Merkel cell carcinoma (MCC) is a rare primary cutaneous carcinoma of the skin who has high aggressiveness, high risk of locoregional and distant spread, a mortality rate considerably higher than that of cutaneous melanoma and a poor survival. Its incidence has increased during the past twenty years. The studies published from 2008 to early 2012 have introduced interesting changes in the understanding of its epidemiology, pathogenesis and consequently in the diagnostic codes and the therapeutic approach. Early and detailed nodal diagnosis with posterior multidisciplinary decision is mandatory. Surgery and Radiotherapy play a fundamental role in the management of this tumor. Both are associated with improved locoregional control and disease free survival; but patients continue to have distant failure because, currently, there is no effective systemic treatment available. Consequently, there remain controversies about its appropriate management, and this review is an attempt to contribute to their clarification.
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