Understanding Uveal Melanoma & Treatment Advances
- Retired teacher Debbie Hensley learned that a freckle in the back of her eye turned cancerous and led her to have a detached retina. She was diagnosed with a rare form of eye cancer called uveal melanoma.
- About 1,500 to 2,000 people are diagnosed with uveal melanoma every year. And even after a first round of treatment, for about half of those diagnosed, the disease returns this time spreading to the liver.
- Tebentafusp (Kimmtrak) was recently approved by the Food and Drug Administration (FDA) to treat some people with melanoma of the eye, also known as ocular melanoma or uveal melanoma, whose cancer has spread or is not able to be treated with surgery. These patients previously had no approved treatment options.
- Other ways to treat Uveal Melanoma, according to the Melanoma Research Alliance, consist of radiation therapy, surgery, laser therapy, chemotherapy, and immunotherapy.
While seeing her ophthalmologist last fall, the Tennessee native admitted she thought the blurry vision she was experiencing was possibly a cataract [when an eye’s natural lens becomes cloudy].
Read MoreThe American Academy of Ophthalmology (AAO) explains, “A nevus is a common, colored growth on or in your eye. Sometimes called a freckle of the eye, it is similar to a mole on your skin. A nevus (plural: nevi) can be in the front of your eye, around the iris, or under the retina at the back of the eye.”
“People can be born with harmless eye nevi. A pigmented spot that develops later in life is usually harmless as well, but may have a greater risk of becoming cancer,” the AAO adds.
“An eye nevus needs to be watched regularly by an ophthalmologist because, like a skin mole, it could possibly develop into cancer of the eye.”
The AAO also notes that it’s possible that eye freckles may be linked to ultraviolet (UV) light.
More Resources On Melanoma
- Examining Your Skin for Melanoma: Remember ABCDE
- How is Melanoma Treated After Surgery? The Landscape of Therapies Explained
- A Biopsy of Your Mole Doesn’t Mean You Have Melanoma
- Be Aware of the Side Effects of Targeted Therapy for Melanoma
- ‘A Game Changer’: New Combination Immunotherapy for Advanced Melanoma Offers More Options For Patients
- Advances in Uveal Melanoma Treatment: Immunocore’s Chief Medical Officer On Their New Standards For Survival
As for how Hensley felt before treatment, she recounted to TODAY.com, “I thought, OK, here we go. What do we have to do first?
“My motto became: List each next step and do it… After that, I really wasn’t scared.”
The AAO also explains that treatment for an eye freckle that has become cancerous “may involve radiation, surgery, laser therapy, or removal of the eye” or “sometimes, waiting and watching the nevus closely is a treatment option.”
Hensley was luckily able to keep her eye as her doctor, Dr. Christopher Bowen, director of ocular oncology at Northwestern Medicine in Chicago, suggested proton therapy as treatment.
After undergoing surgery to get a biopsy of the tumor in her eye, for genetic testing, and have minuscule markers put on her eye for a proton beam to be aimed at, she was ready for treatment.
She underwent five 15-minute long treatments at Northwestern Medicine Proton Center in Warrenville, Illinois, which her doctor says has been successful.
Dr. Bowen told TODAY.com that her tumor has already reduced in size, meaning the retinal detachment should get better on its own.
Hensley will also continue to get checked to see if the cancer had spread to other areas of her body, like her head, chest, abdomen and pelvis.
Now, as her left eye’s vision has worsened, she hopes her story can inspire others to get checked and know eye cancer can happen to anyone.
Hensley—who is now warning anyone hearing her story to seek an eye doctor immediately if they see flashing lights, peripheral vision loss, or new floaters— said, “It’s such an unusual thing.”
Her doctor advises people with eye freckles to have eye exams that include getting dilated so that doctors can look at the back of the eye.
All About Uveal Melanoma
A lot of people don’t realize it, but you can actually get cancer of the eye. So, eye health is another area that we should all be monitoring. Uveal melanoma is a disease that forms in the tissue of the eye. 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.
“It is a disease that happens the most frequently in Caucasians and it happens in patients with hazel, green, or blue eyes more frequently than brown eyes … though we’re not clear of the mechanism that protects darker iris individuals from melanoma,” Dr. Sapna Patel previously told SurvivorNet.
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. Sapna Patel, a melanoma oncologist at MD Anderson Cancer Center, recommends getting regular check-ups to monitor for uveal melanoma.
What is Uveal Melanoma?
“Everyone should get dilated every year,” she said. “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.
