Unusual cases of rare eye cancer puzzling doctors

Doctors are puzzling over dozens of cases — mostly women in their 20s and 30s — of a rare eye cancer in two Southern states. Over the last several years, a group of graduates from Auburn University in Alabama and 18 people in Huntersville, North Carolina say they have been diagnosed with ocular melanoma, usually found in just six out of every million people.

Earlier this year, Ashley McCrary, one of the patients who attended Auburn, started a Facebook group to connect with other people from the university who have been diagnosed with the eye cancer. A separate, similar Facebook community was started a few years ago for people in North Carolina.

While doctors are reluctant to call this a cancer cluster — no cause or common thread has been found — researchers are studying these patients to see if a link, if any, exists between the two groups.

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Well-known figures who have battled cancer
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Well-known figures who have battled cancer

Former U.S. President Jimmy Carter

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Television personality Robin Roberts

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Another basal cell carcinoma. Thanks to frequent checks & amazing doctors, all's well. Looks worse w the dressing o… https://t.co/cFIi0Zhmtr

Actress Shannen Doherty

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Chef Sandra Lee

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Cyclist Lance Armstrong

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Actress Rita Wilson attends

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Former GMA anchor Joan Lunden

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TV host Sharon Osbourne

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Journalist Tom Brokaw

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Actress Christina Applegate

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Comedian Wanda Sykes

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TV personality Giuliana Rancic

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Musician Sheryl Crow

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Musician Rod Stewart

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Fox host and DWTS co-host Erin Andrews 

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Caitlyn Jenner
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What is ocular melanoma?

Ocular melanoma (OM) — melanoma in or around the eye — is a type of cancer that develops in the cells that produce eye color. Just as you can develop melanoma on your skin from mutated pigment cells, you can also develop it inside your eye, but unlike skin melanoma, sun exposure is not the cause.

Although it is the most common eye cancer in adults, ocular melanoma is very rare — about 2,500 adults are diagnosed every year in the U.S. It can occur in all races, at any age, but the average age of diagnosis is 55 years old.

"Most people think of melanoma — the deadliest form of skin cancer — as ominous black spots on the skin, but it can also develop inside your eye. It's rare, accounting for about 5 percent of all melanoma cases, but the eye is the second most common location for melanoma to grow," said Dr. Sapna Patel, a melanoma oncologist at MD Anderson Cancer Center in Houston.

Most eye melanomas form in the part of the eye you can't see when looking in a mirror so they can be difficult to detect. To make matters worse, they often present without any early signs or symptoms.

"Oftentimes patients are asymptomatic at the time of diagnosis and the cancer is diagnosed on a routine eye exam," said Dr. Sunandana Chandra, melanoma medical oncologist at the Robert H. Lurie Comprehensive Cancer Center Northwestern University in Chicago, Illinois. "That's why it is so important to see your ophthalmologist regularly."

If the spot is big enough it may effect one's vision.

Signs of eye melanoma can include:

  • Blurry vision or sudden loss of vision
  • Floaters (spots or squiggles drifting in the field of vision) or flashes of light.
  • Visual field loss (losing part of your field of sight)
  • A growing dark spot on the colored part of the eye (iris)

What causes ocular melanoma?

It's not clear why eye melanomas develop but people with light colored eyes are at higher risk of getting the cancer. Genetic mutations might put people at higher risk.

"It's impossible at this juncture to tell what causes this," said Dr. Jonathan Zager, surgical oncologist and director of Regional Therapies in the Departments of Cutaneous Oncology and Sarcoma, Moffitt Cancer Center in Tampa, Florida. "Suspected contributors include BAP1 mutations and possibly a small link to sun exposure."

Some eye cancers run in the family but most experts believe that the majority of cases develop from random mutations.

"There is ongoing research to try to identify other risk factors, but it's important to note that people who have had a melanoma of the skin aren't at a higher risk of developing of developing an ocular melanoma," said Dr. Zeynep Eroglu medical oncologist and associate member in the Department of Cutaneous Oncology at Moffitt Cancer Center in Tampa, FL.

