Paragangliomas of the head and neck: diagnosis and treatment

Abstract

Paragangliomas of the head and neck (HNP) represent rare tumors of neural crest origin. They are highly vascular neoplasms that are benign in the majority of cases. The site of origin defines the name given those tumors. In the head and neck, they most commonly occur at the carotid bifurcation, where they are referred to as carotid body tumors (CBT). Other common sites of origin are the jugular bulb (jugular paraganglioma; JP), the tympanic plexus on the promontory (tympanic paraganglioma; TP) and the vagal nerve (vagal paraganglioma; VP). Patients with cervical paragangliomas frequently present with a painless, slowly enlarging mass in the lateral neck. In many patients with TP and JP, tinnitus and hearing loss are early symptoms. JP patients often suffer from lower cranial nerve deficits. Evaluation by an imaging modality is necessary to establish the diagnosis. Imaging procedures frequently used include B-mode sonography with color-coded Doppler sonography, computed tomography (CT), magnetic resonance imaging (MRI) and digital substraction angiography (DSA). Debate exists in the literature regarding the different treatment modalities for paragangliomas which include surgery, radiotherapy and stereotactic radiosurgery. The role of preoperative angiography and embolization has also been a matter of discussion. The diagnostic work up and the different treatment options for patients with head and neck paragangliomas will be presented and discussed.

Role of duplex ultrasound in the diagnosis and assessment of carotid body tumour: A literature review

“…On colour flow imaging, hypervascularity of the tumour is seen as irregular colour signals with flow direction being predominantly cephalad (35,36). Typical pulsed Doppler flow patterns from carotid body tumour have a low resistance character with a high diastolic component (37).…”

Head and Neck Paraganglioma: Medical Assessment, Management, and Literature Update

“…Bruits are heard over the ear or mastoid in larger masses. Skull base tumours may also invade the posterior cranial fossa [2,10,17,19].…”

“…They are chemoreceptors influencing respiratory drive and catecholamine secretion [1][2][3][4][5][6].…”

“…Head and neck paraganglioma (HNPGL) or 'glomus tumours' are rare, highly vascular, typically slow growing, and mostly benign neoplasms arising from paraganglia cells; malignancy occurs in less than 5% of cases [2]. The majority of HNPGL are located and classified on the basis of their site of origin, namely: the jugular bulb (glomus jugulare), Jacobsen's nerve plexus (glomus tympanicum), vagus nerve (glomus vagale), and carotid bifurcation (carotid body tumours), the last of which are the most common [4,6].…”

Internal carotid artery ligation for excision of a huge Carotid Body Tumor – Report of a Case –

“…CBT is the most common form, accounting from 50 to 80% of these tumors 1,2) . The first successful operation for CBT was conducted by Albert in 1889, while Kohn first used the term paraganglioma in 1903.…”