Advances in Uveal Melanoma Treatment
For decades there has been no change or advancement in treatment for those diagnosed with metastatic uveal melanoma. But thankfully, a new kind of immunotherapy recently developed by biotechnology company Immunocore is changing the standard for what survival looks like.
Uveal Melanoma is a rare cancer that is found in the middle layer of the eye. About 1,500 to 2,000 people are diagnosed with it every year. And even after a first round of treatment, for about half of those diagnosed, the disease returns this time spreading to the liver.
SurvivorNet previously spoke with Dr. Mohammed Dar, the Chief Medical Officer at Immunocore, to understand their new breakthrough treatment for metastatic uveal melanoma called KIMMTRAK (molecular name tebentafusp-tebn).
The treatment is a weekly immunotherapy that triggers the T-cells in your immune system to fight uveal melanoma tumor cells. With a 50% increase in survival for patients, it’s the first medication ever that has shown increased survival rates for the disease and it does so in a really novel way.
RELATED: How The FDA’s Approval of a New Immunotherapy Improves The Treatment of Eye Cancer
“What we did was to look at a simple blood test, which looks at the presence of tumor DNA,” Dr. Dar told SurvivorNet at the ASCO Annual Meeting this year. “What we found was that by week nine, among these patients who by CAT scans or MRI, there was no improvement in the size of their lesions, around 45% of patients had complete clearance of the tumor DNA in their bloodstream.”
So, to be clear, after taking KIMMTRAK for nine weeks, the lesions or tumors in these patients had not shrunk or disappeared. But the blood test showed that the tumor DNA was gone.
“When we looked at the survival of these patients who cleared their ctDNA blood, these are the patients who had the longest survival,” Dr. Dar said.
Could measuring tumor DNA over tumor shrinkage on skin be a new way of measuring treatment success rates? Dr. Dar thinks it is a possibility. And with the success of KIMMTRAK, he is very excited for future advancements in treatment for the disease.
“Historically, nothing had moved the needle, so no one really focused on it and now it’s shed light that there is actually hope,” Dr. Dar said. “There’s more treatments that people are now evaluating for metastatic uveal melanoma. So it’s a great time for research and development.”
KIMMTRAK is FDA-approved and to be eligible, a patient must be diagnosed with metastatic uveal melanoma or ocular melanoma. A patient also has to do a blood test to see if they have a special marker on the surface of their tumor cells called an HLA Allele.
RELATED: Eye Cancer is Rare, But The Symptoms Are Not Always Obvious; Know What to Look For
Immunocore also announced at ASCO that they are expanding their research to see if KIMMTRAK could also be used to treat the most common melanoma skin melanoma or cutaneous melanoma. The new study will be launched in the United States and Europe.
Tebentafusp (Kimmtrak) was recently approved by the Food and Drug Administration (FDA) to treat some people with melanoma of the eye, also known as ocular melanoma or uveal melanoma, whose cancer has spread or is not able to be treated with surgery. These patients previously had no approved treatment options.
Dr. Jeffrey Weber, NYU Langone Health oncologist and melanoma specialist told SurvivorNet at the time that the approval is a “game-changer,” since the options went from nothing to something that has been shown effective.
Ocular melanoma can be difficult to diagnose since it often develops on part of the eye that can’t be seen. While most people don’t experience symptoms, those that do may find a dark spot on the iris (the colored part of the eye) or conjunctiva (the tissue covering the white of the eye) or experience blurred or distorted vision, a blind spot in side vision or the sensation of flashing lights. Ocular melanoma is often detected during a routine eye exam.
RELATED: Eye Cancer is Rare, But The Symptoms Are Not Always Obvious; Know What to Look For
“Uveal melanoma is a devastating disease that has historically resulted in death within a year of metastasis for our patients,” Dr. John Kirkwood, director of the Melanoma Center at the UPMC Hillman Cancer Center, said in a statement.
“The approval of (tebentafusp-tebn) represents a major paradigm shift in the treatment of metastatic uveal melanoma, and for the first time offers hope to those with this aggressive form of cancer.”
Other ways to treat Uveal Melanoma, according to the Melanoma Research Alliance, are:
- Radiation therapy: The most common treatment for uveal melanoma.
- Surgery: An possible option for small tumors that have not spread beyond the eye.
- Laser therapy: Laser therapy, using a high-energy beam of light to destroy cancer cells.
- Chemotherapy: An uncommon treatment for uveal melanoma, but it may help for advanced cases that have metastasized.
- Immunotherapy: A type of treatment using the body’s immune system to fight cancer. This may be used for advanced cases of Uveal melanoma.
The Sun is Not Your Friend: Most Melanomas Are Caused by Sun Exposure
Contributing: SurvivorNet Staff
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