Phaeochromocytoma

How it presents

Classic features are the triad of headache, sweating and palpitations, often in episodes, with high blood pressure that may be paroxysmal or sustained. Many phaeochromocytomas are now found incidentally during imaging for another reason, and present without the full classical picture.

How it is diagnosed

• Plasma free metanephrines or 24-hour urinary fractionated metanephrines are the first-line biochemical tests.⁶
• CT or MRI to localise the tumour, usually with characteristic imaging features on T2-weighted MRI.
• Functional imaging (such as MIBG or Ga-DOTATATE PET) in selected cases, particularly when extra-adrenal disease, bilateral disease or hereditary syndromes are suspected.
• Genetic counselling and testing should be discussed. A significant proportion of phaeochromocytomas are associated with germline mutations (for example in SDHx, RET, VHL, NF1).⁶

Preparation for surgery

Pre-operative alpha-adrenergic blockade (for example with phenoxybenzamine or doxazosin) is started several weeks before surgery to stabilise blood pressure. Beta-blockers are added only after adequate alpha-blockade if needed for heart rate control. Volume loading and a salt-liberal diet in the days before surgery help reduce hypotension after tumour removal. The team you choose should be familiar with this protocol.⁶

How the operation is done

Robotic or laparoscopic adrenalectomy is the preferred approach for most tumours, with careful attention to early ligation of the adrenal vein and minimising tumour handling to limit catecholamine release. Larger tumours, bilateral disease or invasive disease may need an open approach.⁴ Anaesthetic management includes continuous invasive blood pressure monitoring and rapidly titratable vasoactive drugs to manage intra-operative surges and post-removal hypotension.

After surgery

Most patients see a rapid improvement in catecholamine-driven symptoms and blood pressure. Long-term follow-up with annual biochemical testing is standard, because a small proportion develop recurrence or metachronous disease, particularly when an underlying genetic syndrome is present.⁶