Glomus Jugulare Tumor Presentation and Management: A Case Study

Mark C. Coles

Disclosures

J Neurosci Nurs. 2004;36(4) 

In This Article

Background

Glomus jugulare tumors are rare lesions accounting for approximately 0.6% of head and neck tumors (Greenberg, 2001) and arise from paraganglion cells in the area of the jugular bulb, typically from the superior vagal ganglion. They are one of the paragangliomas—lesions arising from paraganglion cells near the sympathetic ganglia—which are designated based on site of origin. (For example, paragangliomas of the adrenal medulla are pheochromocytomas, and paragangliomas of the carotid bifurcation are carotid body tumors.) Lesions may occur in multiples along the extra-adrenal chromaffin cell system; 40%-50% of all patients with an auto-somal dominant familial variant will demonstrate multiple lesions. Female-to-male ratio may be as high as 6:1. The glomus jugulare tumor is the most common paraganglioma of the head and neck followed by carotid body and intravagal paragangliomas. They are generally slow growing (advancing less than 2 cm in 5 years) and histologically benign (Al-Mefty et al., 2002) although rarely malignancy has been reported. Recurrence is common, however, up to one third in some studies, due to difficulty in achieving complete resection. Glomus jugulare tumors are known to track along vessels, may have finger-like extension into the jugular vein, and may embolize upon resection.

There are several classification systems for glomus tumors, such as the modified Jackson classification table (Greenberg, 2001), which grade the lesion based upon extension into the internal auditory canal or petrous apex. Of particular surgical significance, glomus body tumors may actively secrete catecholamines similar to pheochromocytoma and manipulation may provoke hypertensive crisis (Boulos, Schwartz, & Popp, 2000). Intraoperative release of serotonin, histamine, or bradykinin may also occur causing profound hypotension or shock.

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