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