“Think of sunglasses as sunblock for your eyes,” is an oft-repeated slogan around the New York Eye Cancer Center. While most people tend to think of sunglasses as just a fashion statement, they may actually be a key defense against eye cancer. Sun causes damage to eye tissues just like it does to skin – but since you can’t put sunblock on your eyeballs, you should make sure to wear UV-cancelling sunglasses as well.

Fortunately, other groups are joining in this educational effort.

Last month, Vision Express Stores in Great Britain gave away sunglasses to mark World Retinoblastoma Awareness Week. We applaud this initiative and would encourage similar efforts here in North America.

Ocular melanoma is the most common primary eye tumor in adults. There are around 2,000 new cases every year. These tumors are closely related to skin cancer. Since exposure to the sun is a known risk factor for skin cancer, it follows that UV rays from the sun may also increase the risk of developing this form of eye cancer. There is circumstantial evidence linking exposure to sunlight and ocular melanoma. This form of eye cancer is more common in patients with blue eyes, and who work in outdoor occupations.

When any form of light is absorbed by the body, a reaction occurs resulting in heat and chemical changes. Ultraviolet light rays are particularly energetic and cause more chemical reactions in ocular tissues than visible light. This means more potential damage to the eye.

While the link between sunlight and eye melanomas is not conclusively proven, it is worth a small precaution that will make you look like James Dean anyway!

A good pair of sunglasses will block all UVA, UVB, and UVC rays. You want to make sure the glasses provide 100% UV protection. Many optical shop have a machine called a photometer that measures UV transmission through glasses. You want to wear sunglasses that block all UV radiation or light under 400 nm in wavelength.

Buyer beware: Shades that don’t offer this protection – or cheap pairs that fraudulently claim to – may actually increase the risk of eye cancer by allowing more UV light into the eye.

So choose wisely, make that fashion statement, and protect your eyes at the same time.

The following is a statement from The Eye Cancer Foundation, reprinted here with their permission:

The Eye Cancer Foundation (ECF) today announces a bold new strategic direction with a focus on bringing patients in every country access to an #EyeCancerCure. This will be accomplished through the Eye Cancer Working Day events, sponsored fellowships to train specialists in underserved countries, and the Bioinformatics Grid (BIG) project. The ECF seeks to broaden participation in its efforts, facilitate information sharing, and promote more rigorous research practices.

To this end, the Foundation has revamped its website at the new domain of eyecancercure.com, launching a truly international effort to achieve an #EyeCancerCure for as many patients as possible. Typing “eyecancercure” will now take you to the Foundation’s presence on Facebook, Twitter, and the web.

This new strategic vision reflects both a focusing of scope and a widening of participation. The Eye Cancer Foundation got its start some two decades ago through the efforts of our Chairman Dr. Paul T. Finger to fill the need for an online resource with essential information for eye cancer patients and physicians. The result was The Eye Cancer Network, which has grown and remained essential reading for anyone affected by or treating eye cancer. In the meantime, the ECF built a Board of committed benefactors, a truly global network of eye cancer specialists, and a brigade of passionate volunteers. Together, we have accomplished many important projects, especially developing an international, scientific, AJCC-UICC language used to describe most types of eye cancers.

Today, the ECF has reached a point that we are ready to diversify participation and focus our mission. Dr. Finger generously furnished the vast majority of content on The Eye Cancer Network. He will henceforth be personally developing this trove of information into an “online textbook” for continued use by patients and practitioners for years to come. Meanwhile, the new Foundation website highlights our key projects, inspiration and support for patients, and the world’s largest eye cancer specialist directory. In the coming years, we will be reaching out to eye cancer specialists around the world to provide new community-based content contributed by our many affiliated physicians and patient members. Dr. Finger remains dedicated to his role as Chairman of The Eye Cancer Foundation Board as we seek more contributors to accomplish the ambitious goals we have set for the next 5 years.

Are you ready to achieve an #EyeCancerCure? Now is the time to get involved. Contact us for more information.

A relatively new treatment for retinoblastoma quickly gaining popularity has shown some promise, but a systematic review of reported outcomes published in March provides some reason for caution.

Intra-arterial chemotherapy has rapidly emerged as a treatment for intraocular retinoblastoma, the most common primary childhood eye cancer. It involves inserting a microcatheter into the ophthalmic artery and manually injecting chemotherapy drugs though it directly into the eye.

