Radiotherapy for Glomus Jugulare Tumors: A Single-Institution Experience

Abstract

Paragangliomas or chemodectomas are rare and usually benign tumors arising from the paraganglia, clusters of neuroendocrine cells that are associated with the peripheral nervous system. [1] Glomus bodies are found in anatomic sites such as in the jugular bulb, along the tympanic and auricular branch of the tenth nerve in the middle ear. According to the location, glomus tumors can be classified as tympanic, jugulare, or carotid body or designated as arising from other locations. [2] The mean age at diagnosis ranges from 40 to 50 years. These tumors occur three to four times more frequently in women than in men, suggesting a possible estrogen influence. [3] Clinical presentation of head-and-neck paragangliomas differs according to the site they arise from. Glomus tumors of the middle ear present with pain or discomfort in the ear, pulsatile tinnitus, and hearing loss. Carotid body tumors present as a painless, slow-growing mass in the neck that may be pulsatile and may be associated with bruits. Glomus jugulare tumors originate at the skull base at the jugular bulb and may be associated with bone destruction. Patients with these tumors may develop cranial nerve deficits; typically, cranial nerves 9 through 12 are involved. These tumors can also originate and spread along the tympanic canaliculus, invading superiorly into the middle ear and inferiorly toward the jugular fossa. [4] Rarely, these tumors can be malignant.