Merkel Cell Carcinoma Clinical Practice Guidelines in Oncology NCCN Categories of Evidence and Consensus Category 1: The recommendation is based on high-level evidence (e.g., randomized controlled trials) and there is uniform NCCN consensus. Category 2A: The recommendation is based on lower-level evidence and there is uniform NCCN consensus. Category 2B: The recommendation is based on lower-level evidence and there is nonuniform NCCN consensus (but no major disagreement). Category 3: The recommendation is based on any level of evidence but reflects major disagreement. All recommendations are category 2A unless otherwise noted. Clinical trials: The NCCN believes that the best management for any cancer patient is in a clinical trial. Participation in clinical trials is especially encouraged. Overview Merkel cell carcinoma (MCC) is a rare, aggressive cutaneous tumor that combines the local recurrence rates of infiltrative non-melanoma skin cancer along with the regional and distant metastatic rates of thick melanoma.1–16 Several large reviews document the development of local recurrence in 25% to 30% of all cases of MCC, regional disease in 52% to 59%, and distant metastatic disease in 34% to 36%.1,16,17 MCC has a mortality rate that exceeds that of melanoma;18 overall 5-year survival rates range from 30% to 64%.3,19 A history of extensive sun exposure is a risk factor for MCC. Older white men (≥ 65 years) are at higher risk for MCC, which tends to occur on the areas of the skin that are exposed to sun.20 The NCCN Non-Melanoma Skin Cancer Panel has developed guidelines outlining treatment of...

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