Middle ear neuroendocrine tumours: Insight into their pathogenesis, diagnosis and management.

Recent advances in the diagnosis of neuroendocrine tumours (NETs) have led to the identification of NETs in unusual locations. NETs originating in the middle ear have been reported in a number of case reports with differing terminologies, where they were variously referred to as middle ear adenomas or middle ear carcinoids. In this report, we describe two cases of middle ear NETs with locally advanced disease and with distant metastases, respectively. The embryological origin, pathogenesis and histopathological characteristics, including immunohistochemical markers, are discussed aiming to clarify the terminology of these tumours. Functional imaging modalities (eg, Ga-DOTATATE positron emission tomography/computed tomography/magnetic resonance imaging) play a pivotal role in the diagnosis, staging and determining the optimal systemic therapy in managing NETs of the middle ear. In addition, surgical, local and systemic therapeutic modalities are discussed, including the management option of long-acting somatostatin analogue therapy and targeted radionuclide therapy for somatostatin receptor-positive well-differentiated middle ear NETs.