Paraganglioma glomus tumour

A paraganglioma glomus tumour is a highly vascular, usually slow growing benign tumour. In 3 % of cases it may be malignant (cancerous). It originates from cells of the sympathetic autonomic nervous system.

The incidence of paraganglioma is 1 out of 1.3 million people per year. It occurs throughout the whole body and although rare, is still the most common tumour of the middle ear.

Paraganglioma glomus tumour

Paraganglioma or glomus tumour

Paragangliomas are more common in females and occur mostly in patients aged 40- 70 years.

It is estimated that 3% of paragangliomas are multicentric, thus occurring in more than one location in the body. These multicentric tumours are more commonly found in some genetic familial conditions. It may also occur in conjunction with other neuroendocrine tumours such as pheochromocytomas.

Paraganglioma or glomus tumours cause symptoms due to their local effects but 3 % of them produce hormones with general manifestations such as high blood pressure, headaches, anxiety, pulsating heart beat and sweating. When located in the ear a paraganglioma is referred to either a glomus tympanicus or a glomus jugulare, depending on the size and extent.

The most common symptoms of those originating in the temporal bone are pulsating tinnitus (hearing your heartbeat) and conductive hearing loss.Other symptoms include bleeding, dizziness, vertigo, otorrhoea (ear discharge), facial nerve weakness and sometimes pain. Large tumours that involve the lower cranial nerves often present with hoarseness, swallowing difficulty and weakness of the shoulder and the tongue.

Paraganglioma glomus tumour

Left ear glomus tumour. Maleus bone (M), tympanic membrane (T) and glomus tumour (*)

Patients may present with a bruit (audible pulsating sound), hearing loss and involvement of the cranial nerves. With examination of the ear a bulging red vascular pulsating mass is often seen behind the ear drum. It can extend into the external auditory canal.

Magnetic resonance imaging (MRI), computed tomography (CT), angiography, hearing tests and blood tests form part of some of the investigations that may be performed.

Paragangliomas/ glomus tumours are managed with observation, radiation therapy, surgery or a combination thereof.