From Conductive Hearing Loss to Whipple’s Surgery: A Case Report and Literature Review on Neuroendocrine Tumors of the Middle Ear

Middle ear neuroendocrine tumors (MeNET) are a group of rare tumors and, therefore, challenging for ENT surgeons. Patients usually present nonspecific symptoms, and clinical and radiological examinations do not provide the sensitivity and specificity necessary to make a reliable diagnosis. Even intraoperatively, MeNET may be heterogeneous in its appearance. Consequently, a definitive diagnosis is challenging and based on a combined histopathology and immunohistochemical examination. These patterns, again, are wide-ranging. As a result, numerous terms have emerged in medical history, but they all describe very similar pathologies. Nowadays, we summarize them under MeNET. Overall, they show a rather less invasive growth behavior and appear mostly localized in the tympanic cavity. The number of reported cases of MeNET with highly invasive or metastatic growth is paltry. In this case report, we present a 65-year-old female patient with MeNET who complained of hearing loss and aural fullness in her left ear. After histological evaluation, a MeNET of pancreatic origin was assumed. Additional staging with DOTATOC showed an upregulated somatostatin expression in the pancreatic caput and cauda, and a suspected correlate in MRI was described, leading to a partial pancreatic resection. Surprisingly, histological analysis showed ultimately no evidence of NET in the pancreatic tissue after all.