Understanding Ocular Melanoma
- Australian model Tilly Jac Smith, 32, was diagnosed with ocular melanoma after sudden light sensitivity led doctors to discover eye cancer she initially mistook for “surfer’s eye,” prompting her to urge others to include eye checks in their cancer screenings.
- AIM at Melanoma describes ocular melanoma as a rare cancer arising in the eye that, despite developing from the same melanocyte cells as skin melanoma, is considered a distinct disease — and because most cases originate in the uvea [the pigmented middle layer of the eyeball], the term is often used interchangeably with “uveal melanoma.”
- According to the Melanoma Research Foundation, primary ocular melanoma is treated mainly with radiation or surgery. If the cancer spreads (usually to the liver), tebentafusp-tebn (Kimmtrak)—FDA-approved since January 2022 for HLA-A*02:01-positive patients— is the only approved drug.
- For metastatic ocular melanoma, other treatment options may include liver-focused therapies, focal treatments on individual liver tumors, and/or clinical trials.
- If you have a cancer screening coming up or have recently had one, you may have questions you want answered. SurvivorNet’s proprietary AI tool “My Health Questions” is designed specifically for patients and caregivers.
AIM at Melanoma Foundation describes ocular melanoma as a rare form of melanoma that occurs in the eye, further explaining, “Though it develops from melanocytes, the same cell that causes melanoma in the skin, it should be considered a separate cancer from skin melanoma.”
Read MoreIn Smith’s case, the content creator and travel enthusiast shared her diagnosis with her followers on Instagram, explaining what happened and the symptoms that led to her discovery.
She shared the update in a video clip of herself, writing alongside it, “There’s no such thing as finding it too early. Your reminder to get your skin checked.
“Surgery was today, everything went smoothly and I’m healing. Very lucky to have my village.”
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Smith explained in the footage, “Good morning, everybody. We’re home from holidays and back into the usual routine. … I’m about to go and soak up the last of the ocean before surgery tomorrow. If you weren’t aware, because I wasn’t, you can get skin cancer or melanoma on your eyeball.
“I get my skin checked often for skin cancers, but I’ve never, ever checked my eyeballs. But when we got back from Maldives, I went and got my eyes checked because they were all of a sudden super sensitive to light and what I thought was a trugem seemed to have grown really quickly.”
She continued, “I was sent to a specialist on Friday who diagnosed me with ocular cancer. So I needed to let you know, when you get your skin checked next, if you do it yearly or however often, please get your eyes checked because I had no idea that that was a possibility, and here we are.”
Expert Melanoma Resources
- Advances in Uveal Melanoma Treatment: Immunocore’s Chief Medical Officer On Their New Standards For Survival
- For The First Time, A Potential Treatment Option For All Uveal Melanoma Patients
- What is Uveal Melanoma?
- Am I at High Risk for Melanoma?
- Atypical Moles Don’t Necessarily Mean You Have Melanoma
- Blood Test Could Predict the Best Type of Treatment for Metastatic Melanoma
- The Biopsy Helps Identify Staging and Treatment for Melanoma
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We’re glad to see Smith encouraging others to be aware of melanoma symptoms and seek medical advice when something seems off with your body.
It’s also heartwarming to see Smith is going through her cancer journey with her beloved husband, film director Zach Rogers.
She wrote in a recent post, celebrating their anniversary, “My darling man. 2 years with you. You have shown me what it means to truly love. Thank you for being my rock, for holding me, and for endlessly loving me the way you do. Thank you for being my safe place. And for loving every corner of who I am.
“Thank you for bringing out our childhood joy, And making me laugh with tears daily. Thank you for reminding me of my softness, While also showing me how strong I am. Thank you for being you Z, I’m so proud of you. You inspire me everyday.”
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Understanding Ocular Melanoma
Many people may not know that cancer can develop in the eye, which is why maintaining good eye health and getting regular checkups is an important part of overall wellness.
