What is Merkel cell carcinoma (MCC)?

Updated: Oct 07, 2019
  • Author: Guy J Petruzzelli, MD, PhD, MBA, FACS; Chief Editor: Gregory Gary Caputy, MD, PhD, FICS  more...
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Merkel cell carcinoma

Merkel cell carcinoma (MCC) is a rare cutaneous malignancy that is aggressive and often metastasizes to regional lymph nodes. The pathophysiology of this disease process appears to have an infectious etiology, as evidenced by Feng, who reported clonal integration of a polyomavirus in human MCC. [19] Merkel cell polyomavirus (MCPyV) is unique in the 10-member family of human polyomaviruses (HPyV) in that it causes cancer, particularly in the old and immunosuppressed patient. [20]  It is found in 60-80% of specimens of Merkel cell cancer. [21, 22]

The rarity of this malignancy is further emphasized by data obtained from the Surveillance, Epidemiology, and End Results program, through which Miller and Rabkin determined that the age-adjusted prevalence of MCC is 0.23 cases/100,000 persons and 0.01 cases/100,000 persons for individuals who identify as white or black, respectively. [23]

The average age of onset for MCC is between the late sixth and early seventh decade of life. As with BCC and SCC, MCC has a higher prevalence in patients who are immunosuppressed, with a median age within the fifth decade in this population. [24, 25]  Interestingly, Friedlaender and colleagues observed that in organ transplant patients, the discontinuation of the immunosuppressant cyclosporine caused temporary regression of MCC metastases. [26]

A 2009 study based on 1980-2004 data from a population-based cancer registry showed increases in standardized incidence rates for rare skin tumors, including MCC. [27]  A 2007 report by Bichakjian noted that the incidence of MCC had tripled in the previous 20 years, which was likely attributable to increasing lifespans. [28]

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