Merkel Cell Carcinoma (2024)

Translation: Merkel Cell Carcinoma (1)Merkel Cell Carcinoma (2)Merkel Cell Carcinoma (3)

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A Rare, Aggressive Skin Cancer

Merkel cell carcinoma (MCC) is a rare, aggressive form of skin cancer with a high risk for returning (recurring) and spreading (metastasizing), often within two to three years after initial diagnosis.

How rare is it? Approximately 3,000 new cases of Merkel cell skin cancer are diagnosed annually in the U.S. Experts expect that this will increase to 3,250 cases diagnosed annually by 2025.

Merkel cell carcinoma (MCC) is 40 times more rare than melanoma, with an estimated one case per 130,000 people in the U.S.

Risk factors for MCC include sun exposure, light skin, age over 50 and a weakened immune system.

Singer-songwriter Jimmy Buffett battled MCC for four years prior to passing away on September 1, 2023. Buffett continued to perform throughout treatment for the disease before his death at age 76.

What is a Merkel cell?

Merkel cells are located deep in the top layer of skin. Merkel cells are connected to nerves, signaling touch sensation as “touch receptors.” MCC was named after Merkel cells due to the similar microscopic features; however, recent research suggests that it is unlikely that MCC originates directly from normal Merkel cells.

Merkel Cell Carcinoma (4)

Normal Merkel cells in the skin: In this illustration of a cross-section of the skin, normal Merkel cells are shown in red and connect to nerves shown in yellow. The structures drawn include the epidermis (upper third), dermis (middle), and deeper adipose layer containing the fatty tissue. Arteries are depicted in red and veins are blue. Figure copyright by Paul Nghiem & Quade Medical Group.

What does MCC look like?

MCC tumors often, but not always, appear on sun-exposed areas of the body. The tumors are not nearly as distinctive as other skin cancers and can appear as a pearly pimple-like lump, sometimes skin-colored, red, purple or bluish-red, though they are rarely tender to the touch. The rapid speed at which they grow is what often causes patients and doctors to take notice.

Merkel Cell Carcinoma (5)

A recurrence of Merkel cell carcinoma on the forehead.


Merkel Cell Carcinoma (6)

Merkel cell carcinoma on the lower leg.


Please note: Since not all Merkel cell carcinomas have the same appearance, these photos serve as a general reference for what MCC can look like. If you see something new, changing or unusual on your skin, schedule an appointment with your doctor. Photos courtesy of Paul Nghiem, MD, PhD

How dangerous is MCC?

While MCC is about three to five times more likely to be deadly than melanoma, with early detection, MCC can be treated successfully. If you think you might have MCC, see your doctor. Treatment becomes increasingly difficult once the disease has spread, but new options are now available. Thanks to advances in the field of immunotherapy, MCC survival rates are improving.

A survivor's story

When Luis found out he had Merkel cell carcinoma, a rare form of skin cancer, he was determined to beat it. He shares his harrowing journey from diagnosis to treatment.


Reviewed by:

Sandra D’Angelo, MD
Paul Nghiem, MD, PhD

Last updated: September 2023

This section is made possible with support from

Merkel Cell Carcinoma (7)

Insights on progress in detection, monitoring and treatment of patients with Merkel cell carcinoma.

Q&A with MCC expert Dr. Paul Nghiem

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Merkel Cell Carcinoma (2024)

FAQs

What is the survival rate for Merkel cell carcinoma? ›

When Merkel cell carcinoma is discovered and treated early, and hasn't spread to other parts of the body, about 75% of people live for at least 5 years after diagnosis. The outcomes tend to be worse if the cancer spreads; thus, early detection and treatment are important.

What are the early signs of Merkel cell carcinoma? ›

What are the signs of Merkel cell carcinoma?
  • About the size of a dime and growing quickly.
  • Dome shaped or raised.
  • Firm.
  • Itchy.
  • Similar to a pimple (acne) or insect bite.
  • Skin colored or red, purple or bluish-red.
  • Tender or sore.

