Perineurioma: an unusual cause of an external auditory canal polyp

A 33-year-old woman presented with pruritus of the right ear and on examination was found to have 1 x 1 cm polypoid lesion in the external auditory canal. This was removed and histological examination revealed a well-circumscribed spindle cell lesion arranged in fascicles and whorls. Immunohistochemistry showed the tumor to be EMA-positive but S-100-, desmin-, actin-, and cytokeratin-negative. Ultrastructural examination confirmed perineurial cell origin. There are several lesions in which perineurial cells are present, but a true perineurioma is composed exclusively of perineurial cells. It has characteristic morphological, immunohistochemical and ultrastructural features that help separate it from other nerve and neuroectodermal lesions.