Paragangliomas

Paragangliomas are rare, typically benign neuroendocrine tumors arising from paraganglionic tissue associated with the autonomic nervous system. In the head and neck region, they commonly involve the carotid body, jugulotympanic area, and vagal nerve. These tumors are often slow-growing and may remain asymptomatic for years, but they can present as painless, pulsatile neck masses, cranial nerve deficits, or tinnitus depending on their location. Functional paragangliomas secrete catecholamines, leading to systemic symptoms such as hypertension, palpitations, and sweating, though most head and neck paragangliomas are nonfunctional.

Diagnosis involves clinical evaluation, imaging studies including contrast-enhanced CT, MRI, and functional scans such as ¹²³I-MIBG or PET-CT to assess tumor extent, vascularity, and potential multicentricity. Genetic testing is recommended, as paragangliomas are often associated with hereditary syndromes, including SDH mutations. Histopathology confirms the diagnosis post-surgically, displaying characteristic “zellballen” patterns.

Management is primarily surgical, with careful preoperative planning due to the tumor’s high vascularity and proximity to cranial nerves and major vessels. Preoperative embolization may reduce intraoperative bleeding. In selected cases, radiotherapy or observation may be appropriate for small, asymptomatic tumors. Long-term follow-up is essential to monitor for recurrence or metastasis, particularly in hereditary or multifocal cases.