Paragangliomas / Glomus Tumors of the Head and Neck
Paragangliomas are rare, neuroendocrine tumors that can involve different areas of the skull base and neck. Also known as “glomus” tumors, paragangliomas are named for the structures they arise from: carotid body tumors are the most common, followed by those involving a large vein called the jugular bulb (glomus jugulare), those involving the middle ear (glomus tympanicum), and finally the vagus nerve (glomus vagale).
Paragangliomas may occur at any age, though the majority are diagnosed between ages 40 and 60. Most paragangliomas occur in isolation, but 3-10% of patients can have multiple tumors. Up to 40% of paragangliomas arise from heritable genetic mutations, the most common of which involve the succinyl dehydrogenase gene. A few tumors are associated with other neuroendocrine diseases such as neurofibromatosis type 1, multiple endocrine neoplasia type 2, von Hippel-Lindau disease.
Less than 10% of paragangliomas from the head and neck are malignant and able to spread to other locations. The majority are benign and show no spread or metastasis.