Recurrent neuroendocrine adenoma of the middle ear (NAME)

MA are very rare, benign, glandular tumors originating from the middle ear mucosa.

A 54-year-old patient presented with hypacusis left that had persisted since 03/2021 and a feeling of pressure in the left ear. Anamnestic there is a state after NAME left with a tumor resection in 04/2015 outwards and a state after second-look operation in 01/2016 in our clinic without evidence of a recurrence.

Clinically, there was a tumor in the upper part of the left ear canal, growing from the middle ear. In the DVT of the temporal bone, there was an Tumor in the medial external auditory canal and in the left tympanic cavity. Audiometrically a combined hearing loss on the left with a sound conductive component of 20-40 dB was evident.

Intraoperatively, a tumor could be identified starting the middle ear mucosa, growing to the antrum, the tensor tympani muscle and the oval niche. A tumor resection with auditory canal enlargement and split-thickness skin reconstruction, middle ear surgery with atticoantrotomy and TPL type III were performed.

Histologically, fibrous tissue with neuroendocrine differentiated solid-trabecular epithelial complexes was found, consistent with the clinical evidence of the middle ear adenoma.

A presentation is currently taking place at a neuroendocrinological tumor conference with the planning of a DOTANOC PET / CT under the question of complete removal and the exclusion of metastases. A second look operation is planned in 18 months.

The very rare NAME are difficult to classify clinically and histopathologically in the differential diagnosis. The presumably high recurrence rate of these tumors and their dignity, which cannot always be clearly assessed, require a correspondingly radical surgical approach and long-term controls with second-look operations.

Publication History

Article published online:
24 May 2022