Contemporary Management of Merkel Cell Carcinoma

Abstract

Merkel cell carcinoma is a rare, aggressive cutaneous neuroendocrine tumor, often diagnosed in later stages. Most of cases are related to either ultraviolet exposure or infection with the Merkel cell polyomavirus. Immunocompromised patients are at higher risk for the development of the disease. Treatment in the initial stages consists of resection and radiotherapy. Metastatic disease has a very poor prognosis and shows a limited durable response to chemotherapy. Combination of surgery, radiation therapy, and systemic therapy is used for the treatment of metastatic disease. Recently, the advent of programmed cell death protein 1 axis agents has improved outcomes in metastatic disease. There are ongoing clinical trials evaluating these agents in resected disease.

Keywords: Cutaneous neuroendocrine tumor; Merkel cell carcinoma; Merkel cell polyomavirus; Nonmelanoma skin cancer.