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Neurothekeoma (peripheral nerve sheath myxoma) of the external auditory canal.

A patient who presented with an isolated soft-tissue tumor in the lateral portion of the external auditory canal, which after removal was diagnosed histopathologically as a peripheral nerve sheath neurothekeoma, is presented. Peripheral nerve sheath myxomas (neurothekeomas or perineural myxomas) have been reported to occur on the central area of the face, arms and shoulders. This is the first case in which a tumor of this type has been reported to develop in the external auditory canal. The distinguishing histopathological characteristics and the differential diagnosis of this tumor are discussed.