CLEVELAND, Ohio — The death due to skin cancer of Jimmy Buffett, whose music evoked a vibe of sun, sand and island life, has thrust into the spotlight the dangers from sunlight exposure and what routinely can be done to limit the risk.
Buffett died Sept. 1 at age 76 after battling a form of skin cancer known as Merkel cell carcinoma, primarily thought to be caused by sun exposure.
Here in Ohio, it can be tempting to soak up the sun whenever it makes an appearance. But doctors say protecting yourself from the sun is the best protection against skin cancers, including Merkel cell carcinoma.
“Things like staying out of the sun in the brightest part of the day, wearing sunscreen and reapplying that frequently, especially if you are swimming or sweating, and wearing barrier clothing like hats or other clothing,” Lucy Boyce Kennedy, an oncologist who specializes in high-risk skin cancers at the Cleveland Clinic, said in explaining preventative measures.
Merkel cell carcinoma is a rare form of skin cancer that develops just beneath the skin and in hair follicles. Between 2,000 and 3,000 cases of Merkel cell cancer are diagnosed in the U.S. every year. In comparison, roughly 60,000 new cases of malignant melanoma and over 1 million new cases of nonmelanoma skin cancer are diagnosed annually.
It commonly appears in areas exposed to the sun such as the head, neck, arms and legs. It occurs mostly in Caucasians aged 60 to 80 and is seen about twice as often in men than women.
Less frequently, it is found in younger people with suppressed immune systems, like transplant patients, and patients with HIV. However, in people with normal immune systems, the more time a person spends in the sun, the more likely they are to develop this rare form of cancer.
It usually shows up as a firm, flesh-colored bump, said Boyce-Kennedy, and because it’s so rare, it’s also frequently misdiagnosed.
“We do see patients where Merkel cell has been thought to be a cyst or another benign lesion,” she said, adding that it is a particularly aggressive form of skin cancer, and can grow and change quickly.
“If a patient were to notice something on their skin that was changing rapidly, it’s always prudent to have your dermatologist or other doctor take a look at it,” Boyce-Kennedy said.
Like other more common skin cancers, damage caused by ultraviolet radiation from the sun or tanning beds converts healthy skin cells to cancerous ones. However, in the case of Merkel cells, a virus is frequently also involved.
About 80% of Merkel cell tumors are infected with the Merkel cell polyomavirus. And although it is still poorly understood, researchers believe this plays a role in changing the genetics of the Merkel cells, making it easier for them to become cancerous.
But the picture isn’t quite that simple. Merkel polyomavirus is extremely common. It turns out that almost everyone gets it eventually, and yet most of them will never develop cancer.
“We know that the virus has a role to play in the development of Merkel cell cancer, but is it the cause of Merkel cell? I don’t think that’s entirely correct,” explained Dr. Ankit Mangla, an oncologist at University Hospitals Seidman Cancer Center.
It can be successfully treated if it is caught in time. However, many people often fail to recognize the signs, and wait too long for diagnosis and treatment, mistaking skin cancer for age spots, or other non-cancerous conditions. Early-stage Merkel cell cancers have a good prognosis, said Mangla, and even more so since the introduction of a class of drugs known as check-point inhibitors.
The first of these drugs, avelumab, was approved for use in Merkel cell cancer in March of 2017. Two more have received FDA approval to treat Merkel cell cancer since.
These new drugs have revolutionized the treatment, says Mangla, in part because they are much more effective than chemotherapy, which he says never worked very well in Merkel cell cancers.
He said the side effects are frequently much more tolerable, or non-existent, than those of chemotherapy, a plus for older patients who might not be good candidates for chemotherapy.
“I am treating a 91-year-old with stage 4 Merkel cell cancer right now,” said Mangla.
There has been a growing understanding over the last decade or so that the immune system plays an important part in regulating cancer by recognizing rogue cancer cells and destroying them before they can proliferate and form tumors.
It’s one of the reasons that people with immune deficiencies are prone to developing cancer. But certain cancers have developed ways to evade immune recognition in people with healthy immune systems too, and this is where the drugs come in — by blocking the cancer cells’ cloaking devices.
In the meantime, doctors agree that prevention is the best medicine, especially for fair-skinned people who are frequently out in the sun. The best way for anyone to prevent Merkel cell cancer, or any skin cancer for that matter, is cheaper and easier than any cancer drug: cover up, and wear sunscreen.