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Sharp increase in incidence of a rare but aggressive form of skin cancer in Sweden

News: May 23, 2016

The number of Swedish cases of the rare but fast-growing, life-threatening form of skin cancer Merkel cell carcinoma has doubled in the past 20 years. Such were the results of a recently conducted study at Sahlgrenska Academy, University of Gothenburg.

In the last few decades we have seen an exponential increase in the number of cases of skin cancer in Sweden. The incidence of the three main types of skin cancer – malignant melanoma, squamous cell carcinoma and basal cell carcinoma – are growing by about 5–6% per year.

However, there are a large number of rare forms of skin cancer that are not as well-studied. One of the most significant rare forms of skin cancer is Merkel cell carcinoma (MCC). These skin tumors are fast-growing and life-threatening, which means that very early diagnosis is crucial.

Merkel cell carcinoma

Researchers at Sahlgrenska Academy have studied all cases of Merkel cell carcinoma that were reported to the Swedish Cancer Registry between 1993 and 2012. The cases have been analyzed to investigate demographic, clinical and epidemiological data.

During this 20-year period, 606 cases of MCC where diagnosed in 594 patients. The number of cases per 100,000 in population increased by more than 100%, from an incidence rate of 0.23 to 0.49.

Significant increase

“Our results show that there has been a significant increase of the incidence of MCC in Sweden. An average of 24 new cases of Merkel cell carcinoma were diagnosed per year during the period of 1993–2001. During the period from 2002 until 2011, the figure rose to 34 cases per year and, in 2012, 47 new cases of MCC were discovered in Sweden”, said John Paoli, Dermatologist and Associate Professor at Sahlgrenska Academy.

Low survival rates

97% of the patients had no known spreading to other parts of the body (metastases) in connection with the diagnosis, but despite this, only 67.3% survived 5 years after the diagnosis. Survival rates were significantly lower than expected for the general population of the same age and sex during this period.

Uv radiation

“More than half of all MCCs were located in the head and neck region, which suggests that uv radiation from the sun is of great significance in this diagnosis, just as it is for common forms of skin cancer,” said Oscar Zaar, Dermatologist and Doctoral student at Sahlgrenska Academy.

The study Merkel cell carcinoma incidence is increasing in Sweden was published online in May in the scientific journal Journal of the European Academy of Dermatology and Venereology.


● Merkel cell carcinoma (MCC) is a rare but aggressive form of skin cancer. Early detection and treatment are important.
● The risk of recurrence as well as spreading to the lymph nodes or other organs is relatively high after surgery and/or radiation therapy.
● There are links between these skin tumors and light skin type, sun exposure, high age and a compromised immune system, as well as a clear link to a virus, called the Merkel Cell Polyomavirus.
● MCC often starts with the sudden growth of a bump on or under the skin that is red, pink or violet in color and relatively hard. The tumor rapidly grows into a nodule. Bleeding can occur at more advanced stages.

John Paoli, Dermatologist and Associate Professor at Sahlgrenska Academy, University of Gothenburg

Oscar Zaar, Dermatologist and Doctoral student at Sahlgrenska Academy, University of Gothenburg



Originally published on: sahlgrenska.gu.se


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