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The three main surgical approaches to the tumor are the translabyrinthine (incision behind the ear to reach the bony labyrinth), the retrosigmoid (incision behind the ear to reach cerebellopontine angle) and the middle cranial fossa (incision in front of the ear to access the IAC from above). [21]
Neuroendocrine adenoma middle ear (NAME) is a tumor which arises from a specific anatomic site, the middle ear. [1] NAME is a benign glandular neoplasm of middle ear showing histologic and immunohistochemical neuroendocrine and mucin-secreting differentiation (biphasic or dual differentiation).
The tumors are usually small, with a mean size of about 1.2 cm. [2] The tumors are separated into three histologic types based on the dominant findings: Ceruminous adenoma [2] Ceruminous pleomorphic adenoma [2] [5] Ceruminous syringocystadenoma papilliferum [2] All of the tumors are unencapsulated, but are usually well defined or circumscribed.
Ceruminous adenocarcinoma is a malignant neoplasm derived from ceruminous glands of the external auditory canal.This tumor is rare, with several names used in the past. [3] [4] Synonyms have included cylindroma, ceruminoma, ceruminous adenocarcinoma, not otherwise specified (NOS), ceruminous adenoid cystic carcinoma (ACC), [1] [2] and ceruminous mucoepidermoid carcinoma.
With treatment, the likelihood of hearing preservation varies inversely with the size of the tumor; for large tumors, preservation of hearing is rare. Because acoustic neuromas, meningiomas and most other CPA tumors are benign, slow growing or non-growing, and non-invasive, observation is a viable management option.
Cholesteatoma is a destructive and expanding growth consisting of keratinizing squamous epithelium in the middle ear and/or mastoid process. [1] [2] Cholesteatomas are not cancerous as the name may suggest, but can cause significant problems because of their erosive and expansile properties.
This is a very rare tumor, since only about 1 in 35,000 to 40,000 people have VHL, of whom about 10% have endolymphatic sac tumors. Patients usually present in the 4th to 5th decades without an gender predilection. The tumor involves the endolymphatic sac, a portion of the intraosseous inner ear of the posterior petrous bone. [1] [5]
By gross description, there is usually a solitary, polypoid, reddish mass behind an intact ear drum (tympanic membrane). The tissue is often friable, measuring <2 cm in most cases. All tissue should be processed in order to exclude a concurrent cholesteatoma.