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.
- Plasma free or urinary fractionated metanephrines are preferred biochemical tests because metabolites are produced more continuously than episodically released catecholamines.
- Sampling conditions, posture, acute illness, obstructive sleep apnoea, stress and several medicines produce false-positive metanephrines. Borderline elevation needs controlled repeat or specialist evaluation, not immediate surgery.
- Establish biochemical evidence before localisation imaging in most non-emergency presentations. Once confirmed, CT or MRI and selected functional imaging define site, multiplicity and metastatic disease.
- Never biopsy an adrenal or paraganglionic mass until phaeochromocytoma has been excluded; needle manipulation can provoke a catastrophic catecholamine crisis.
- Pre-operative alpha-adrenoceptor blockade and salt/fluid preparation are supervised by a specialist MDT. Add beta-blockade only after adequate alpha blockade if tachycardia still requires it.
- The MHRA made domperidone contraindicated in confirmed or suspected phaeochromocytoma in July 2026 because it can trigger severe hypertension; stop it and choose an alternative.
- A substantial minority of PPGL is hereditary. NHS genomic assessment can identify RET, VHL, SDHx, MAX, TMEM127 and other susceptibility genes, changing surveillance and family care.
- Metastatic potential is defined by spread to a site where chromaffin tissue is not normally present, not by benign-looking histology alone; long-term biochemical and imaging surveillance is required.
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.
Labile or resistant hypertension, orthostatic hypotension, atrial or ventricular arrhythmia, Takotsubo-like cardiomyopathy, myocardial infarction with unobstructed arteries or unexplained pulmonary oedema should prompt contextual consideration.
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.
- After biochemical evidence, obtain anatomic and selected functional imaging through the PPGL MDT; do not biopsy the lesion.
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.Postural hypotension, tachycardia, nasal congestion and prolonged postoperative hypotension can occur. Expand volume carefully and coordinate with anaesthesia; pregnancy and major cardiac disease need individual review.
Selective alpha-1 blockerAgent and dose are chosen by the specialist centre and titrated to seated and standing blood pressure, symptoms and operative timing.First-dose and postural hypotension occur, and shorter blockade does not remove intra-operative risk. Salt/fluid planning and experienced anaesthesia remain necessary.