Merkel cell carcinoma (primary cutaneous neuroendocrine carcinoma): an overview on management

Merkel cell carcinoma is an uncommon but aggressive primary cutaneous neuroendocrine (small cell) carcinoma. There is ongoing debate regarding the optimal treatment of this disease. The early literature comprised small institutional studies with inherent weaknesses. Recent data have emerged from larger studies, including those from Australian institutions, that adds support to a multimodality approach as best practice. Despite this, the outcome for patients with unfavourable disease remains poor and in most series 25-30% of patients die as a direct result of Merkel cell carcinoma. The head and neck is the commonest site for presentation (50-60%) and wide excision (2-3 cm) of the primary lesion is usually recommended, although achieving this is often difficult within functional and cosmetic constraints. All clinically node-negative patients should be considered candidates for elective nodal treatment and those with clinical nodal disease should undergo nodal dissection and adjuvant radiotherapy. Recent evidence suggests that patients treated with surgery and adjuvant locoregional radiotherapy experience a better disease-free survival compared with those undergoing surgery alone. The role of platinum-based chemotherapy is evolving. The aim of this article is to discuss relevant issues in the management of a patient with Merkel cell carcinoma.