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Things in your home that can cause cancer
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Things in your home that can cause cancer

Your big, comfy couch

Your favorite sofa could be killing you, and not just because it lures you away from activity: Many sofas, mattresses, and other cushioned furniture are treated with TDCIPP, a flame retardant known to cause cancer (i.e., a carcinogen). TDCIPP was used so frequently prior to 2013 that a study out of Duke University found it in the blood of everyone they tested. It's also one of ten chemicals most frequently found in household dust, according to this study.

What can you do?

Consider replacing cushioned furniture you purchased prior to 2013, according to the Natural Resources Defense Council—and check furniture labels on any purchases.

And while you're at it, make sure you aren't around any of these other causes of cancer.

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Your curtains and carpets

Cadmium is a carcinogenic byproduct of cigarette smoke. If you smoke in your house, cadmium and other cigarette smoke by-products may be lurking, especially on soft surfaces such as curtains and carpet—even long after the smell of smoke is gone. There's even such a thing as third-hand smoke and it's resistant to even the strongest cleaning products. Here's where you can learn more about third-hand smoke and its dangers.

What can you do?

Quit smoking—here are 23 tips to kick start kicking the habit—and never allow smoking in your home.

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Your leather recliner

Chromium (VI) is a known carcinogen found in tanned leather, wood furniture, certain dyes and pigments used in textiles, and cement. To give you an idea of the prevalence of chromium VI, one study out of Denmark found that almost half of imported leather shoes and sandals contained some level of the carcinogen.

What can you do?

As with TCIPP, pay attention to labeling. And don't be shy about asking questions of your furniture salesperson.

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Your garden

Dioxin is a carcinogen that forms as a chemical byproduct and ends up in our soil and water. It's in the dust on shelves, the dirt on floors, and the residue on vegetables. Your risk of cancer from dioxin exposure may be greater than one in one-thousand, says clean-living guru, Sophia Ryann Gushée.

What can you do?

Wear gloves when working in the garden, and always wash up before heading inside. Additionally, avoid backyard burning of household trash.

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Your old fridge

According to cancer.org, carcinogenic PCBs can turn up in old appliances, fluorescent lighting fixtures, and electrical transformers. While no longer commercially produced in the United States, PCBs are still manufactured and used in developing countries, and of all PCBs ever produced, up to 70 percent are still in the environment. Diet is another major source of exposure, according to Gushée.

What can you do?

Get rid of those old appliances and fluorescent light fixtures. Pay attention to advisories regarding PCB-contaminated fish and fish-eating wildlife.

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Your cleaning products

Formaldehyde is a known carcinogen found at home in food, cosmetics, a variety of cleaning products (such as dishwashing liquids, fabric softeners, and carpet cleaners), paint, foam insulation, and on permanent press fabrics. In addition, you can be exposed by breathing smoke from gas cookers and open fireplaces.

What can you do?

Here is a list of household products that contain formaldehyde, according to the U.S. Department of Health and Human Services. Choose your cleaning products carefully—here are some chemical-free ways to clean your home. Also, be sure to ventilate your cooking areas.

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Your closets

The dry-cleaning chemical perchloroethylene (tetrachloroethylene or "perc") is a carcinogen that can build up wherever you store your dry-cleaned clothes. It's also found in spot removers, shoe polish, and wood cleaners.

What can you do?

Wear gloves when polishing your shoes and cleaning wood. If you dry-clean your clothing, try to find a dry-cleaner who doesn't use perc. And check out the times you can feel free to ignore the dry-clean only label.

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Your vinyl flooring and miniblinds

Phthalates are suspected of causing cancer and may adversely affect human reproduction or development. They're found in vinyl flooring, shower curtains, synthetic leather, miniblinds, wallpaper, and anything made with PVC vinyl. They're also found in food packaged in plastic.

What can you do?

Stay away from products made with PVC vinyl. Look for products that are labeled as phthalate-free. Toss plastic toys made before 2008, according to mindbodygreen.com, and switch to glass and stainless containers and bottles. And reconsider your use of plastic wraps and food containers.

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Your favorite rice and chicken dinner

Everyone knows arsenic is poisonous, but in smaller doses, it's also carcinogenic. Yet you can find it in foods you probably eat regularly—including chicken, rice, and certain fruit juices, as well as in degreasing products, dyes, furniture wax, glues, lubricants, nylon, and paints.

What can you do?

