Phaeochromocytoma explained: symptoms and treatment

What a phaeochromocytoma is

The adrenal glands are two small glands, one above each kidney, that make hormones including adrenaline. A phaeochromocytoma is a tumour that grows in the core of an adrenal gland and produces too much of these stress hormones, releasing them into the bloodstream in bursts. Most are non-cancerous, though a minority can spread. A closely related tumour called a paraganglioma can grow in similar hormone-producing tissue elsewhere in the body. Because the hormones involved are the same ones the body releases in a fight-or-flight response — speeding the heart and tightening blood vessels — an overload causes the heart to race and blood pressure to climb. Understanding that the tumour is essentially an uncontrolled source of stress hormones helps make sense of its otherwise puzzling, come-and-go symptoms.

The symptoms it causes

The classic combination is episodes of pounding headache, heavy sweating and a fast, thumping heartbeat, often striking together and then easing. Between episodes a person may feel completely well. High blood pressure is common and may be constant or come in sudden surges; sometimes it is very high and hard to control with usual treatments. Other symptoms include anxiety or a sense of panic, tremor, pallor, chest or tummy pain, nausea and weight loss. Episodes can be triggered by exertion, stress, certain foods or medicines, or pressure on the abdomen. Because these symptoms overlap with far more common conditions such as anxiety, panic attacks and ordinary high blood pressure, phaeochromocytoma is often not the first thing considered — which is why the pattern of dramatic, recurring surges is an important clue.

How it is diagnosed

Diagnosis usually starts with tests that measure the breakdown products of stress hormones in the blood or in urine collected over 24 hours; high levels point towards a hormone-producing tumour. Preparation for these tests matters, as some medicines and foods can affect the results, so your clinician will advise. If the hormone tests suggest a phaeochromocytoma, imaging such as a CT or MRI scan is used to locate the tumour in the adrenal gland or elsewhere, and specialised scans can help confirm hormone-active tissue. Because a proportion of these tumours are linked to inherited conditions, genetic testing and specialist advice are often offered, which can also guide screening of relatives. This step-by-step approach — confirm the hormone excess first, then find the source — is important to avoid both missing the tumour and acting on a false alarm.

How it is treated

The main treatment is an operation to remove the tumour, which in many people cures the condition. However, surgery cannot be done safely straight away, because handling the tumour can trigger a dangerous surge of hormones. So before any operation, people are carefully prepared over a period of time with specific medicines that block the effects of the excess hormones and bring blood pressure and heart rate under control; this preparation is essential for a safe operation. The surgery is usually carried out by teams experienced in these tumours, often through keyhole techniques. After removal, hormone levels and blood pressure are monitored, and long-term follow-up is arranged because the tumour can occasionally return or, rarely, spread. Where a tumour cannot be fully removed, other treatments may be used under specialist care.

Living with and after a phaeochromocytoma

Because these tumours are rare and can be linked to inherited conditions, care is usually led by specialists in hormone disorders, often in centres with particular experience. Long-term follow-up with blood or urine tests is important even after successful surgery, to catch any recurrence early, and relatives may be offered screening if an inherited cause is found. Anyone known to have a phaeochromocytoma should make sure other doctors, dentists and anaesthetists are aware before any procedure, as certain medicines and situations can provoke a hormone surge. Learning the personal pattern of symptoms, keeping to follow-up appointments, and knowing when to seek urgent help all support living well. With proper preparation and treatment, the outlook for most people with a non-cancerous phaeochromocytoma is good.