Jimmy Buffett, the singer-songwriter known for songs such as “Margaritaville” and “A Pirate Looks at 40,” died on Sept. 1 at the age 76 at his home in Sag Harbor, Long Island, four years after receiving a diagnosis of Merkel cell carcinoma (MCC).
One of the singer’s daughters, Delaney Buffett, took to social media on Wednesday to share details about her father’s last days.
“Despite the pain, he smiled every day,” she wrote in a message posted to Instagram. “He was kind when he had every excuse not to be. He told us not to be sad or scared, but to keep the party going.”
According to the American Cancer Society, MCC, also referred to as neuroendocrine carcinoma of the skin, trabecular carcinoma or trabecular cancer, is both a rare type of skin cancer and one of the most dangerous, more likely than common skin cancers to spread to other parts of the body and potentially very difficult to treat if it spreads.
“We can talk about the bad but also the good, (which is) that it is a potentially curable cancer if caught early,” Dr. Manisha Thakuria, director of the Merkel cell carcinoma clinic at the Dana-Farber Cancer Institute and assistant professor of dermatology at Harvard Medical School, told CURE®. “And we cure lots of patients with Merkel cell carcinoma who are living great lives. But it can be a really nasty cancer … when it’s caught late, when there are certain tumor characteristics that that that make it worse and certain host or patient characteristics that maybe make you more vulnerable.
“So, in terms of how fast (it spreads) or what’s the prognosis of someone with Merkel cell, it’s really entirely dependent on their stage. So, at the time that they’re diagnosed, is that early stage, stage 1, which has an excellent prognosis? The vast majority of those patients are cured, even though we do follow them for at least five years, just to be sure that that they don’t have a recurrence. But some patients who have rapidly spreading disease that is diagnosed and it’s already stage 3 or stage 4. Some of those patients really don’t do well. And I would say that when Merkel cell is bad, it is probably one of the worst. It’s a really heartbreaking, horrible thing to see how fast it can spread.”
Merkel cell carcinoma, Thakuria said, is a very rare cancer, affecting approximately 2,400 patients in the United States per year.
“That number is increasing at a rate of about 8% per year,” she said. “So, it’s a rapidly increasing incidence. However, just to put it into perspective, melanoma, which is the skin cancer that most people have heard of as the dangerous skin cancer, will affect over 100,000 people in 2023, and probably double that number if you include the in-situ melanomas, which are fully curable cancers, usually with simple surgeries.
“And so, Merkel cell really is like a totally different ballgame, because it’s so rare. It really is truly an orphan cancer. … A lot of patients haven’t heard of it. A lot of doctors haven’t even heard of it. Dermatologists certainly are aware of the disease. And I specialize in Merkel cell carcinoma, but I am also a practicing dermatologist who does general derm(atology) and I think it’s a diagnosis that sort of strikes fear in the hearts of many because it’s a diagnosis you don’t want to miss — but (it) can be hard to catch early.”
“It’s very common for a dermatologist to see a lesion on the skin and understand that there’s something abnormal here,” Dr. Brent Moody of the Skin Cancer Surgery Center in Nashville told CURE in 2021. We need to biopsy this lesion and figure out what it is. More likely than not, we’re going to think that it’s one of the other more common types of skin cancer, and usually we just get back a pathologic diagnosis.
“(MCC) doesn’t have a typical appearance, per se, but generally, they’re going to be pinkish red bumps, more common in sun-exposed areas. The head and neck are the most common areas, but they can be anywhere head to toe. My general rule of thumb, as a dermatologist specializing in skin cancer, is that if you have a new skin lesion, you need to see your dermatologist to have that evaluated.”
TMZ, which broke the news of Buffett’s cause of death, reported that he began receiving hospice care less than two weeks before his death and his skin cancer had “turned into lymphoma.” Thakuria disputed the latter point.
