Neuroendocrine neoplasms of the middle ear: Unpredictable tumor behavior and tendency for recurrence

R.J.L. van der Lans, M.S.D. Engel, J.A. Rijken, E.F. Hensen, E. Bloemena, M. van der Torn, C.R. Leemans, C.F.G.M. Smit

Research output: Contribution to JournalArticleAcademicpeer-review


© 2021 The Authors. Head & Neck published by Wiley Periodicals LLC.Background: Middle ear adenomatous neuroendocrine tumors (MEANTs) are rare temporal bone tumors. This study evaluates its clinical behavior and therapy outcome. Method: Retrospective case review in a tertiary referral center evaluating histopathology, immunohistochemistry, treatment, and outcome. Results: Nine patients were diagnosed with MEANT. One patient presented with locally invasive tumor and underwent extensive en-bloc tumor resection with adjuvant radiotherapy. Seven of eight patients with locally non-aggressive tumor confined to the tympanomastoid space underwent tumor resection. Two patients were disease-free, five presented recurrence, even after apparent successful surgery. All tumors showed neuroendocrine features. Histopathology and immunohistochemistry did not yield prognostic tumor characteristics. Conclusion: MEANTs are rare tumors with uncertain biological behavior and subsequent unpredictable clinical course. The preferred treatment is complete surgical tumor resection. They have a high tendency for recurrence, irrespective of negative intermediary surgery. As of yet, there are no prognostic biomarkers, including histopathology and immunohistochemistry.
Original languageEnglish
Pages (from-to)1848-1853
JournalHead and Neck
Issue number6
Publication statusPublished - 1 Jun 2021


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