According to AIM at Melanoma Foundation, ocular melanoma is a rare eye cancer that develops in pigment-producing melanocytes and is separate from skin melanoma.
“Ocular melanoma most often affects the middle layer of your eye (the uvea), which includes the colored portion (the iris), the muscle fibers around the lens (ciliary body), and the layer of blood vessels that lines the back of the eye (choroid),” AIM at Melanoma explains.
Melanoma can also occur in the conjunctiva (the clear membrane covering the eye’s white part and inner eyelids). Melanoma in the choroid, iris, or ciliary body is called uveal melanoma; in the conjunctiva, it’s called conjunctival melanoma, the foundation adds.
Though both arise in the eye, these two types differ biologically and clinically from each other—and from skin melanoma. Since uveal melanoma makes up most ocular melanoma cases, “ocular melanoma” is sometimes used to mean uveal melanoma specifically.
For The First Time, A Potential Treatment Option For All Uveal Melanoma Patients
“Both types of ocular melanoma are rare. Uveal melanoma is diagnosed in about 2,500 people each year in the U.S.—but it is the most common primary cancer of the eye in adults,” says AIM at Melanoma.
Ocular melanoma most commonly develops around age 60, however, it can affect people of any age. Conjunctival melanoma is even rarer, occurring in fewer than 1 case per million people.
It’s important to understand that unlike melanoma of the skin, uveal melanoma is not caused by UV rays from the sun … the cause of this disease is still being studied by researchers.
Possible risk factors for uveal melanoma include:
- Light skin and eye color
- Strong family history of cancers
- Personal history of cancer
- Certain mutations that cause familial uveal melanoma, such as BAP1, PALB-2, MBD4, or NF-1
- Choroidal nevus, a type of lesion in the back of the eye
- Ocular melanocytosis, another type of lesion in the eye
Even though the disease is rare, Dr. Patel recommends getting regular check-ups to monitor for uveal melanoma.
“Everyone should get dilated every year,” she told SurvivorNet. “Even if you don’t need corrective lenses or glasses, contact lenses … it’s still important to have your eyes dilated and examined for physical changes such as melanoma or moles in the eye.”
If you do have any of the risk factors above or notice any changes in your vision, be sure to see an ophthalmologist as soon as possible. Early detection is key in treating any cancer, including uveal melanoma.
Treating Ocular Melanoma
According to the Melanoma Research Foundation, treatment for a primary tumor (when the cancer is confined to the eye) may consist of:
- Radiation — the recommended first-line treatment for most small and medium-sized tumors
- Surgery: enucleation (full eye removal) for very large or advanced tumors; iridectomy or iridocyclectomy for iris-only tumors; trans-scleral local resection (removing the tumor through an opening in the eye wall) for select large tumors
- Other treatments sometimes used for ocular melanoma include thermotherapy, cryotherapy, and Gamma Knife radiosurgery
If the disease becomes metastatic (most often spreading to the liver), tebentafusp-tebn (Kimmtrak) is the only FDA-approved drug for this cancer. It was cleared in January 2022 for adults with unresectable or metastatic uveal melanoma who test positive for the HLA-A*02:01 marker.
RELATED: How The FDA’s Approval of a New Immunotherapy Improves The Treatment of Eye Cancer
For metastatic ocular melanoma, other treatment options may include liver-focused therapies, focal treatments on individual liver tumors, and/or clinical trials.
Dr. Jeffrey Weber, NYU Langone Health oncologist and melanoma specialist, previously told SurvivorNet that the approval of tebentafusp-tebn (Kimmtrak) is a “game-changer,” since the options went from nothing to something that has been shown effective.
Dr. Weber said the approval of tebentafusp for ocular melanoma may lead to new treatments for other types of melanomas as well, such as cutaneous melanoma (melanoma of the skin), which is the most common type of melanoma worldwide.
“If it worked in ocular, why wouldn’t it work in cutaneous?” he added. “By the end of the year, we’ll hear more about whether tebentafusp works in skin melanoma. That’d be maybe a little bit of a game-changer too, because it’s probably not super toxic. So I like that idea.”
