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Cutaneous Neuroendocrine (Merkel Cell) Carcinoma


Merkel cell carcinoma (MCC) is the eponymous term for primary neuroendocrine carcinoma of the skin, subcutis, or both.

Merkel Cell Carcinoma

Clinical Findings

Merkel cell carcinoma is uncommon. Its annual age-adjusted incidence in the United States was 0.44 in 100,000 in 2001. MCC typically occurs on chronically sun-damaged skin of elderly white individuals but can also occur in younger patients on sun-protected sites and in individuals with dark skin. The mean age at presentation is 61 years. Most tumors present on the extremities or the head and neck region. The buttocks are also not infrequently involved. Truncal tumors are rare. Approximately 10% of patients present with metastatic disease with no known primary tumor. There is an increased incidence of MCC in immunosuppressed patients (14-fold increased risk among patients with HIV; 10-fold increased risk after solid organ transplantation). Recently, a polyomavirus (Merkel cell polyoma virus) has been found in the majority of Merkel cell tumors.

Merkel Cell Carcinoma—Disease Fact Sheet

Patient Group

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    Mean age at presentation is 61 years

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    Immunosuppressed patients

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      14-fold increased risk in HIV-positive patients

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      Tenfold increased risk after organ transplantation

Site of Involvement

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    Head and neck region

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    Extremities

Clinical Findings

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    Papule, purple or violaceous nodule, or “cystlike” dermal or subcutaneous mass

Prognosis

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    Depends on stage and histologic features

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      Favorable for small circumscribed tumors confined to the dermis

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      High recurrence risk for large tumors with lymphatic tumor emboli

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