Centers in some 30 countries have adopted the technique, and a few have completely replaced systemic chemotherapy and radiation with intra-arterial chemotherapy as the primary treatment for retinoblastoma.

Last year, several doctors set out to perform a systematic review to summarize reported outcomes of intra-arterial chemotherapy. They published their results in the Journal of the American Medical Association, Ophthalmology in March, 2016.

The researchers performed comprehensive searches in Medline, Embase, Cochrane, and Web of Science from inception through January 2015. They included any peer-reviewed English-language publication that described outcomes related to toxicity or efficacy in at least four patients. Through their search, the doctors identified 28 publications that met their inclusion criteria. The researchers ultimately evaluated cases encompassing 655 patients, 757 eyes, and a total of 2,350 catheterizations.

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While they found the treatment yielded a 66% eye-preservation rate, the number of children who subsequently developed metastases raises concern. The researchers concluded that while the treatment seems promising, more study is needed.

“Metastases have been observed, and long-term follow-up is needed. “Until the results of clinical, prospective studies are available, it is recommended that intra-arterial chemotherapy be offered selectively among other options, with fully informed discussion about all possible risks, benefits, and uncertainties.”

The researchers pointed out that the prior method of systemic chemotherapy addressed the issue of metastases, while intra-arterial chemotherapy does not.

“The most important critique of the IAC literature is the uncertain rate of metastases. Eye salvage is only valuable when achieved without a risk to life. Systemic chemotherapy provides protection against metastases, especially in children who may have unknown high-risk pathologic features in retained eyes, but IAC does not.”

While promising, intra-arterial chemotherapy may not be the panacea some doctors hope. The recently published systematic review of reported outcomes indicates some caution is necessary and more study is definitely needed.

You can read the complete study in the March 2016 issues of the JAMA Ophthalmology. Access it HERE.

Data released Monday to coincide with World Retinoblastoma Awareness Week (May 8-15) reveals a troubling lack awareness of this deadly form of eye cancer.

Retinoblastoma is the most common eye cancer in young children. If not detected early, it’s also the most life-threatening. Each year, doctors diagnose more than 5,000 new cases of retinoblastoma. If caught early, this form of eye cancer is treatable and the eye can usually be saved. But unfortunately, awareness of this disease is low, and it often goes undiagnosed for long periods of time.

A recent survey conducted by the Childhood Eye Cancer Trust found that only 28% of parents had ever taken their children ages six and younger to an optician, general practitioner, or other health professional for an eye check. Half of the parents surveyed weren’t even aware that children can develop eye cancer.

Patrick Tonks, chief executive of CHECT, said it is imperative to increase awareness among both parents and physicians.

“For too long retinoblastoma has been shrouded in darkness – children are left in the dark because some or all of their vision has been stolen and parents and healthcare professionals are in the dark because of a lack of awareness.”

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Delay in diagnosing retinoblastoma can lead to the loss of an eye, or even death. Parents should always trust their instincts and there are several symptoms they should be aware of. These include unusual squinting, changes in the color of the iris, and painless redness or inflammation of eye. White reflections in the eye will also sometimes show up in photos taken with a flash camera.

It’s not only important for parents to be aware of retinoblastoma. Physicians should always address parental concerns relating to eye health and be sure to follow up on any symptoms. It is also recommended that doctors perform a red reflex test following symptoms such as squint or leukocoria to rule out retinoblastoma.

Simple awareness and follow-through can save a child’s vision, or even her life.

As a parent, you should trust your instincts. If you sense something might be wrong, you’re probably right. Ignoring your gut feeling could lead to tragic consequences.

Timely diagnosis is key to treating eye cancer. When caught early, doctors can often remove the tumor and preserve sight. But waiting too long can lead to loss of an eye or even death.

A British mother learned the consequences of delayed diagnosis the hard way.

According to a Press Association story, Stephanie Beasley noticed something was wrong with her daughter’s eye when she was 9 months old:

“Ezmai had a lazy eye and we spoke to a health visitor and then took her to the pre-school vision team but they told me to come back in six months. I kept taking her but they couldn’t find anything wrong. My instincts told me there was something wrong with Ezmai but I felt no-one believed me and that I was going mad.”

