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The diagnosis of MCC is rarely made on clinical grounds. Histological and immunohistochemical studies are usually required to confirm the clinical suspicion. Given the frequent occurrence of occult lymph node metastasis, sentinel lymph node biopsy should be performed once distant metastasis has been ruled out by cross\u2010sectional imaging. Primary tumors without evidence of organ metastases are treated with complete surgical excision with appropriate surgical margins. Radiation therapy should be considered at all stages of the disease.<\/jats:p><jats:p>For advanced MCC that is no longer amenable to curative treatment by surgery or radiation therapy, there is currently no established systemic therapy for which an improvement in recurrence\u2010free survival or overall survival has been demonstrated in a prospective randomized trial. However, immunotherapy using PD\u20101\/PD\u2010L1 blockade seems to be superior to chemotherapy.<\/jats:p><jats:p>Various factors warrant that further diagnostic and therapeutic interventions be determined by an interdisciplinary tumor board. 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