Serve only organic chicken, and follow these rice-related guidelines issued by Consumer Reports. Check the labels on your household products; people following a gluten-free diet may be at particular risk of arsenic exposure.

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Your insulation

Asbestos has been out of favor for decades, thankfully, but you can still find it in the insulation of older homes. As the insulation eventually deteriorates, asbestos fibers become airborne. Since asbestos fibers stick to clothing and shoes, workers exposed to asbestos on the job can also bring asbestos into their homes.

What can you do?

Follow these guidelines to reduce asbestos exposure in your home.

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Your styrofoam cups

Styrene is a known carcinogen widely used in the manufacturing of polystyrene plastics, which can be made into foam and rigid plastic products such as cups, plates, trays, utensils, packaging, and packing peanuts. Styrene may leach into your hot coffee or soup if you're using styrofoam containers. It's also present in cigarette smoke and in all of these home maintenance, automotive, and crafting productsWhat can you do? Avoid using styrofoam to hold hot foods and liquids, and read your product labels carefully. Find out the 12 foods you should never microwave.  

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Your library books

According to the Library of Congress and other government sources, medical supplies, library books, and museum artifacts may all be sterilized or fumigated with ethylene oxide, which is a known carcinogen. What can you do? Mostly a problem for people who work with the chemical, you can minimize exposure by not bringing items into your house that have been exposed to ethylene oxide.

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Your weedkiller

No one likes weeds, but if you decide to decimate them using herbicides such as Roundup, which contains the carcinogen glyphosate, you may be raising your risk for cancer.

What can you do?

Make a practice of carefully reading the labels on your weedkillers. And consider using some of these natural weed killers that don't contain dangerous chemicals.

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Your bug spray

Pantry pests and other creepy crawlies can carry disease. But if you eliminate them using chemical pesticides, you're increasing your risk of cancer. Chemical pesticides include those that you use on your pets, such as flea collars and tick-repellant.

What can you do?

Seek out less-toxic pesticide alternatives—or make your own, like this natural tick repellent that works.

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Your granite counter

Radon is formed naturally from the radioactive decay of uranium in rocks and soil. It raises the risk of lung cancer—especially if you also smoke, says Ashley Sumrall, MD, FACP, a Charlotte-based oncologist. If you live in an area where the amount of uranium and radium in rocks is high, you can be exposed to radon through cracks in your foundation. You can also be exposed to radon if you have a granite countertops.

What can you do?

If you live in an area with high levels of uranium and radium, or if you have granite countertops, consider having your home's radon levels measured. Here's what you need to know about radon testing.

Next, find out the causes of cancer that might surprise you.

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How is it treated?

"If diagnosed, your choice for treatment is removal of the eye or radiation. They're both equally effective, but you lose your vision with both. Radiation is not performed for vision preservation, just for cosmetic preservation," said Patel.

Immunotherapy drugs like pembrolizumab (Keytruda) or nivolumab (Opdivo) that work well in patients with much more common skin melanomas are far less effective for patients with ocular melanoma, therefore patients with metastatic eye cancer are candidates for clinical trials with newer agents that may show more promise than the currently available drugs, said Eroglu.

Eroglu and Zager, who are leading research for the treatment of OM that has traveled to the liver — the FOCUS trial — hope that percutaneous hepatic perfusion, a surgical procedure that delivers high dose chemotherapy to the liver, is the key to increasing survival in these patients.

How likely is survival after diagnosis?

When the cancer is confined to the eye, the 5-year survival rate is 80 percent. Survival rates tend to be better if the cancer is found early, but early detection can be difficult since the cancer does not present with symptoms most of the time.

"Half of cases will ultimately metastasize. But it can be a delayed metastasis," said Patel. "Thirty percent of people will have metastasis at year five, 40 percent at year 10, and 45 percent at year 15. The cancer cells go directly into the bloodstream and typically travel to the liver."

Once the cancer spreads it becomes much more difficult to treat.

"We can't use chemo to treat. What works for skin melanoma typically doesn't work for eye melanoma."

Therefore, 5-year survival for metastatic eye cancer is less than 10 percent.

"We don't know what causes this cancer, we don't know the underlying risk factors, so it is challenging to recommend preventive strategies, but in general it's a good idea for people to wear sunglasses with ultraviolet protection," said Patel.

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