What to know about Merkel cell carcinoma, the rare skin cancer that led to the death of Jimmy Buffett

“Margaritaville” singer Jimmy Buffett died on Sept. 1 after a four-year battle with a rare skin cancer.

Buffet’s website revealed he had been diagnosed with Merkel cell carcinoma, but had been able to perform until recently, when his health began to decline.

Here’s what you need to know about MCC, including signs and symptoms, what causes it and how it’s treated.

PHOTO: Microscopic image of a Merkel cell carcinoma, a highly aggressive type of skin cancer.

Microscopic image of a Merkel cell carcinoma, a highly aggressive type of skin cancer.

David A. Litman/Shutterstock

What is MCC?

Merkel cell carcinoma is much less common than the main types of skin cancer, such as basal cell carcinoma, squamous cell carcinoma and melanoma.

MCC is very rare — about 40 times rarer than melanoma — with an estimated 3,000 cases every year in the U.S., according to the Skin Cancer Foundation (SCF).

MCC is a neuroendocrine cancer, meaning it is a cancer of the cells that are connected to nerves, which likely play a role in touch sensation.

It is a rare, aggressive form of cancer that has a high risk of recurring and spreading within two to three years of being initially diagnosed, the SCF says.

What are the symptoms?

The first symptom of MCC is typically a fast-growing, painless tumor on part of the skin that is sun-exposed, according to the National Cancer Institute.

The nodules are usually shiny or pearly and may appear skin-colored in

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