The First Reported Case of Primary Intestinal-type Adenocarcinoma of the Middle Ear and Review of the Literature.

Objective: Adenocarcinoma of the middle ear is a rare entity that must be distinguished from other adenomatous tumors of the temporal bone. We present the first patient of an intestinal-type adenocarcinoma originating from the middle ear. Patients: A 58-year-old woman presented with an 8-year history of left otorrhea. Her middle ear effusions were quite thick and gummy. She underwent eight sets of pressure-equalization tubes within 3 years. In 2011, her physical examination identified a middle ear mass, and she underwent mastoidectomy. A middle ear adenoma was resected. She underwent an additional three mastoidectomies for recurrence, with pathology from the 4th mastoidectomy revealing a diagnosis of adenocarcinoma. Imaging, at that time, showed an extensive temporal bone and Eustachian tube tumor. Interventions: She underwent a left subtotal temporal bone resection, parotidectomy, infratemporal fossa resection, dural resection, and microvascular free flap reconstruction followed by postoperative proton beam radiotherapy. Main Outcome Measures: The final pathology report revealed intestinal-type adenocarcinoma. Results: Treatment was successful and the patient remains disease free 4 years later. Conclusion: This is the first report of an intestinal-type adenocarcinoma of the middle ear and temporal bone. This patient also illustrates the difficulty of accurate histologic diagnosis of adenomatous tumors of the middle ear. Copyright (C) 2017 by Otology & Neurotology, Inc. Image copyright (C) 2010 Wolters Kluwer Health/Anatomical Chart Company

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