Metastastic potential of middle ear neuroendocrine tumours

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.