Over the weekend, rock legend Jimmy Buffett passed away following a lengthy battle with a rare form of skin cancer.

In a statement posted to his website, Buffett’s death on September 1 was attributed to Merkel cell carcinoma, which he had been diagnosed with four years ago. Buffett, who did not publicly disclose his diagnosis, remained active by performing until the end of his life.

The death of the “Margaritaville” singer, who famously embodied a ‘beach bum’ aesthetic and ethos, coincided with Labor Day weekend, which is traditionally viewed as the end of summer in the U.S.

Given Buffett’s prominence in popular culture and sudden death due to a rare form of skin cancer, it’s worth exploring what Merkel cell carcinoma is, what risk factors contribute to it and what treatment options remain available to patients.

According to the Cleveland Clinic, Merkel cell is a rare but aggressive form of skin cancer that can be life-threatening. The disease affects around 3,000 Americans per year, manifests on the epidermis, (the skin’s outer layer), and typically spreads to lymph nodes and other organs. 

It’s caused by ultraviolet light exposure, which often comes from being in sunlight long-term. Other causes include a weak immune system, a viral infection or old age. 

Additionally, eight-in-10 patients with Merkell cell carcinoma have the Merkel cell polyomavirus (MCP), a common childhood virus. However, there isn’t a way to screen for MCP and the medical community is continuing to research how the virus causes skin cancer in some people but not others.

The Mayo Clinic stated that the disease’s most common feature is the appearance of flesh-colored or bluish-red nodules on the face, head or neck. The organization added that Merkel cell carcinoma grows fast and spreads quickly to other parts of the body, which makes detection such a key aspect for survival.

The Cleveland Clinic noted that while providers can surgically remove the tumor, Merkel cell carcinoma often comes back. 

Merkelcell.org, a collective of Seattle-based physicians and researchers working on the disease, estimates that one-third of all patients with the disease succumb to it. 

Merkelcell.org also offers a state-by-state registry of specialists to offer patients the best chance to detect the disease early, treat it and defeat it.