Middle ear adenoma and adenocarcinoma

Adenomas and adenocarcinomas originating from the middle ear are few in number and have met with significant controversy in the literature. Our experience with 11 previously described patients is updated and five new cases are added. These tumors were seen in persons whose ages ranged from 7 to 77 years. The most common initial symptom was decreased hearing (11 cases), followed by otorrhea (4 cases), and otalgia (4 cases). At the time of diagnosis, facial nerve weakness was present in several patients (seven), and this was a poor prognostic sign. These glandular tumors engender controversy regarding histologic origin and the pathologic demarcation between adenoma and adenocarcinoma. In this review, we show that otorrhea, bone erosion, facial nerve involvement, and aggressive clinical behavior can be the result of lesions that appear microscopically benign. Furthermore, we present evidence of the similarity of these glandular lesions to paragangliomas and carcinoid tumors. Finally, pathologic data are presented to develop the previously unreported theory that the origin for this group of tumors is paraganglionic tissue.