Middle Ear Neuroendocrine Tumors: A Case Series Highlighting Diagnostic and Management Challenges

OBJECTIVE: Middle ear neuroendocrine tumors (MeNETs) are rare lesions that may mimic more common middle ear pathology. Although typically indolent, they have potential for local invasion and delayed recurrence. This study aims to expand understanding of MeNETs by characterizing their presentation, management, histopathology, and long-term outcomes. STUDY DESIGN: Retrospective case series. SETTING: Two tertiary, university-affiliated medical centers. METHODS: Five patients with histologically confirmed MeNETs were identified through retrospective chart review. Data collected included demographics, presenting symptoms, imaging, operative reports, pathology, and follow-up. Histological evaluation involved hematoxylin and eosin staining and immunohistochemistry. RESULTS: All patients presented with unilateral middle ear symptoms such as hearing loss, tinnitus, or otalgia. Imaging demonstrated soft tissue masses in the middle ear cavity, occasionally with ossicular erosion. All underwent tympanomastoidectomy, and each required at least 1 additional operation, either a second-look or revision for residual or recurrent disease. Histopathology confirmed low-grade neuroendocrine tumors with characteristic immunostaining, and no metastases were identified. Two patients developed delayed local recurrence years after initial surgery. CONCLUSION: MeNETs are uncommon, low-grade tumors that can be mistaken for other middle ear lesions. Management requires surgical excision, and revision surgery is frequently necessary. While overall prognosis is favorable, the potential for delayed recurrence underscores the importance of long-term follow-up and consideration of a planned second-look procedure. This case series contributes to the limited literature guiding diagnosis and treatment of these rare tumors.