Phaeochromocytoma
A phaeochromocytoma is a catecholamine-secreting adrenal medullary tumour — diagnosed by metanephrines, localised by CT/MRI, and cured by adrenalectomy only after adequate alpha-blockade (alpha before beta, always).
The big picture
Chromaffin cells of the adrenal medulla secrete catecholamines (adrenaline, noradrenaline). A tumour of these cells releases catecholamines, causing episodic or sustained hypertension with the classic triad of headache, sweating and palpitations. Extra-adrenal tumours are called paragangliomas. A notable fraction are hereditary (e.g. MEN2, VHL, SDH, NF1).
Diagnose with metanephrines, block ALPHA before BETA, fill the tank, then operate — never beta-block first and never biopsy.
Mechanism pathway
Tap any step to see why it happens.
Symptom sorter
The common presentation.
Diagnostic algorithm
Each step answers one question. Tap to expand.
Treatment ladder
Cure by adrenalectomy, but only after adequate medical preparation; alpha-blockade precedes beta-blockade, with volume repletion.
Procedure chooser
- Adrenalectomy (laparoscopic for most; open for large/invasive); meticulous anaesthetic/haemodynamic management
Complications
- Hypertensive crisis, arrhythmia, catecholamine cardiomyopathy, stroke
- Intra-operative haemodynamic swings
- Post-resection hypotension
- Crisis if inadequately blocked
- Adequate alpha-blockade and volume repletion before surgery
- Never beta-block first; never biopsy
- Phentolamine for crisis; fluids/vasopressors for post-resection hypotension
Follow-up
- BP and symptom resolution
- Biochemical cure (metanephrines)
- Recurrence/metachronous or metastatic disease
- Postoperative metanephrines to confirm cure; lifelong biochemical surveillance, especially in hereditary disease
- Normotension and normal metanephrines after resection
- Persistent/recurrent catecholamine excess; metastatic disease
- Biochemical recurrence or syndromic surveillance
- Hereditary syndromes, bilateral/recurrent disease, rare malignancy
If treatment fails
If catecholamine excess persists or recurs, ask: residual/recurrent tumour, a second (e.g. contralateral or paraganglioma) tumour, or metastatic disease?
Red flags
Unopposed alpha vasoconstriction → hypertensive crisis. Alpha always first.
Can precipitate a crisis — never biopsy; diagnose biochemically.
Inadequate blockade — treat with phentolamine; ensure preparation next time.
Summary tables
Phaeochromocytoma essentials
| Step | Action |
|---|---|
| Suspect | Spells / resistant HTN / incidentaloma |
| Diagnose | Plasma or urinary metanephrines |
| Localise | CT/MRI (± functional imaging) |
| Prepare | Alpha-blockade → beta-blockade → volume |
| Cure | Adrenalectomy; then surveillance + genetics |
Memory hooks
Headache + sweating + palpitations + hypertension = phaeo.
Diagnose with metanephrines (not biopsy).
ALPHA before BETA — always.
Fill the tank (volume) before surgery.
Think hereditary: MEN2, VHL, NF1, SDH.
Board traps
Resistant hypertension + spells given a beta-blocker → crisis.
Adrenal mass biopsied → hypertensive crisis (unrecognised phaeo).
Cure assumed without postoperative metanephrines or syndrome surveillance.
Clinical cases
A 40-year-old with episodic headaches, sweating and palpitations is found to have resistant hypertension and a 4 cm adrenal mass. Raised plasma metanephrines confirm phaeochromocytoma.
What is the correct preparation before adrenalectomy?