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Metastastic potential of middle ear neuroendocrine tumours
Michelle Sophia Dominique Engel1,2, Erik Frans Hensen3, Elisabeth Bloemena4
1Amsterdam University Medical Center, Department of Otolaryngology & Head and Neck Surgery, Vrije Universiteit Amsterdam, Amsterdam, Netherlands.
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.
Insights
Some middle ear neuroendocrine tumors (MeNETs) can aggressively spread distantly and be fatal, even after appearing indolent. Early diagnosis and long-term monitoring are crucial for managing these rare temporal bone neoplasms.
Area of Science:
- Oto-oncology
- Neuroendocrine Tumors
- Temporal Bone Neoplasms
Background:
- Middle ear neuroendocrine tumors (MeNETs) are rare neoplasms.
- While often indolent, a subset exhibits aggressive behavior, including local invasion and metastasis.
Purpose of the Study:
- To describe the clinical presentation, progression, and outcomes of MeNETs.
- To highlight cases with unfavorable clinical courses.
Main Methods:
- Retrospective review of 3 patients with MeNETs.
- Analysis of clinical features, diagnostic findings, management, and outcomes.
Main Results:
- All 3 patients developed distant metastases 6-16 years post-diagnosis.
- Two patients died from the disease.
- No specific clinical features predicted an unfavorable course.
Conclusions:
- A subset of MeNETs can have aggressive, metastatic behavior and lead to death.
- Thorough diagnostic work-up and extended follow-up are vital for MeNET patients.
