Glomus Jugulare Carotid Body Tumors

Glomus jugulare tumors arise from the paraganglionic tissue surrounding the jugular vein in the area of the middle ear. These tumors most often present late in life, in the sixth or seventh decade, and may have a genetic predisposition. They are locally invasive and highly vascular. Although presenting symptoms include complaints similar to those typical of acoustic neuromas, with tinnitus or hearing loss, the key differentiating features of these tumors are pain and the presence of loud pulsations in the ear. Occasionally, blood may drain from the ear. On examination, the patient may have tumor visible in the ear canal and, with larger lesions, evidence of cranial nerve or cerebellar dysfunction. The differential diagnosis includes neuromas, cholesteatomas, meningiomas, vascular malformations, or metastatic disease.

MRI with gadolinium enhancement demonstrates a brightly enhancing lesion and defines the spread and involvement of the local structures. Angiography is important to define the presence of vascular involvement before any attempts are made to remove the tumor surgically. Often, preoperative embolization of the involved vascular territories and intraoperative electrophysiological monitoring will help in surgical removal, which can be curative. Radiotherapy, including the use of stereotactic radiation or gamma knife, is an option for patients who are poor risks for surgery or who have residual disease that appears to be growing on serial MRI examinations. Prognosis is excellent.

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