These are rare tumours; the most common form is known as phaeochromocytoma and arises from the adrenal gland. Paraganglioma typically refers to extra-adrenal variants of this tumour, which most commonly arise in the head and neck region. They have multiple names based on their location, including glomus jugulare (base of skull associated with jugular foramen), carotid body tumour (carotid artery tumour) or glomus tympani (tympanic membrane tumour). They are usually non functional as opposed to phaeochromocytoma.
Diagnosis
The diagnosis must be considered for tumours arising in the head and neck region in close proximity to vessels, at the base of skull, and in the paravertebral regions. Given that most tumours are non-secretory, patients typically present with a mass lesion.
Management
Surgery
For tumours not located in the base of skull, surgical excision is often curative. Local recurrences are not uncommon.
Radiotherapy
Radiotherapy provides an alternative treatment option and has similar rates of local control and potentially less toxicity, particularly in the base of skull or tympanic membrane (80-90%).
The usual dose is 45 Gy in 25 fractions delivered to the tumour with a small margin (0.5 cm GTV-CTV, 0.5 cm CTV-PTV).
Toxicities are less than for mucosal head and neck therapies due to the lower dose.