Beasley said she took Ezmai to four different health care providers, but the child was not diagnosed with retinoblastoma until she was two. By then, the cancer had advanced to the point that surgeons had to remove her eye in order to save her life.

Beasley has now teamed up with the The Childhood Eye Cancer Trust in Great Britain to raise awareness of the importance of early diagnosis of retinoblastoma. According to the organization, 21% of children diagnosed with this rare form of cancer in 2015 experienced a delay of at least six months between their first visit to a general practitioner and examination by a specialist.

Symptoms of retinoblastoma include unusual squinting, changes in the color of the iris, and painless redness or inflammation of eye. White reflections in the eye will also sometimes show up in photos taken with a flash camera.

“We looked back at an old photo of Ezmai at Christmas in 2014 and we can see a white glow in her eye but at the time we had absolutely no idea this could mean she had cancer,” Beasley said.

The Eye Cancer Foundation offers a free-downloadable retinoblastoma poster and a program, and sponsors a program to teach fellows to become retinoblastoma specialists in unserved and underserved countries. Dr. Paul Finger said it is imperative to make medical professionals more aware of the symptoms.

“Clearly, early detection and prompt treatment of children with retinoblastoma is crucial for saving both their vision and life.”

If you notice any of unusual symptoms in your children, you should have them examined by a specialist as soon as possible. Trust your instincts and don’t allow doctors who are not trained in detecting eye cancer dissuade you from getting an examination by a specialist.

This is definitely a situation where you would rather be safe than sorry.

A new study suggests patients diagnosed with uveal melanoma could benefit from immunotherapies that harness the body’s own anti-tumor T-cell populations.

Uveal melanoma is a cancer of the eye involving the iris, the muscle surrounding the lens, or the choroid – a vascular layer with connective tissue between the retina and the sclera. It is a rare form of eye cancer with no effective therapies once it spreads to other parts of the body.

Uveal melanoma is related to more common cutaneous (skin) melanoma. Doctors have had success treating skin cancers with immunotherapies that boost naturally existing anti-tumor T-cell responses. In essence, this approach boosts the body’s natural immune system response to fight the tumor.

For instance, a drug called Keytruda was used to successfully treat Jimmy Carter’s melanoma. But despite the success of immunotherapy in attacking more common skin cancer tumors after it spreads, the role of this type of treatment for metastatic uveal melanoma remains unclear.

Fifteen researchers from the National Cancer Institute and other institutions embarked on a study to determine if this type of anti-tumor immune response exists against uveal melanoma.

To conduct the study, researchers surgically procured metastases from the livers of eye cancer and skin cancer patients. They then compared the tumor cell structures along with their associated T-cells using a variety of techniques.

Researchers found that despite the fact both are related to melanoma, the tumor cells differed in melanin content, the antigens provoking an immune response, and in their genetic mutations. The T-cells attacking the tumors were also different. Researchers found that anti-tumor T-cells in cutaneous melanoma were predominantly CD8+ while those in uveal melanoma were CD4+ dominant.

While the effectiveness of T-cells against the tumors was much higher in cutaneous melanoma, researchers found a subset of T-cells in uveal melanoma that had robust anti-tumor reactivity, comparable in magnitude.

Researchers say the absence of melanin pigmentation in the original tumor, before it metastasized to the liver, strongly correlated with the production of highly effective T-cells. In other words, a group of uveal melanoma tumors with lower melanin levels do seem to produce a promising immune system response that attacks the tumor

Knowing that a subset of uveal melanoma tumors with the ability to provoke an immune response exists opens the door to further research. Doctors can build on this knowledge to potentially discover therapies that will boost anti-tumor T-cell production and/or effectiveness to successfully treat this form of eye cancer even after it spreads..

Your smartphone camera flash can detect a rare eye cancer, potentially saving a child’s life.

That was the case for an Arizona mother who inadvertently detected retinoblastoma in her young son after using a flash phone camera to take pictures of him. Andrea Temarantz noticed a white glow in the left eye of her son Ryder in the photographs she’d taken. Suspecting it was due to a faulty phone camera, Temarantz switched to a better device. When the white spot remained, she took Ryder to see a doctor. The physician identified a tumor, and diagnosed him with retinoblastoma, a potentially fatal eye cancer.

