What is Merkel Cell Carcinoma?

What is Merkel Cell Carcinoma

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How is it Diagnosed

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Stages of Merkel Cell Carcinoma

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Treatment Options

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Helpful Resources

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What is Merkel Cell Carcinoma?

Merkel Cell Carcinoma is a rare but highly aggressive neuroendocrine skin cancer. Merkel Cell Carcinoma arises from uncontrolled growth of cells in the skin that share some characteristics with normal Merkel cells of the skin.

The number of cases of Merkel Cell Carcinoma is increasing rapidly, having quadrupled in the past few decades.

Merkel Cell Carcinoma has the potential to be lethal and appropriate management often should involve several types of therapy such as surgery, radiation, and sometimes systemic medications. Multidisciplinary evaluation of patients with this cancer is critical to determine and carry out a proper treatment plan.

What are the Symptoms?

Merkel Cell Carcinoma usually develops on sun-exposed skin (e.g. head, neck and arms) as a painless, firm lump that can be red-purple or skin-coloured. Patients frequently point out a new Merkel Cell Carcinoma to their doctor because a lump is growing rapidly and looks unusual.

Although these cancers typically arise on sun-exposed skin it can occur anywhere on the body, including sun-protected areas such as the buttock or scalp under hair.

What are the Risk Factors?

Factors strongly associated with the development of Merkel Cell Carcinoma include age over 65 years, fair skin, history of extensive sun exposure, chronic immune suppression (e.g. kidney or heart transplantation or HIV), and the Merkel cell polyomavirus.

Age relation to MCC

The risk of developing Merkel Cell Carcinoma increases with advancing age. 75% of Merkel Cell Carcinoma patients are over the age of 65 years at time of diagnosis, with the average age for developing Merkel Cell Carcinoma being 74. Merkel Cell Carcinoma is slightly more common in men than women for each age group.

Sunlight

It is believed that ultraviolet radiation from the sun plays a significant role in the development of Merkel Cell Carcinoma.

Merkel Cell Carcinoma is most commonly found on sun exposed areas of the body (e.g., head, neck, arms) in older Caucasian individuals, who may also have other sun-induced skin cancers. There are more Merkel Cell Carcinoma cases in sunny climates.

While extensive sun exposure is a risk factor for Merkel Cell Carcinoma, Merkel Cell Carcinoma can also occur on sun-protected skin, such as a hair-covered scalp.

Immune function

Patients with weakened immune systems are at significantly higher risk of developing Merkel Cell Carcinoma. Long-term suppression of the immune system (for many years) appears to be a risk factor for Merkel Cell Carcinoma in some patients. Conditions associated with weakened immunity include HIV/AIDS, kidney or heart transplantation, and autoimmune diseases requiring medications that suppress the immune system, chronic lymphocytic leukaemia (CLL) and certain types of lymphoma. The risk of developing Merkel Cell Carcinoma is 8 times greater in HIV patients, 10 times greater in organ transplant patients, and about 40 times greater in CLL. While patients with profound immune suppression are at a higher risk of developing Merkel Cell Carcinoma, over 90% of all people who develop Merkel Cell Carcinoma have no known immune deficiency.

Merkel cell polyomavirus

The Merkel cell polyomavirus (MCPyV) was discovered by the University of Pittsburgh laboratory of Drs Patrick Moore & Yuan Chang in 2008 and found to be frequently present in Merkel Cell Carcinoma tumours. The virus was found in 8 of 10 tumours tested, and was associated with the DNA of the tumour cells in such a way to suggest that it is involved in the development of Merkel Cell Carcinoma.

Since then, many studies have validated this initial report, finding MCPyV in the vast majority (about 80%) of Merkel Cell Carcinoma patients. Studies now show that the majority of people have been exposed to MCPyV by adulthood, but it appears that the virus does not cause any symptoms except in the very rare situations in which it leads to Merkel Cell Carcinoma.

Merkel Cell Carcinoma in Australia

The incidence of Merkel Cell Carcinoma is highest in Australia and approximately twice that of Europe and US.

While most Merkel Cell Carcinoma cases (approximately 80%) in Europe and North America are associated with the MCPyV, much lower rates (24%) of Merkel Cell Carcinoma tumours with the virus were reported in Australia.

In cases where MCPyV infection is not present, Merkel Cell Carcinoma is triggered by UV damage causing DNA mutations. Australia has particularly high rates of sun exposed Merkel Cell Carcinoma, thought to be due to its higher UV index compared with other countries and highly sun exposed patient population.

Diagnosis

Treatment

Staging