Pheochromocytoma
Also known as: Adrenal medullary tumour, Catecholamine-secreting tumour, Paraganglioma (when outside the adrenal gland)
A pheochromocytoma is a rare tumour of the adrenal gland that produces excessive amounts of the stress hormones adrenaline and noradrenaline. The surges of these hormones cause episodes of severe high blood pressure, pounding headaches, sweating and a racing heart that can come on suddenly and last minutes to an hour. Between episodes, blood pressure may be persistently high or even normal. Most pheochromocytomas are benign, but the hormone surges can trigger strokes, heart attacks and dangerous rhythm disturbances if the tumour goes unrecognized, particularly during surgery or pregnancy. Around a third to 40 percent are linked to inherited genetic conditions. The tumour is diagnosed with blood or urine tests and imaging, and surgical removal after careful medication preparation is usually curative.
At a Glance
- Common symptoms
- Episodes of severe headache, heavy sweating and a rapid or pounding heartbeat, the classic triad, High blood pressure that is persistent or comes in sudden spikes, Pallor, tremor and feelings of intense anxiety or dread during an episode
- Main treatments
- Surgical removal of the tumour, usually laparoscopically, which cures the condition in most cases, Pre-operative preparation for at least 1 to 2 weeks with alpha-blocker medication (such as phenoxybenzamine or doxazosin) to control blood pressure and prevent dangerous surges during surgery, Beta-blockers added after alpha-blockade to control heart rate (never started first, as this can worsen blood pressure)
- Category
- Endocrine · Rare; a very small proportion of people with high blood pressure, with a sizeable share linked to inherited syndromes
- See a doctor if
- Repeated episodes of severe headache, sweating and a racing heart, particularly with high blood pressure
Symptoms
Causes
- A tumour arising from the adrenaline-producing cells of the adrenal medulla
- Inherited mutations in genes such as RET (MEN2), VHL, NF1 and the SDH genes, which account for a substantial minority of cases
- Similar tumours arising outside the adrenal gland (paragangliomas) along nerve pathways
- In most sporadic cases the cause is unknown
Risk Factors
- A family history of pheochromocytoma or paraganglioma
- Inherited syndromes including multiple endocrine neoplasia type 2, von Hippel-Lindau disease and neurofibromatosis type 1
- Age 20 to 50, although it can occur at any age
- A previously treated pheochromocytoma, which can recur
Diagnosis
- Blood or 24-hour urine tests for metanephrines, the breakdown products of adrenaline and noradrenaline
- CT or MRI of the abdomen to locate the tumour
- Specialized nuclear medicine scans (such as MIBG or PET scans) to find tumours outside the adrenal gland or check for spread
- Genetic testing, recommended for everyone diagnosed, given the high rate of inherited cases
- Review of medications and conditions that can raise metanephrine levels and cause false positives
Treatment
- Surgical removal of the tumour, usually laparoscopically, which cures the condition in most cases
- Pre-operative preparation for at least 1 to 2 weeks with alpha-blocker medication (such as phenoxybenzamine or doxazosin) to control blood pressure and prevent dangerous surges during surgery
- Beta-blockers added after alpha-blockade to control heart rate (never started first, as this can worsen blood pressure)
- Increased salt and fluid intake before surgery as advised, to restore blood volume
- Long-term medication, radionuclide therapy or chemotherapy for the minority of tumours that are malignant or cannot be removed
- Lifelong annual follow-up with metanephrine testing, as recurrence can occur years later
- Supportive: avoiding known triggers such as certain medications, heavy exertion or tyramine-rich foods until the tumour is removed
Prevention
- Pheochromocytoma cannot be prevented, but genetic testing allows early detection in affected families
- If you carry a related gene mutation, have regular screening with metanephrine tests as advised
- Report episodic headaches, sweating and palpitations with high blood pressure to your doctor
- Attend lifelong follow-up after treatment
- Tell every doctor, dentist and anaesthetist about your diagnosis, as certain drugs can provoke a crisis
When to See a Doctor
- Repeated episodes of severe headache, sweating and a racing heart, particularly with high blood pressure
- Extremely high blood pressure with chest pain, shortness of breath, severe headache, confusion or vision changes (seek emergency care)
- High blood pressure that is hard to control, develops at a young age, or spikes unpredictably
- An adrenal mass found on a scan
- A family history of pheochromocytoma or related genetic syndromes
Frequently Asked Questions
Related Conditions
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