Discussion:
Neuroendocrine adenomas of the middle ear are benign epithelial tumors. Clinical presentation is nonspecific and vary in each patient. Consequently, the diagnosis is established after histological and immunohistochemical examinations5-7.
Some of the important differential diagnosis for Neuroendocrine adenoma of middle ear includes glomus tympanicum, glomus Jugulare, congenital cholesteatoma which are difficult to exclude on imaging or clinically. Thus, they are usually diagnosed by histopathology. In Our case, the Radiological imaging was suggestive of glomus tumor. However, on Histopathology, it was proved otherwise.
The pathogenesis of this tumor is still under discussion as different authors have proposed multiple origins. Some have a view that they are originated from the cells of the mucosa of the middle ear. Some are suggesting that they developed from off-site embryonic nests of glandular cells in the middle ear mucosa.7,8,17Recently Katabi and Torske and Thompson proposed that it may originate from undifferentiated pluripotential endodermal stem cells since epithelial cells with neuroendocrine differentiation do not exist in the middle ear.16,17
The most preferred treatment option is surgical excision with Middle ear exploration. 10-15 If Ossicular chain is involved, tympanoplasty with ossicular chain removal is treatment of choice, depending upon the involvement of the ossicle. If lesion is small, localized to middle ear cleft and is not invading surrounding structures like in our case, excision with trans-canal approach can be performed. If the lesion involves most of the middle ear cleft, tympanoplasty with mastoidectomy is recommended. 10,11