A 33-year-old woman presented with pruritus of the right ear and on ex
amination was found to have 1 x 1 cm polypoid lesion in the external a
uditory canal. This was removed and histological examination revealed
a well-circumscribed spindle cell lesion arranged in fascicles and who
rls. Immunohistochemistry showed the tumor to be EMA-positive but S-10
0-, desmin-, actin-, and cytokeratin-negative. Ultrastructural examina
tion confirmed perineurial cell origin. There are several lesions in w
hich perineurial cells are present, but a true perineurioma is compose
d exclusively of perineurial cells. It has characteristic morphologica
l, immunohistochemical and ultrastructural features that help separate
it from other nerve and neuroectodermal lesions.