Learning More About Melanoma
Melanoma is the most dangerous form of skin cancer. It starts in the same cells that give your skin, hair, and eyes their color. In melanoma, the cells change in a way that allows them to spread to other organs.
Changes to a mole you’ve had for a while or a new growth on your skin could be signs of melanoma, according to SurvivorNet’s experts. You’ll want to watch them and tell your doctor about any changes you notice.
WATCH: How do you perform a skin check using the ABCDEs?
You’re most likely to find melanoma on sun-exposed skin areas like your face, neck, arms, and legs. Surprisingly, you might also find them in other places as well, like:
- The palms of your hands or soles of your feet
- On your eyes or mouth
- Under your nails
SurvivorNet experts recommend avoiding unprotected sun exposure because ultraviolet (UV) radiation can lead to melanoma. Tanning beds pose ultraviolet radiation risks for skin cancer and should be avoided. Many dermatologists recommend using spray tans to reduce the risk of melanoma skin cancer.
What Are the Symptoms of Melanoma? And Treatment Options?
The most important thing to look out for when it comes to finding melanoma is a new spot on your skin or a spot that is changing in size, shape, or color, SurvivorNet’s medical experts say.
When you check your skin, use the acronym ABCDE as your guide:
- Asymmetrical moles: If you drew a line straight down the center of the mole, would the sides match?
- Borders: Is the mole irregular or jagged?
- Colors: Are there multiple distinct colors in the mole?
- Diameter: Is the mole larger than 6 millimeters (mm), about the size of a pencil head eraser?
- Evolution: Has the mole’s color, shape, or size changed over time?
RELATED: The Genetic Mutation That Drives Many Metastatic Melanomas
If you answered “yes” to any of these questions, our experts say it’s time to see your dermatologist for a skin check.
Melanoma treatment has come a long way. Survival rates have risen dramatically, thanks to a “treatment revolution,” say SurvivorNet’s experts. With breakthrough treatments like targeted therapy and immunotherapy now available, people who are diagnosed today have a much better chance of living a long and healthy life than ever before.
If you’re diagnosed with melanoma, there’s a good chance surgery is going to be the treatment your doctor recommends. In the early stages of the disease, removing the cancer should lead to a cure. The question is typically not whether you’ll get surgery, but which kind you’ll have.
WATCH: Dermatologic Surgeon Dr. Nima Gharavi, On The “Gold Standard Treatment” For Melanoma
For an early-stage melanoma that is close to the skin surface, Mohs surgery might be an option. This technique removes skin cancer, layer by layer, until all the cancer is gone.
“What is being done is that you’re able to remove a very conservative margin around the cancer and study it in, essentially, real time,” explains Dr. Sumaira Aasi, a professor of dermatology and Director of Mohs and Dermatologic Surgery at Stanford.
“The Mohs surgeon will take a conservative cut circumferentially around the cancer, where we’re able to preserve healthy tissue. We’re able to process the tissue and look at the cancerous tissue and know where there are still tumor cells persisting. As the Mohs surgeon removes the cancer, it’s mapped out.”
Dr. Aasi adds that the surgeon may go back specifically to the areas where the cancer cells were present and take out another conservative margin or amount of cancerous tissue and repeat the process until the cancer is out completely.
In general, stage I melanoma surgery consists of the simple, in-office removal of the cancerous cells by a dermatologist. If the cancer is thicker, your surgeon will remove it through a technique called wide excision surgery.
The removal of stage II and III melanomas are performed by surgeons or surgical oncologists, not dermatologists. You may also have a sentinel lymph node biopsy to see if the melanoma has spread to the first lymph node where it’s most likely to travel. If your cancer has reached this first lymph node, it may have spread to other neighboring lymph nodes, and possibly to other organs. Where the cancer is will dictate your treatment.
