BACKGROUND: Merkel cell carcinoma is a rare, aggressive, malignant primary cutaneous neuroendocrine tumor. The objective of this study was to evaluate the epidemiological profile of patients with Merkel cell carcinoma, the clinical characteristics of the tumor, time between manifestation of signs and symptoms and initiation of treatment, survival and causes of death. METHODS: Thirty-two patients with Merkel cell carcinoma were evaluated retrospectively. Clinical history and staging were correlated with 1 and 2-year survival. RESULTS: Most patients (69%) were female, mean age was 72 years and 93% were fair-skinned. The most commonly affected sites were the head/neck, trunk and limbs. Other malignancies were found in 6 patients. Mean time between the appearance of signs/symptoms and initiation of specialist treatment was 12.2 months, with regional lymph nodes being affected at that time in 13 (40%) cases and distant metastases being present in 4 (12%). Following specialist treatment, 1 and 2-year survival was 53% and 47%, respectively. Tumor size < 2 cm was indicative of more favorable prognosis. CONCLUSIONS: Late diagnosis contributed to high lethality due to rapid local and distant progression of the disease.
Neuroendocrine carcinoma; Merkel cell carcinoma; Prognosis
