What is a glomus jugulare tumour?

Glomus jugulare tumours are exceedingly rare but entirely benign tumours that arise within the bone of the base of the skull.  They occur from very specialised cells in the wall of the jugular vein which drains blood from the brain.  These are modified nerve cells (paraganglia cells) and as a result, a glomus jugulare tumour is also known as a paraganglioma.  Even more rarely, they can be part of a genetic condition where people can have multiple paraganglioma tumours elsewhere in their body including in their neck or adrenal glands. 

What symptoms can they cause?

The symptoms that arise from a glomus jugulare tumour relate to their position deep within the skull base.  The nearest structure to them is the inner ear and as a consequence, the most common presenting symptom is hearing related, either pulsatile tinnitus (noise within the ear corresponding with the heartbeat) or hearing loss.  As they enlarge, they can also have an impact on the nerves for voice and swallowing and occasionally people can present with voice hoarseness and swallowing difficulties. 

Where are glomus jugulare tumours treated?

Due to their rarity, glomus jugulare tumours are typically only managed by a few specialists within the UK.  The surgeons of the Brain and Spine Clinic have managed (including operated on) significant numbers and will be able to offer you all the benefits of this considerable expertise.

What are the treatment options?

As with the vast majority of benign tumours occurring in the relatively inaccessible skull base, the management of a glomus jugulare tumour is very individualised and will be dependent on factors such as your symptoms, the size of the tumour as well as your other medical conditions.  They are typically very slow growing and on occasion can appear to stop growing for periods.  If symptoms are minimal, it is, therefore, entirely possible that you will simply be advised to undergo scan monitoring in the first instance.

The management of your tumour will not only be discussed between you and your surgeon, but also at a Skull Base Multidisciplinary Team (MDT) Meeting. These are held fortnightly at Salford Royal Hospital, with a team of experts with a huge combined experience of managing such problems. Your surgeon will convey the outcome and recommendations of this meeting to you. Whilst this will provide you with all the information you require about your glomus jugulare tumour, as well as a detailed analysis of the advantages and disadvantages of all management options, the most important factor in the final decision is you. Your personal opinion of what is best for you is undoubtedly the most relevant aspect of how you will be managed.

What does treatment involve?

The treatment of a glomus jugulare tumour has very much to be tailored to the individual.  If they are small and asymptomatic, it is entirely possible that your tumour may be simply observed with follow up scans in the first instance.  If you tumour is then shown to be growing, one of 2 treatment options will be possible.  The first of these is surgery.  If you have intact function of your voice and swallowing nerves, surgery needs to be very careful in order not to jeopardise the function of these nerves.

The alternative treatment for a growing tumour would be radiotherapy. For smaller tumours this can be a specialised form of radiotherapy called stereotactic radiosurgery, which is given as a single dose.  This is very effective in halting the growth of these tumours, whilst preserving nerve function.  It does not remove the tumour but in halting its growth, it can very likely prevent it from causing neurological harm. Larger tumours require multiple smaller doses of radiotherapy given the larger volume that requires treating. This is called fractionated radiotherapy.

If you have a larger tumour which has already resulted in loss of the voice and swallowing nerves, and if it is pressing on your brain, then it is very likely that surgery will be advised.  Such surgery is a major undertaking, requiring a whole team to carry it out safely.

The first part of the work-up for surgery will entail having a cerebral angiogram carried out.  This will establish the blood supply of the tumour and whether it is possible to safely block off the blood supply.  Surgery to a glomus jugulare tumour can really only be safely done after effective embolisation of the tumour (occlusion of the blood supply to the tumour) as they are so rich in blood vessels.

After angiography and embolisation, surgery is usually carried out a few days later. This will involve an approach both through the top part of the neck as well as drilling away bone from the base of the skull.  In so doing, the tumour can be approached in a safe way with control of the large blood vessels in the vicinity.  Whilst such operations are rare, we have considerable experience of doing such surgery with good results.

As part of our routine management for patients presenting with a newly diagnosed glomus jugulare tumour, they will be referred on for an opinion from one of our endocrinology colleagues.  The aim of this is two-fold.  Firstly it is to establish that the glomus jugulare tumour is not a secretory tumour.  On occasion, glomus jugulare tumours can secret adrenalin-like hormones and you will be tested for this.  Very rarely, they can also be associated with other sorts of tumours elsewhere in the body, including in the neck or adrenal glands in the abdomen.  You will, therefore, undergo routine screening for this.