Merkel cell carcinoma is a rare and aggressive cutaneous malignancy of neuroendocrine origin-an often-missed diagnosis due to the wide histopathologic differential diagnosis of malignant small blue cell tumors. The advent of electron microscopy and immunohistochemistry staining for cytokeratin 20, a shared neuroendocrine marker, greatly improved diagnostic accuracy. Over the past decade, staging, treatment, and surveillance of the cancer have progressed at a remarkably rapid pace. Herein, the authors provide an update on the current guidelines around diagnosis and management and review the exciting advancements on the horizon.
Keywords: Guidelines; Immunotherapy; Merkel cell carcinoma; Merkel cell polyomavirus; Neuroendocrine tumor.
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