Is Merkel cell more aggressive than melanoma? ›

While MCC is about three to five times more likely to be deadly than melanoma, with early detection, MCC can be treated successfully. If you think you might have MCC, see your doctor. Treatment becomes increasingly difficult once the disease has spread, but new options are now available.

Is Merkel cell cancer curable? ›

Merkel Cell Carcinoma Treatment. Merkel cell carcinoma is frequently curable with surgical and nonsurgical therapies, particularly if caught early. Treatments are often highly individualized, depending on a patient's general health, as well as the tumor's location, size, depth, and degree of spread.

What causes death with Merkel cell carcinoma? ›

Merkel cell carcinoma tends to travel first to nearby lymph nodes. Later it may spread to your brain, bones, liver or lungs, where it can interfere with the functioning of these organs. Cancer that has metastasized is more difficult to treat and can be fatal.

How long does it take for Merkel cell carcinoma to metastasize? ›

The mean latency from the primary tumor diagnosis to systemic metastasis was 2.1 years and the mean latency between the radiologic diagnosis of the metastases and death was 299 days. Metastases were recorded in several organ systems in most of the cases, and at least two separate metastatic sites in 63% of the cases.

How long did Jimmy Buffett have Merkel cell carcinoma? ›

After battling skin cancer for four years before his death, "Margaritaville" singer Jimmy Buffett died on September 1, 2023, at age 76, from Merkel cell carcinoma, according to his website.

What can be mistaken for Merkel cell? ›

Because Merkel cell carcinomas can mimic other skin appearances, it has a high risk of being misdiagnosed. About 56% of Merkel cell carcinomas are thought to be noncancerous when first examined. 4 They can easily be mistaken for cysts or infected hair follicles.

What is the average age for Merkel cell disease? ›

Age is a risk factor for most types of cancer, including MCC. The average age of diagnosis of MCC is around 70 years old.

Who is most at risk for Merkel cell? ›

Merkel cell carcinoma (MCC) is a rare, aggressive neuroendocrine tumor of the skin with increasing incidence. It most frequently presents on the head and neck region of elderly, white males.

What virus causes Merkel cell carcinoma? ›

The Merkel cell polyomavirus (MCPyV)

The virus was found in 8 of 10 tumors tested, and was associated with the DNA of the tumor cells in such a way to suggest that it is involved in the development of MCC.

Does Merkel cell run in families? ›

Researchers don't yet know all of the DNA changes that can result in MCC, but they have found that many of these cancers have changes in tumor suppressor genes. MCC does not seem to run in families, so the DNA changes that lead to MCC are not likely passed on (inherited) from a person's parents.

What does early Merkel cell carcinoma look like? ›

MCC usually develops on sun-exposed skin (e.g., head, neck, arms) as a painless, firm bump that can be red-purple or skin-colored. Patients frequently point out a new MCC to their doctor because a bump is growing rapidly and/or does not look like anything the patient has ever had before.

Does Merkel cell carcinoma show up in blood work? ›

Blood tests

People with MCC might also have their blood tested for antibodies to the Merkel cell polyomavirus (MCV) around the time they start treatment. For people who have antibodies to MCV, the levels should fall over time if treatment is working.

What is a Stage 4 Merkel cell carcinoma? ›

The tumor has spread to skin that is not close to the primary tumor or to other parts of the body, such as the liver, lung, bone, or brain.

Is Merkel cell carcinoma terminal? ›

MCC is a fatal disease, and patients have a poor chance of survival. Moreover, MCC lacks distinguishing clinical features, and thus by the time the diagnosis is made, the tumour usually have metastasized. MCC mainly affects sun-exposed areas of elderly persons.

What are the odds of getting Merkel cell carcinoma? ›

First, some good news: Merkel cell cancer is exceptionally uncommon. Roughly 1,600 cases occur in the US annually.

What is the average age for Merkel cell carcinoma? ›

Age is a risk factor for most types of cancer, including MCC. The average age of diagnosis of MCC is around 70 years old.

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