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Clinical features and diagnosis of Merkel cell (neuroendocrine) carcinoma and sebaceous cell carcinoma of the skin

INTRODUCTION

Merkel cell carcinoma (MCC) of the skin is a rare, aggressive cutaneous malignancy that predominantly affects elderly Caucasians and has a propensity for local recurrence and regional lymph node metastases. A variety of other terms have been used to describe this tumor, including neuroendocrine or primary small cell carcinoma of the skin, trabecular cell carcinoma, and anaplastic cancer of the skin.

The epidemiology, pathogenesis, clinical features, and diagnosis of MCC are discussed here. Sebaceous carcinoma arising in the skin is also discussed here. The staging and treatment of patients with MCC are discussed separately. (See "Staging and treatment of Merkel cell (neuroendocrine) carcinoma".)

EPIDEMIOLOGY AND RISK FACTORS

MCC was first described in 1972 [1]. Epidemiologic studies based upon information from the Surveillance, Epidemiology and End Results (SEER) database have shown a dramatic rise in incidence, with a rate of increase of about 8 percent per year and a tripling of the overall incidence between 1986 and 2001 [2,3].

MCC is predominantly a disease of whites, with an annual age-adjusted incidence of 0.23 per 100,000 for Caucasians and 0.01 for blacks [4]. Among whites, the ratio of melanoma to MCC is approximately 65 to 1. Incidence rates are higher in males compared to females (0.65 and 0.26 cases per 100,000 person-years, respectively, in 2001).

MCC is typically seen in older individuals, with a median age at diagnosis of about 70 years [1]. However, MCC occurs at a younger age in immunosuppressed subjects, including organ transplant recipients, patients with B cell malignancies, and those infected with HIV. (See 'Immunosuppression' below.)
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