After surgery, the removed tissue and lymph nodes will go to a specialist called a pathologist, who will measure the melanoma and find out if it has clear margins. Having clear margins means the cells around the area of tissue that was removed don’t contain any melanoma. When there aren’t any cancer cells left around the removed area, your cancer is less likely to come back.
Once your cancer spreads, treatment gets a little more complicated, but there are still ways to stop it. New treatments have vastly improved the outlook for people with metastatic, or stage IV, melanoma.
Targeted drugs and immunotherapy have been shown to be more effective than chemotherapy. So, with many more choices, there is no standard treatment. Treatment will vary based on your condition and whether there is recurrent disease.
Research has found that immunotherapy drugs such as Keytruda (pembrolizumab) and Opdivo (nivolumab) helped some people live longer. Combining immunotherapy drugs Yervoy (ipilimumab) and Opdivo (nivolumab) has also extended survival. Opdivo (nivolumab) + relatlimab is a new therapy option added to the National Comprehensive Cancer Network guidelines in 2022. The combination of two immunotherapies is called Opdualag.
For those with the BRAF mutation, targeted drugs which shrink or slow the tumor can be a good option. This could include a combination of drugs, such as:
- Zelboraf (vemurafenib) and Cotellic (cobimetinib)
- Braftovi (encorafenib) and Mektovi (binimetinib)
- Tafinlar (dabrafenib) and Mekinist (trametinib)
- Zelboraf (vemurafenib) and Cotellic (cobimetinib) can also be combined with atezolizumab.
“Every patient is different and every situation is different,” says Dr. Anna Pavlick, medical oncologist at Weill Cornell Medicine. She emphasized that “there is no cookie-cutter recipe,” for treating stage IV melanoma.
There Is No “Cookie Cutter Recipe” for Treating Stage Four Melanoma
Dr. Pavlick also notes the importance of personalized care and treatment. “It really is a matter of looking at the tools we have so that we can pick the right tools to give the patient the best outcome.”
So, while there is no one-size-fits-all approach to treating metastatic melanoma, your doctor will work with you to develop a treatment plan that is tailored to your individual situation. Advances in research and technology are making the fight against metastatic melanoma more hopeful than ever.
Leading Experts Urge Us to Be Proactive
“If I had any advice for you following a cancer diagnosis, it would be, first, to seek out multiple opinions as to the best care,” National Cancer Institute Chief of Surgery Steven Rosenberg told us in a previous interview, “because finding a doctor who is up to the latest of information is important.”
As we highlight in several areas of SurvivorNet, highly respected doctors sometimes disagree on the right course of treatment, and advances in genetics and immunotherapy are creating new options. Also, in some instances the specific course of treatment is not clear cut. That’s even more reason why understanding the potential approaches to your disease is crucial.
At the National Cancer Institute, there is a patient referral service that will “guide patients to the right group depending on their disease state so that they can gain access to these new experimental treatments,” Rosenberg says.
Cancer Research Legend Urges Patients to Get Multiple Opinions
Furthermore, getting another opinion may also help you avoid doctor biases. For example, some surgeons own radiation treatment centers. “So there may be a conflict of interest if you present to a surgeon that is recommending radiation because there is some ownership of that type of facility,” Dr. Jim Hu, director of robotic surgery at Weill Cornell Medical Center, tells SurvivorNet.
Other reasons to get a second opinion include:
- To see a doctor who has more experience treating your type of cancer
- You have a rare type of cancer
- There are several ways to treat your cancer
- You feel like your doctor isn’t listening to you, or isn’t giving you good advice
- You have trouble understanding your doctor
- You don’t like the treatment your doctor is recommending, or you’re worried about its possible side effects
- Your insurance company wants you to get another medical opinion
- Your cancer isn’t improving on your current treatment
RELATED: Be Pushy, Be Your Own Advocate, Says Leading Expert
Bottom line, being proactive about your health could be a matter of life or death. Learn as much as you can from as many experts as you can, so that you know that you did your best to take control of your health.
Contributing: SurvivorNet Staff
Learn more about SurvivorNet's rigorous medical review process.
