Abstract

Objective. Middle ear neuroendocrine tumours (MeNETs) are rare neoplasms of the temporal bone. Although the clinical behaviour of MeNETs is often indolent, a subset demonstrates aggressive progression with locally invasive and metastatic disease. This study aims to describe the clinical presentation, progression, and outcomes of middle ear neuroendocrine tumours in 3 patients with particularly unfavourable clinical courses.
Methods. We retrospectively reviewed 3 patients diagnosed with MeNETs who developed locally invasive tumours and distant metastases. Data on clinical features, diagnostic findings, management and long-term outcomes were reviewed.
Results. All 3 patients described in this report developed distant metastases over a period of 6 to 16 years after initial diagnosis. Two of the 3 patients eventually died from their disease. No distinct clinical features could be identified that were predictive of an unfavourable course.
Conclusions. A subset of MeNETs can follow an aggressive clinical course with metastatic spread and, in some cases, tumour-related death, even after an initial period of seemingly benign behaviour. Comprehensive diagnostic work-up and prolonged follow-up are essential for patients with MeNETs.

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Michelle Sophia Dominique Engel - Amsterdam University Medical Center, Department of Otolaryngology & Head and Neck Surgery, Vrije Universiteit Amsterdam, Amsterdam, Netherlands; Department of Otolaryngology, Catharina Hospital, Eindhoven, Netherlands. Corresponding author - m.engel@amsterdamumc.nl

Erik Frans Hensen - Department of Otolaryngology & Head and Neck Surgery, Leiden University Medical Center, Leiden, Netherlands https://orcid.org/0000-0002-4393-7421

Elisabeth Bloemena - Department of Pathology, Amsterdam University Medical Center, Vrije Universiteit Amsterdam, Amsterdam, Netherlands https://orcid.org/0000-0002-0221-9538

Rik Johannes Leonardus van der Lans - Amsterdam University Medical Center, Department of Otolaryngology & Head and Neck Surgery, Vrije Universiteit Amsterdam, Amsterdam, Netherlands https://orcid.org/0000-0002-5361-6752

Charles René Leemans - Amsterdam University Medical Center, Department of Otolaryngology & Head and Neck Surgery, Vrije Universiteit Amsterdam, Amsterdam, Netherlands https://orcid.org/0000-0001-7887-083X

Conrad Frits Smit - Amsterdam University Medical Center, Department of Otolaryngology & Head and Neck Surgery, Vrije Universiteit Amsterdam, Amsterdam, Netherlands https://orcid.org/0000-0003-4774-7219

Jeroen Jansen - Department of Otolaryngology & Head and Neck Surgery, Leiden University Medical Center, Leiden, Netherlands https://orcid.org/0000-0002-3955-0152

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This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

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Copyright (c) 2025 Società Italiana di Otorinolaringoiatria e chirurgia cervico facciale

How to Cite
Engel, M. S. D., Hensen, E. F., Bloemena, E., van der Lans, R. J. L., Leemans, C. R., Smit, C. F., & Jansen, J. (2025). Metastastic potential of middle ear neuroendocrine tumours. ACTA Otorhinolaryngologica Italica, 45(6), 431–436. https://doi.org/10.14639/0392-100X-A971
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