Approach Considerations

Imaging Studies

Plain skull radiography may show enlargement of the lateral jugular foramen and fossa. Axial and coronal computed tomography (CT) scanning with thin sections are superior at demonstrating the extent of bone destruction. Magnetic resonance imaging (MRI) with gadolinium-diethylenetriamine pentaacetic acid (DTPA) contrast is the best imaging study for delineating tumor limits. Glomus tumors on T1- and T2-weighted MRI have characteristic soft tissue mixed intensity with intermixed high-intensity signals and signal voids (ie, salt and pepper appearance) representing fast flowing blood. A combination of CT scanning and contrast MRI is the imaging regimen of choice for glomus jugulare tumors.^[5]

Unless carotid arteriography is necessary for preoperative evaluation and/or embolization, noninvasive techniques are preferred; however, for large tumors involving the internal carotid artery (ICA), preoperative carotid arteriography with cross-compression or trial balloon occlusion is recommended. The venous drainage systems also need to be carefully studied before sinus occlusion is carried out during surgical resection.

For tumors with large intracranial extension, vertebral arteriography is advised to exclude arterial feeders from the posterior circulation.

Staging

The Glasscock-Jackson and Fisch classifications of glomus tumors are widely used. Staging is as follows^[26]:

Type A - Tumor limited to the middle ear cleft (glomus tympanicum)
Type B - Tumor limited to the tympanomastoid area with no infralabyrinthine compartment involvement
Type C - Tumor involving the infralabyrinthine compartment of the temporal bone and extending into the petrous apex
Type C1 - Tumor with limited involvement of the vertical portion of the carotid canal
Type C2 - Tumor invading the vertical portion of the carotid canal
Type C3 - Tumor invasion of the horizontal portion of the carotid canal
Type D1 - Tumor with an intracranial extension less than 2 cm in diameter
Type D2 - Tumor with an intracranial extension greater than 2 cm in diameter

Treatment & Management

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Media Gallery

Lateral view of the initial carotid arteriogram of a 20-year-old woman who presented in June 1970 with episodic hypertension, headaches, and palpitations. Urine catecholamine levels were elevated, and a pheochromocytoma was suspected. She underwent a negative exploratory laparotomy. She subsequently developed palsies of the IX, X, XI, and XII cranial nerves on the right side. A norepinephrine-secreting glomus jugulare tumor with intracranial and cervical extension was identified on radiologic and arteriographic imaging. Arrows delineate the tumor blush. The arrowhead demonstrates a branch of the middle meningeal artery providing blood supply to the tumor. This branch was embolized.
A significant decrease of tumor vascular blush (arrows) following embolization of a norepinephrine-secreting glomus jugulare tumor with intracranial and cervical extension.
CT imaging demonstrates the extent of bony destruction (white and black arrows) by the tumor. The normal jugular foramen on the left (arrow head) is shown for comparison. The patient subsequently underwent surgical resection of the extracranial portion of this extensive tumor. The remaining intracranial portion was treated with radiation therapy (54 Gy). Follow-up evaluations, including imaging and laboratory investigations, demonstrated long-term control of both tumor growth and catecholamine production.
Lateral carotid arteriogram obtained 22 years after radiation therapy in a 20-year-old woman who presented in June 1970 with episodic hypertension, headaches, and palpitations.
Corresponding MRI of the tumor depicted in the previous image indicating no evidence of tumor growth over time.