Phaeochromocytoma and paraganglioma (PPGL) are rare tumours that generally lead to excessive secretion of catecholamines. Adrenalin is one such hormone that you may be familiar with. We generally consider phaeochromocytoma and paraganglioma together because they tend to behave like one another and are therefore investigated and managed in a similar fashion. They may also be a manifestation of an underlying genetic problem which requires consideration in every patient with this disease.
The catecholamines produced by these tumours and their breakdown products can circulate in excess and can result in unusual symptoms. Classically, these tumours secrete these hormones in an episodic fashion, leading to acute episodes of headache, facial flushing, anxiety, sweating and high blood pressure. In between episodes, you may feel fine and some patients may be completely symptom free. The problem may also be identified when having a scan for a different reason (adrenal incidentaloma) or if you react unusually to other medical treatment (eg. when undergoing sedation/anaesthetic for an unrelated matter). Without management a phaeochromocytoma or paraganglioma can be dangerous and requires prompt recognition, confirmation of the diagnosis and management.
As with many adrenal problems, investigation involves imaging, blood tests and urine tests. There are many different types of investigation available and your ANZES recognised endocrine surgeon will be able to guide you through this. In most cases, the diagnosis is evident on the initial screening tests.
Once the diagnosis is secured, management should commence quickly. Whilst surgery is generally the next logical step to remove the tumour this cannot be undertaken until the patient is prepared with medication that reduces the impact of the excessively elevated circulating catecholamines. This is because any attempt to manipulate the tumour during the operation can lead to a further surge in catecholamines that can cause problems with blood pressure. It is for this reason that a multidisciplinary team is generally involved in the care of patients with PPGL and medicine required to prepare for surgery will generally be managed by an endocrinologist.
Once adequately prepared, surgical removal of the catecholamine producing tumour (adrenalectomy) is the only way to cure the problem. Experience tells us that this is usually the single most useful way of managing the problem and will ensure that problems associated with the catecholamine excess are corrected. Your ANZES recognised endocrine surgeon will be able to assist you in making these decisions about your health.
Being a neuroendocrine disease, Professor Gundara has a special interest in PPGL and was fortunate to have trained in Australia’s leading research (Kolling Institute) and endocrine surgical unit (Royal North Shore Hospital) when it comes to this disease. He maintains an active research brief in this area.
