From the Guidelines
Head and neck paragangliomas are rare, slow-growing neuroendocrine tumors that develop from specialized neural crest cells called paraganglia, typically arising in the carotid body, jugular foramen, vagal nerve, or tympanic region, with most being benign and non-functional, but approximately 10% can secrete catecholamines, causing symptoms similar to pheochromocytoma 1.
Overview of Head and Neck Paraganglioma
These tumors are usually slow-growing and hypervascular, with the potential to become locally aggressive, and can be found in all anatomic sites of distribution of parasympathetic paraganglia 1.
Key Characteristics
- Most paragangliomas (about 90%) are benign and non-functional
- Approximately 10% can secrete catecholamines like epinephrine and norepinephrine, causing symptoms similar to pheochromocytoma such as hypertension, headaches, and palpitations
- Patients often present with a painless neck mass or symptoms related to compression of adjacent structures, including hoarseness, difficulty swallowing, or hearing loss depending on the tumor location
- Diagnosis typically involves imaging studies like CT, MRI, and sometimes nuclear medicine scans, along with catecholamine testing to rule out functionality
- Treatment approaches vary based on tumor size, location, and patient factors, with options including surgical resection, radiation therapy, or watchful waiting for slow-growing tumors in elderly patients, with preoperative angiography with embolization recommended for all jugular, large (>4 cm), or locally invasive carotid/vagal PGLs 1
Genetic Basis and Screening
- Approximately 30-40% of cases have a genetic basis, particularly mutations in succinate dehydrogenase (SDH) genes
- Genetic testing and family screening are important considerations, especially for patients with SDHD pathogenic variants, which have a high risk of developing hereditary head and neck paragangliomas (HNPGLs) 1
- Long-term follow-up is essential as these tumors can recur years after initial treatment, with a median growth rate of 1.0 mm/year and a median doubling time of 4.2–5.7 years 1
From the Research
Overview of Head and Neck Paraganglioma
- Head and neck paragangliomas are rare, slow-growing, generally benign tumors of neuroendocrine cells associated with the peripheral nervous system 2, 3, 4, 5, 6
- They commonly involve the carotid body, jugular bulb, vagal ganglia, and temporal bone 2, 3, 5
- Patients with cervical paragangliomas frequently present with a painless, slowly enlarging mass in the lateral neck, while those with tympanic paraganglioma and jugular paraganglioma often experience tinnitus, hearing loss, and lower cranial nerve deficits 3, 5
Diagnosis and Treatment
- Evaluation by an imaging modality is necessary to establish the diagnosis, with commonly used procedures including B-mode sonography, computed tomography (CT), magnetic resonance imaging (MRI), and digital subtraction angiography (DSA) 3, 5
- Treatment options include surgery, radiotherapy, stereotactic radiosurgery, and observation, with the choice of treatment depending on the individual patient's condition and the tumor's characteristics 2, 3, 4, 5, 6
- Radiotherapy is a safe and efficacious treatment associated with minimal morbidity, while surgery is reserved for patients in good health whose risk of associated morbidity is low 2
- Observation is a reasonable option for asymptomatic patients with a limited life expectancy, and conservative management approaches via observation and newer radiation therapy techniques have become more common 2, 4, 6
Clinical Presentation and Epidemiology
- The clinical presentation of head and neck paragangliomas usually involves a painless lateral mass associated with symptoms such as hoarseness, hearing loss, tinnitus, and cranial nerve deficits 5
- Up to 40% of head and neck paragangliomas are inherited, mostly linked with mutations of succinate dehydrogenase complex 5
- The incidence of head and neck paragangliomas is low, ranging from 0.3 to 1 per 100,000 every year 5