What to Know About Merkel Cell Carcinoma, the Rare Cancer That Killed Jimmy Buffett

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"Margaritaville" singer Jimmy Buffett died of Merkel cell carcinoma, a rare but aggressive form of skin cancer, according to a tribute posted on his official website.

The 76-year-old music icon was diagnosed with the disease 4 years ago and had been undergoing treatment.

MedPage Today asked Raman Madan, MD, a dermatologist and assistant clinical professor at Northwell Health in New York, to weigh in on what physicians and patients should know about the uncommon diagnosis.

Indeed, Merkel cell carcinoma is incredibly rare, Madan told MedPage Today. He may see a patient diagnosed with Merkel cell carcinoma once every few years, while he may see a patient diagnosed with melanoma, a much more common form of skin cancer, once a week.

"I very rarely, if ever, think 'does this person have Merkel cell?'" Madan said.

However, the same rules apply in identifying abnormalities on the skin that warrant a biopsy.

Merkel cell carcinoma often resembles other forms of skin cancer, and a biopsy indicating the diagnosis can be surprising given how rare cases are, Madan said.

In fact, there are only around 2,000 cases of Merkel cell carcinoma diagnosed in the U.S. each year, according to the American Cancer Society. As for the most common forms of skin cancer, there are some 5.4 million basal and squamous cell skin cancers diagnosed each year in about 3.3 million Americans.

Merkel cell carcinoma starts when cells in the skin -- known as Merkel cells for the German doctor Friedrich Merkel who first described them -- begin to grow out of control.

Merkel cells are "thought to be a type of skin neuroendocrine cell, because they share some features with nerve cells and hormone-making cells," according to the American Cancer Society. Merkel cell carcinoma "tends to grow quickly and can be hard to treat if it spreads beyond the skin."

"I think the big thing here is, it is more aggressive," Madan said. "We don't know exactly why it is more aggressive."

Based on staging-related data from individuals diagnosed from 2012 to 2018, the 5-year survival rate for Merkel cell carcinoma was 75% if localized, 61% if regional, and 24% if distant, according to the American Cancer Society.

For early-stage Merkel cell carcinoma, treatment may consist of simply removing the cancer. In such cases, the 5-year survival rate is often high. However, other early-stage skin cancers have even higher 5-year survival rates approaching 100%.

The issue is that Merkel cell carcinoma "can spread quickly," Madan explained. If it reaches the lymph nodes or other parts of the body, patients are likely to also require chemotherapy or radiation.

Typically, Merkel cell carcinoma is painless early on, though later stages of the disease can lead to symptoms like weight loss, night sweats, and enlarged lymph nodes.

Merkel cell carcinoma is "much more common in white patients over the age of 50 who have had a lot of sun exposure," Madan said.

Additionally, research has shown that a virus called Merkel cell polyomavirus has been detected in 80% of Merkel cell carcinomas, though Madan noted that the presence of the virus often does not lead to cancer.

Furthermore, while overall figures remain incredibly low, the number of people diagnosed with Merkel cell carcinoma has been rising in recent decades. That could have to do with improved accuracy of diagnostic tests, or it could be due to increases in risk factors, according to the American Cancer Society's website: "For instance, people are living longer, and more people are living with weakened immune systems."

Bottom line, even though it is an aggressive form of cancer, "You shouldn't live in fear of having Merkel cell," Madan said.

As for Buffett, the tribute on his official website reads in part, "He filled arenas with fans who called themselves 'Parrot Heads,' and popularized a signature blend of folk, country, and Caribbean music with lyrics that often reflected [his] world travels."

Buffett continued to play shows throughout treatment for his cancer, the tribute noted. His last show was a surprise performance in Rhode Island in early July.

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