What Exactly Is A Paraganglioma And How Is It Treated?

Many kinds of tumour fall under the category of neuroendocrine tumours, which means they impact the neuroendocrine system, the body’s apparatus for linking together the neurological (nerve) system and the endocrine system (glands that release hormones, such as the pancreas and thyroid).

As the endocrine system includes glands in various parts of the body, neuroendocrine tumours can be found in various parts of the body. One type is a paraganglioma, a rare type that can be found in several locations but is most commonly found in the head, neck, stomach or pelvis.

The rarity of a paraganglioma does not, however, mean it is an untreatable thing to be terrified of. Indeed, the first thing to note is that in most cases it is benign, which means it is not cancerous. Only a fifth of paragangliomas are malignant.

Paragangliomas have no clear cause, although hereditary factors may play some role, and they can occur in patients of any age, although they most commonly appear in people aged between 20 and 50.

Symptoms Of Paragangliomas

In many instances, these tumours are asymptomatic, so patients may be unaware that they have them. When symptoms do arise, these commonly include headaches or high blood pressure. Headaches and a pounding heart are also among the known symptoms, caused when the tumour releases too much adrenaline or noradrenaline into the bloodstream.

This is one of the most important reasons for treating these tumours. Even if they are benign, some will grow and embed themselves more deeply into the surrounding tissue.

Not only does this make it harder to remove them, but the amounts of adrenaline and noradrenaline they can release can have increasingly severe effects and may ultimately become life-threatening.

A key characteristic of these tumours is that they grow very slowly. This is normal for any kind of benign tumour, but in this case, it can still occur with malignant tumours. This can make an early diagnosis of cancer very hard. Cancer is often only detected when it has spread or recurred.

Methods such as MRI scans and urine tests can help detect a paraganglioma, after which a further examination will determine the course of action.

The Role Of Radiotherapy In Treatment

Like any benign tumour, some clinicians will favour a conservative approach that involves monitoring it. However, you may seek a second opinion and even private treatment if you are unhappy with the situation, such as if you are enduring notable and persistent symptoms.

Treatments usually start with surgery to remove the tumour, but, as can often be the case for tumours, the location may inhibit this, either making total excision or even partial excision a risky step. This is particularly true if, for example, it is next to the carotid artery. In other instances, severing an adjacent nerve may lead to a loss of feeling or function.

It is in instances like this that radiotherapy will be particularly useful. As with any tumour, the process works by directing radiation at the tumour, disrupting its DNA. This causes tumour cells to die and also prevents them from being able to replicate. This effect is the same on benign and malignant tumours alike, causing them to shrink.

How Radiotherapy Is Given

The delivery of radiation may work in two ways. External beam therapy will direct radiation beams at the tumour in a very precise fashion, using stereotactic radiotherapy to ensure the radiation is concentrated in exactly the right area while minimising exposure for surrounding tissue and therefore limiting the side effects.

Another method of delivery is internal radiation therapy, which involves either an injection or ingestion of a radioactive substance to place it next to the affected area.

Radiotherapy may be used in conjunction with other treatments, for example, as a means of shrinking the remaining part of a tumour if surgery has only been able to remove some of it. Other treatments used in combination with radiotherapy include chemotherapy and targeted therapies, with these often being deployed in cases of malignant tumours.

Further radiotherapy may be used in the event of a malignant paraganglioma metastasising, which is where secondary cancer develops.

When Can Observation Be Appropriate?

Small, asymptomatic, non-secretory tumours may be monitored with periodic imaging and biochemistry, particularly in older patients or when surgery/radiation risks outweigh benefits.

What Should You Do Next?

For most patients, the overall prognosis is very good, with a benign tumour posing no cancer problem and timely treatment preventing it from growing and producing higher levels of adrenaline and noradrenaline that can lead to severe and potentially dangerous symptoms.

However, because a paraganglioma is a rare condition, diagnosis may be delayed as more commonly occurring alternatives are investigated. Even when diagnosed, its rarity means patients cannot always be sure of getting the ideal treatment.

In such cases, you should contact us, as we understand the condition well and how to provide the right kind of treatment for your specific circumstances. If you need radiotherapy for a paraganglioma, we’re happy to review your case and advise on options.

Explore the Queen Square website to learn more about our advanced radiotherapy treatments for paraganglioma.