Since the first report of cutaneous neuroendocrine carcinoma (Merkel c
ell carcinoma) in 1972, knowledge of the biological behavior of this t
umor has continued to increase. This study reviews three previously un
reported cases in addition to reports on 429 patients collected from t
he literature. The presenting features of these 306 patients were anal
yzed for frequency and then were analyzed by the Cox proportional haza
rd model for the relationship of each to recurrence and outcome. This
study confirms that cutaneous neuroendocrine carcinoma is an aggressiv
e disease; that the most common cause of death in these patients is by
distant metastasis; and that men have succumbed more frequently than
women. As a result of this study, aggressive treatment of cutaneous ne
uroendocrine carcinoma is recommended, especially in the male patient.