Merkel cell carcinoma and the eye

Abstract

Merkel cell carcinoma (MCC) is a rare cutaneous neuroendocrine tumor with a poor five-year survival rate. Yearly cases have risen nearly 350% since the early 1980s, and these are predicted to increase as the overall US population ages. MCC of the eyelid is uncommon and can be misdiagnosed as other benign inflammatory and neoplastic eyelid disorders. Although MCC of the head and neck is often more aggressive than it is at other sites, eyelid MCC shows a lower disease-specific mortality rate. A biopsy is essential for accurate diagnosis, including an immunohistochemical panel of CK20 and TTF-1, although other markers may be necessary. Staging can be assessed clinically through physical examination findings and imaging and/or pathologically with sentinel lymph node biopsy or fine-needle aspiration. Pathologic staging more accurately predicts the prognosis. Eyelid MCC treatments include Mohs micrographic surgery to allow for complete clearance and adequate reconstruction of lost tissue, followed by adjuvant radiotherapy. In advanced disease, immunotherapies are preferred over traditional chemotherapy and are a subject of ongoing research.