Neuroendocrine tumors of the middle ear (MEANT) – Case report of 2 cases

Introduction
Neuroendocrine tumors of the middle ear (MEANT) are a rare disease, of which less than 150 cases have been reported. Related symptoms are conductive hearing loss, tinnitus, aural fullness and, more rarely, facial nerve paralysis or carcinoid syndrome. In a small minority of cases, lymph node metastases, most commonly within the ipsilateral parotid gland, have been described.

Material and Methods
We herein present two new cases of MEANT.

Results
Case 1: A 56-year-old male patient presented with left sided unilateral cephalgia, otorrhea as well as hearing loss. Ear microscopy revealed a bulging tympanic membrane, a CT scan showed a mass in the tympanic cavity. The diagnosis of MEANT was established after a sample was taken via tympanoscopy. Metastases were excluded by SSTR PET Scan. Subsequent resection and tympanoplasty type IIIa were performed. Follow up is provided annually and to date, no evidence of recurrence occurred.
Case 2: A 32-year-old female presented with the incidental finding in a CT scan of a mass in the right external auditory canal and middle ear. Microscopically, a polypoid tumor, attached to the malleus was detected. Resection of the mass and tympanoplasty type IIIa was performed and histological workup established the diagnosis MEANT. A postoperatively performed SSTR PET scan revealed excluded metastases. Annual follow up was without findings to date.

Conclusions
We herewith present two unpublished cases of MEANT, an extremely rare low grade malignant disease. In 2017, Marinelli et al proposed a TNM grading system, according to which would be classified as pT2a and pT2c respectively. None of the cases showed a sign of recurrence nor metastasis so far, however follow up is crucial as metastases can occur up to 13 years post surgery.

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