While rare, retinoblastoma is a very aggressive eye cancer found almost exclusively in young children. Leukocoria (white pupil) and misaligned eyes (strabismus) are the most common symptoms. In other cases, the child may have developed neovascular glaucoma and may be in pain. Retinoblastoma treatment typically requires the cooperation of an ophthalmic oncologist, pediatric oncologist, and radiation therapist. Over the last 30 years, treatment has evolved from enucleation (removal of the eye), to eye-sparing radiotherapy. More recently doctors have treated selected patients  with chemotherapy-based multi-modality therapy.

An early diagnosis is critically important for successful treatment. Retinoblastoma is almost always fatal when left untreated.

When flash photography creates a white pupil in the eye of a child, it could be that the light is illuminating a tumor not otherwise visible. While a white spot in the eye is not always indicative of a tumor, a visit to the doctor for a diagnosis is always a good precautionary measure.

Temarantz’s experience was not an isolated case. A Rockford, Illinois, child owes his life to a camera flash and a vigilant parent. Julie Fitzgerald noticed a white spot in showing up in photos of her son Avery’s left eye. After reading stories online about similar cases, she decided to take her son to see a doctor. Avery was ultimately diagnosed with retinoblastoma. Unfortunately, his left eye had to be removed, but the diagnosis saved his life.

Using a smartphone’s camera flash to detect a potential instance of retinoblastoma is effective enough that the UK-based Childhood Eye Cancer Trust conducted an ad campaign in 2014 to raise awareness of its life-saving potential for early detection.

A drug thrust into the limelight by Jimmy Carter shows promise for treating melanoma of the eyelid.

Keytruda (pembrolizumab) gained FDA approval for limited treatment of metastatic melanomas in late 2014. The agency gave its blessing for front-line treatment in patients with unresectable or metastatic melanoma on Jan. 7 of this year.

The drug is a form of immunotherapy and works by unleashing the body’s immune system to attack cancerous melanin cells. If the cancer is not caught early, it can spread deep into the skin and spread to other organs. At that point, chemotherapy becomes generally ineffective. Testing has shown Keytruda can drastically increase survival rates for advanced skin cancer.

Keytruda is what is known as a PD-1 inhibitor. T-lymphocyte cells are the body’s primary cancer fighter. Tumors express proteins called PD-L1 and PD-L2. These “program death” proteins lure T-lymphocyte cells to bind to them. Eventually, this results in T-cell exhaustion and reduces their ability to fight the cancer. Keytruda works by targeting the PD-1 receptors, allowing the body’s immune system fight the cancer cells. UConn doctors say they have had great success using Keytruda, according to Dr. Upendra P. Hegde, an associate professor in the Department of Medicine.

“Keytruda is the first PD-1 inhibitor drug that is allowing us to shrink the melanoma tumors in up to 35 percent of our UConn Health patients, and we are continuing to see more progress over time.”

A study reported in the Wall Street Journal last spring compared Keytruda to an older immunotherapy called Yervoy. The study revealed a significant increase in overall survival rates.

“The study…tested Keytruda against Yervoy in more than 830 patients whose melanoma had spread to other parts of the body. About one-third of patients received Keytruda once every two weeks, one-third received it every three weeks, and the remaining one-third received the standard four cycles of Yervoy. One year after the start of treatment, 74% of patients who received Keytruda every two weeks and 68% of those who received it every three weeks were still alive, compared with 58% for those who received Yervoy. Measured another way, Keytruda reduced the risk of death by 31% to 37% versus Yervoy, according to the results, which were published online Sunday by the New England Journal of Medicine and presented at the annual meeting of the American Association for Cancer Research in Philadelphia.”

A similar drug approved for melanoma treatment has shown similar promise. Opdivo (nivolumab) works in the same way as Keytruda, blocking the PD-1 receptors. A study presented at the 2015 Society for Melanoma Research Congress showed the 2-year overall survival rate with frontline nivolumab was 57.7%.

Jimmy Carter underwent treatment for his melanoma with Keytruda. He was diagnosed with an aggressive form of skin cancer that had spread to his brain and liver. In December, the former president announced he was cancer-free, putting the cancer-fighting drug in the national spotlight.

Learn more about metastatic tumors here.