Diagnostic pitfalls of Merkel cell carcinoma and dramatic response to chemotherapy

Merkel cell carcinoma (MCC) is an unusual malignant tumor that arises from neuroendocrine cells with features of epithelial differentiation. We describe a MCC patient with unusual clinical, histopathological, and immunohistochemical features. Although the microscopic, immunohistochemical, and ultrastructural characteristics of MCC have been well defined, diagnostic difficulties remain, particularly in distinguishing it from lymphoma involving the skin, as suggested by our case. This is an unusual case in which dense lymphoid infiltration masked the true tumor. All the immunohistochemical markers of MCC except neuron-specific enolase (NSE) were negative. The dramatic response to primary chemotherapy was also very noteworthy.