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, ⁶⁸Ga-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.

中文翻译：

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中耳神经内分泌肿瘤：深入了解其发病机制、诊断和治疗


神经内分泌肿瘤 (NET) 诊断的最新进展导致了不寻常位置的 NET 的识别。起源于中耳的 NET 在许多病例报告中都有不同的术语，其中它们被不同地称为中耳腺瘤或中耳类癌。在本报告中，我们描述了两例分别患有局部晚期疾病和远处转移的中耳 NET 病例。讨论胚胎学起源、发病机制和组织病理学特征，包括免疫组织化学标记，旨在澄清这些肿瘤的术语。功能成像模式（例如⁶⁸ Ga-DOTATATE 正电子发射断层扫描/计算机断层扫描/磁共振成像）在中耳 NET 的诊断、分期和确定最佳全身治疗中发挥着关键作用。此外，还讨论了手术、局部和全身治疗方式，包括长效生长抑素类似物治疗和靶向放射性核素治疗生长抑素受体阳性分化良好的中耳NET的治疗选择。