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Merkel Cell Carcinoma (MCC) is a skin cancer, but while most skin cancers are nothing to fear, MCC is one with a high mortality rate. Roughly 1/3 of those diagnosed with MCC die.
What is Merkel Cell Carcinoma?
Merkel cell carcinoma (MCC), sometimes referred to as a neuroendocrine carcinoma of the skin, arises from the uncontrolled growth of Merkel cells in the skin. It is a rare skin cancer with roughly 1500 cases per year in the United States, making it about 40 times less common than melanoma. MCC has the potential to be lethal, and thus prompt aggressive treatment is warranted. Merkel cell carcinoma (MCC) occurs most often on the sun-exposed face, head, and neck.
MCC is associated with immunosuppression, UV exposure, age over 50 and a newly discovered polyomavirus that is common on the normal-appearing skin of most people. The cause of MCC has not yet been firmly established, and therapeutic interventions at late stage disease are frustratingly ineffective. Researchers are investigating immune evasion strategies employed by MCC, and how to overcome them using immunotherapy approaches — which as the potential to help anyone with cancer.
MCC is caused by a virus in 8 out of 10 cases. It’s a virus that many get as children, but the virus causes cancer only rarely. A series of molecular events must occur for tumors to develop, and each of these events is rare. Various studies provide hope that the human immune system one day could be recruited to help clear tumors in some patients. Researchers are looking for ways to stimulate virus-specific immunity.
What does MCC look like?
MCC usually develops on sun-exposed skin as a firm, painless, flesh-colored to red-violet bump. The initial small bump tends to grow rapidly over weeks to months.
What is a Merkel cell?
Merkel cells are found in the epidermis (outer layer of the skin). Although the exact function of Merkel cells is unknown, they are thought to be touch receptors. They have both sensory and hormonal functions and are sometimes referred to as neuroendocrine cells.
Who gets MCC?
Individuals over the age of 65 are far more likely to develop MCC. Fair skin and a history of extensive sun exposure also increase the chances. This cancer is found most commonly 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. MCC is associated with weakened immune function, such as in patients with HIV or organ transplants. However, most people who get MCC are not immune suppressed.
What immune problems make beating MCC more difficult?
People with severe defects in "T lymphocytes" (key cells of the immune system) have a 10-30 times greater chance of developing MCC and twice the chance of losing the battle with MCC compared to people with a normal immune system. The conditions that cause such severe T lymphocyte defects include HIV (AIDS), taking immune suppressive drugs after solid organ transplantation, and certain immune cancers such as chronic lymphocytic leukemia. In contrast, suffering from more "colds" than average will not importantly affect your ability to fight MCC.
Research
The leading research team in the world is based in Seattle under the direction of Paul Nghiem, MD, PhD. Dr. Nghiem oversees both the clinic and research teams at the University of Washington and Fred Hutchinson Cancer Research Center in Seattle. He has cared for over 500 patients with MCC and has read, lectured and written about this uncommon and challenging disease. His contributions to the MCC literature can be viewed at his laboratory website at http://www.pnlab.org.
Get connected
• Google Group - Online support group for those diagnosed with MCC and their families http://groups.google.com/group/merkelcell
• MerkelCell.org - Web site run by MCC researchers
• Wikipedia page
• Merkel Cell Fund Facebook group
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