Cervical Paraganglioma

Abstract

Paragangliomas are rare tumors that arise from neural crest cells occurring at an incidence of between 1 in 30,000 and 1 in 100,000 (Papaspyrou et al, Head Neck-J Sci Spec 34(5):632–637, 2012). Ninety percent of these tumors occur in the adrenal paraganglia and are termed pheochromocytoma (Moore et al, Otolaryngol Head Neck Surg 154(4):597–605, 2016). Among the paragangliomas that occur outside of the adrenal gland, 85% are located in the abdomen, 12% in the thorax, and 3% in the head and neck (Moore et al, Otolaryngol Head Neck Surg 154(4):597–605, 2016). The carotid bifurcation is the most common paraganglioma tumor subsite within the head and neck, accounting for 60–78% of head and neck paragangliomas (Abu-Ghanem et al, Head Neck 38(Suppl 1):E2386–2394, 2016). Paragangliomas of the head and neck can also arise from other sites, including the jugulotympanic region, vagus nerve, sympathetic trunk, laryngeal paraganglia, ciliary ganglion, nasal cavity, and paranasal sinuses (Piccini et al, Endocr-Relat Cancer 9(2):149–155, 2012; Langerman et al, Head Neck 35(7):930–933, 2013). Head and neck paragangliomas are typically parasympathetic in origin and non-secreting (non-chromaffin paragangliomas); only 3–5% hypersecrete catecholamines (Suarez et al, Head Neck 35(8):1195–1204, 2013). Paragangliomas can either be hereditary or sporadic. When hereditary, gene mutations are usually found in the succinate dehydrogenase or mitochondrial complex II genes (Piccini et al, Endocr-Relat Cancer 9(2):149–155, 2012). Most sporadic paragangliomas present as a single tumor, with only 10–20% demonstrating multifocality. However, familial paragangliomas are multifocal in up to 80% of cases (Lee et al, Cancer 94(3):730–737, 2002). This chapter will focus on head and neck paraganglioma of the lateral cervical region, specifically carotid body and vagal paragangliomas.