What to know about Merkel cell carcinoma, Jimmy Buffett’s rare cancer

By Jen Christensen, CNN

(CNN) — Singer-songwriter Jimmy Buffett, who died September 1, had Merkel cell carcinoma, a skin cancer that’s diagnosed in fewer than 3,000 people in the US every year.

Skin cancer is the most widespread form of the disease in the United States, according to the US Center for Disease Control and Prevention, but Buffett’s death may be the first time people have heard of Merkel cell carcinoma. The condition was named for German scientist Friedrich Merkel, who first described that type of cell in 1875.

Merkel cells are thought to be a kind of neuroendocrine cell found at the base of the surface of the skin, the epidermis. They’re close to the nerve endings in the skin that let you feel a light touch.

Merkel cell carcinoma happens when something makes them grow out of control. Although scientists don’t fully understand what causes it, they believe the cancer is connected to UV light exposure, a weakened immune system due to disease or age, and the Merkel cell polyomavirus (MCV), which is found in nearly all the tumors of this cancer.

Nearly everyone gets MCV as a child, but it doesn’t cause any symptoms. The virus was discovered in 2008.

Merkel cell carcinoma typically shows up on a person’s face or neck or in other areas that are often exposed to the sun, like the arm. It can also be found in places like inside the nose or esophagus.

It looks like a raised red or purple lump or pimple, unlike melanoma, another serious form of skin cancer that shows up as a dark spot.

A Merkel cell carcinoma may be mistaken for a cyst, but cysts can be painful while these spots often are not.

Buffett, who was 76, had been living with the cancer for four years, according to his official website.

If it’s caught early enough, the chance of living at least five years after diagnosis is pretty good – about 75%, according to the American Cancer Society – but if it has spread past the skin, likelihood of five-year survival dips to 24%.

Although rare, it is the second most common cause of skin cancer death after melanoma, according to the National Cancer Institute. It can be so deadly because it grows and spreads quickly, and it often can come back after treatment, usually within two or three years of diagnosis.

Men are twice as likely to develop this cancer than women. About 90% of the people who have it are White, and about 80% are 70 and older. The risks may be related to how much sun damage a person has had over their lifetime, the American Cancer Society says.

Because so few people have had this form of cancer, research is limited, and it is difficult to know exactly how to fight it. The American Cancer Society recommends that people who have Merkel cell carcinoma at any stage try to enroll in a clinical trial so they will have access to the latest treatments.

If the cancer is in an early stage, doctors will probably do a biopsy of the suspicious spot and then surgically remove the spot and some of the tissues around it. They may also do a biopsy of a nearby sentinel lymph node to detect whether the cancer has spread. The American Cancer Society says cancer is detected in the lymph nodes of 1 in 3 people who have Merkel cell carcinoma.

Doctors may also try chemotherapy and/or radiation, depending on the stage. Patients may get immunotherapy, medicine that helps their body destroy the cancer cells.

There’s no good way to prevent MCV, the common virus linked to Merkel cell carcinoma, but there are good ways to protect yourself from this cancer.

Early detection is key. Examine your skin every month, and get a regular checkup from a doctor. The American Academy of Dermatology offers free skin cancer screenings across the country every year.

Boosting your immune system may help prevent cancer, the CDC says. Get proper sleep, avoid drinking too much, avoid smoking, maintain a healthy weight, eat a healthy diet, and get regular exercise.

There’s also a lot you can do to protect yourself from damaging ultraviolet (UV) rays. Never use a tanning bed. When you’re outdoors, stay in the shade as much as possible, and wear protective clothing, a hat and sunglasses, the American Cancer Society says.

Children should also stay covered up and wear sunscreen. Babies younger than 6 months should be kept out of direct sunlight, since skin damage can accumulate over time, according to the American Academy of Pediatrics.

Even on overcast or cold days, be sure to wear sunscreen on your face. Many lotions and makeup have some form of UV protection.

The US Dermatological Association suggests wearing water-resistant sunscreen with an SPF of 30 or higher.

If you’ll be in direct sun, the Environmental Working Group, a nonprofit consumer advocacy group that has spent more than a decade testing sunscreens, recommends lotion sunscreens over sticks. Apply enough to fill a shot glass – about an ounce – every two hours while you’re in the sun.

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