“That’s just not accurate,” she said. “One cancer doesn’t turn into another cancer. That’s not a thing. … But Merkel cell carcinoma, certainly, it does go to lymph nodes, it likes to go to lymph nodes, that’s a very common way that it metastasizes. It also can metastasize through the bloodstream. So, it does sort of favor going to places like the liver, bone, other distant lymph nodes, it can go to even lung, it can go to brain but that that doesn’t happen quite as often as the other counterpart in skin cancer, melanoma.”
Risk factors of MCC
“These cancers most often start on skin that’s exposed to the sun, like the face (the most common site), neck and arms,” according to the American Cancer Society’s website. “But MCC can start anywhere on the body. Merkel cell tumors often look like firm, pink, red, or purple lumps or bumps on the skin. They usually don’t hurt, but they’re fast-growing and can sometimes open up as ulcers or sores. Nearly all MCCs start on the skin, but a very small portion start in other parts of the body, such as inside the nose or esophagus.”
Per the American Cancer Society, risk factors for MCC include Merkel cell polyomavirus infection, exposure to ultraviolent rays (with sunlight as the main source) and having light-colored skin and a weakened immune system.
MCC is very rare among people younger than age 50, and approximately 80% of patients with MCC are older than age 70 — and men are almost twice as likely to develop MCC.
Importance of a multidisciplinary care team
Thakuria recommended that patients who receive a diagnosis of MCC see an oncologist who specializes in that disease type as part of a multidisciplinary care team.
“I think going to your local academic center is probably a good idea, even just for a consult, to get some advice and discussion, but I’ve been, in some cases, very pleasantly surprised by excellent, multidisciplinary care at (cancer) centers that aren’t as big,” she said. “But I think because this is such a rare disease, it is truthfully hard to find experts outside of big centers. Because the patient volume is just so, so low, it is hard to develop an expertise in something that’s so rare.”
In an obituary posted to Buffett’s website, his family requested that, in lieu of flowers, donations be made to his foundation, Singing for Change, Brigham and Women’s Hospital and Dana-Farber Cancer Institute in Boston or The University of Texas MD Anderson Cancer Center in Houston.
“My dad repeatedly told us how much he appreciated the doctors, nurses and every person who was there for him during this battle with cancer,” Delaney Buffett wrote. “So to those who took care of my dad at home and in the hospital, I want to thank you for giving us more time together. I am eternally grateful.”
“It’s always hard to find silver linings in losses, but I am glad to see Merkel cell having a little bit of a spotlight on it, and I hope that it will increase research funding for Merkel cell,” said Thakuria. “Things are a lot better now than when I started doing this about 12, 13 years ago, in terms of industry interest in Merkel cell; when I started, there was zero interest, and we were borrowing regimens for our stage 4 patients from other cancers, because there were no specific ones that were known to work well in Merkel cell and now that’s changed.”
Adjuvant therapy (given after primary treatment) — with the immunotherapy (treatment that stimulates or suppresses the immune system to help a patient’s body target cancer cells) Opdivo (nivolumab) improved the rates at which patients with Merkel cell carcinoma survived without signs of the disease compared with observation, according to findings from a recent study published in The Lancet.
The PD-1 inhibitor Zynyz (retifanlimab-dlwr) was approved by the Food and Drug Administration (FDA) for the treatment of adults with metastatic or recurrent MCC earlier this year. The anti-PD-1 therapy Keytruda (pembrolizumab) was approved by the FDA for the treatment of adult and pediatric patients with recurrent locally advanced or metastatic MCC in 2018, preceded by a 2017 approval of Bavencio (avelumab), a PD-L1-inhibitor, for the treatment of adult and pediatric patients over the age of 12 with metastatic MCC.
“The advent of immunotherapy as a treatment for Merkel cell is probably one biggest advances that has ever been, and it might be the biggest advance that any person who works in cancer has ever seen,” Thakuria said, “and in Merkel cell it’s particularly true because Merkel cell is one of the most responsive to immunotherapy.”
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