Merkel cell carcinoma of the skin.

BACKGROUND: Neuroendocrine/Merkel cell carcinoma (MCC) of the skin is an uncommon tumour. Currently, there are only limited data available on the natural history, prognostic factors, and patient management of MCC.

OBJECTIVES: To review our experience and build the largest database from the literature.

METHODS: Twenty-eight cases from the London Regional Cancer Center were combined with 633 cases obtained from the literature searched in English, French, German, and Chinese for the years 1966 to 1998. The database included age, sex, initial disease status at presentation to the clinic, site of primary, any coexisting disease, any previous irradiation, sizes of primary/nodal/distant metastases, management details, and final disease status. A new modified staging system was used: stage Ia (primary disease only, size > 2 cm), stage Ib (primary disease only, size > 2 cm); stage II (regional nodal disease), and stage III (beyond regional nodes and/or distant disease).

RESULTS: Age > 65 years, male sex, size of primary > 2 cm, truncal site, nodal/distant disease at presentation, and duration of disease before presentation (< or =3 months) were poor prognostic factors. Surgery was the initial treatment of choice and it significantly improved overall survival (p =.004).

CONCLUSIONS: We identified poor prognostic factors that may necessitate more aggressive treatment. The suggested staging system, incorporating primary tumour size, accurately predicted outcomes.