Synopsis
Recognise catecholamine-secreting tumours, obtain valid metanephrine testing, prevent drug and procedural crises, coordinate alpha blockade and surgery, and identify inherited risk.
- Phaeochromocytoma arises from adrenal medullary chromaffin tissue; paraganglioma arises from extra-adrenal sympathetic or parasympathetic paraganglia. The umbrella abbreviation is PPGL.
- The classic episodic triad is headache, sweating and palpitations, often with pallor, tremor or panic-like symptoms, but sustained hypertension, incidental imaging or no symptoms are also possible.
- Catecholamine release can cause paroxysmal or sustained hypertension, orthostatic hypotension, cardiomyopathy, arrhythmia, myocardial injury, stroke, pulmonary oedema or multi-organ failure.
Key red flags
Abrupt pounding headache, drenching sweat, forceful palpitations, pallor, tremor and fear lasting minutes to hours with episodic hypertension is the classic pattern. Attacks may be spontaneous or triggered by exertion, anaesthesia, drugs or tumour pressure.
Investigation priorities
Detect functional PPGL using continuously produced catecholamine metabolites.
Management branches
Compatible spells, resistant/labile hypertension, adrenal mass or syndromic context without current crisis.
- Review symptoms, family history, blood-pressure pattern, all medicines and acute confounders, then select plasma or urinary metanephrines with the receiving laboratory's collection instructions.
- For borderline elevation, improve posture/rest and interference conditions and repeat or seek endocrinology advice. For marked elevation, expedite specialist referral and avoid provoking medicines and procedures.
Key medicines
PhenoxybenzamineSpecialist initiation commonly begins at a low oral dose and is titrated over days to pressure and postural response using current BNF guidance.
Selective alpha-1 blockerAgent and dose are chosen by the specialist centre and titrated to seated and standing blood pressure, symptoms and operative timing.