Rare Presentation of Middle Ear Neuroendocrine Tumor: A Case Report.

Abstract

BACKGROUND Neuroendocrine tumors (NETs) of the middle ear are exceptionally rare, representing less than 2% of primary ear tumors in adults. These neoplasms originate from neuroendocrine cells within the middle ear cavity, posing significant diagnostic and therapeutic challenges. CASE REPORT We present a 27-year-old man with a 2-month history of left-sided hearing loss, tinnitus, and ear fullness. Comprehensive assessments, including tuning fork tests, pure tone audiometry, computed tomography (CT) scan of the temporal bone, and magnetic resonance imaging (MRI), were conducted. Differential diagnoses, included cholesteatoma and other middle ear masses, but chronic suppurative otitis media was not considered due to the intact tympanic membrane. Intraoperative findings revealed a pale pink mass encasing the ossicular chain, confirmed as a neuroendocrine tumor (CK [+], Syn [+++], CK18 [+], EMA [++], CD56 [-], CgA [+], Ki67 [2%+]) by immunohistochemistry. The tumor was excised via an endoscopic tympanotomy. Postoperative follow-up over 2 years showed no recurrence or metastasis, and the patient reported no significant hearing impairment affecting quality of life. CONCLUSIONS Middle ear neuroendocrine tumors require meticulous diagnostic workup and precise surgical intervention. This case adds to the limited body of evidence supporting that middle ear neuroendocrine tumors, although rare, can be managed effectively with tailored surgical approaches. Continued case reporting will be vital to enhance understanding and to refine treatment guidelines for optimal patient outcomes.