Carcinoid Tumor of the Middle Ear: Clinical Features, Recurrences, and Metastases

Objective: Present four new cases of carcinoid tumor of the middle ear, two of which developed late recurrences and regional metastases. Review the literature to identify the clinical features, rate of recurrence, and incidence of metastasis of carcinoid tumor of the middle ear. Study Design: Retrospective chart review. Setting: Tertiary referral hospital. Patients: Eligibility criteria consist of a diagnosis of carcinoid tumor of middle ear. Intervention: Surgical excision of primary and metastatic disease. Main Outcome Measure: Clinical characteristics, rate of recurrence, and incidence of metastasis of carcinoid tumor of the middle ear. Results: Forty-six patients with carcinoid tumor of the middle ear are included in this report, 42 patients were identified from a review of the literature, and 4 new patients are presented. The most common presenting symptom was hearing loss. Surgical excision was the treatment with radical mastoidectomy being the most common procedure. Ten (22%) patients developed locally recurrent disease, and four (9%) developed regional metastases. Conclusions: Carcinoid tumor of the middle ear is an infrequent cause of a middle ear mass, with only 46 cases published. Despite previous assertions of benignancy, the findings of this study suggest that carcinoid tumor of the middle ear is indeed a potential low-grade malignancy with documented metastatic potential. Almost all middle ear adenomatous tumors (“adenoma” and “carcinoid”) show evidence of neuroendocrine differentiation, and so at least some middle ear carcinoids (“adenomas”) appear to represent well-differentiated neuroendocrine carcinomas. Presentation and symptoms are consistent with a middle ear mass and rarely include carcinoid syndrome. Surgical treatment is recommended and tailored to the extent of disease. Patients with carcinoid tumor of the middle ear require indefinite follow-up for possible recurrence or metastasis.