Neuroendocrine adenomas of the middle ear – a case Serie of three patients

Introduction:

Neuroendocrine adenomas, also known as carcinoids of the middle ear, are rare. Pathogenesis remains unclear. One hypothesis implies the development from pluripotent stem cells. The clinical findings are not specific. Cholesteatoma, neurinoma and paraganglioma are mentioned as differential diagnoses. The definite diagnosis is determined by immunohistochemistry.

Methods:

From 2009 until 2016 three patients (age between 28 and 48 years) were treated on a neuroendocrine adenoma of the middle ear. Medical history, examination findings, therapy and long-term course were analysed.

Results:

The first 2 patients complained of tinnitus and hearing loss in the affected ear. The radiological diagnosis was a paraganglioma. In case 1 tumor resection was performed in combination with a tympanoplasty type III PORP. In case 2 tumor removal was subtotal in order to save the ossicular chain. The third patient was operated in another hospital. Histologically there was a suspicion of an adenocarcinoma of the middle ear. Therefore, subtotal petrosectomy with preservation of the inner ear and the facial nerve was performed. In all cases the immunohistochemical examination showed the typical finding of a neuroendocrine adenoma without malignancy. The patients 1 and 3 are free of recurrence in the long-term course. In case 2 the residual tumor showed no further growth up to date.

Conclusion:

The therapy of neuroendocrine adenomas remains a complete resection. An adjuvant therapy is not recommended, the local recurrence rate is up to 20%. Regional metastases were only mentioned in few cases. Therefore clinical and radiological follow-ups are necessary.

Publication History

Publication Date:
23 April 2019 (online)