Abstract
A case of a primary neuroendocrine (Merkel cell) carcinoma arising in the anterior skull base involving the dura, both frontal lobes, and the paranasal sinuses is presented. The tumor was completely removed by an enlarged bifrontal transbasal approach. The neuropathological, immunohistological, and electron microscopical investigation revealed all characteristics of a Merkel cell carcinoma, normally presenting as a skin carcinoma of the head and neck. The history, treatment, neuropathology, and possible explanation for this rare manifestation are discussed.