gov/types/eye/hp/intraocular-melanoma-treatment-pdq
• DailyMed, KIMMTRAK (tebentafusp-tebn) prescribing information — https://dailymed.nlm.nih.gov/dailymed/services/v2/spls/24a49f57-d2fc-4ffe-9eb1-fe0460c6b067.xml

How this article was prepared

An AI-assisted editorial system helped prepare this page. No named medical reviewer has reviewed it unless one is listed.

The National Cancer Information Foundation publishes Cancer Explained. This page is for learning. It is not medical advice and does not suggest a test or treatment.

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