PassFinals
Endocrinology & Metabolic

Phaeochromocytoma

A catecholamine-secreting tumour of adrenal chromaffin tissue causing episodic or sustained catecholamine excess, with headache, palpitations, sweating and potentially life-threatening cardiovascular instability.

In a nutshell

A catecholamine-secreting adrenal or extra-adrenal chromaffin tumour. Think of PPGL when episodic or sustained hypertension accompanies headache, palpitations and sweating, but confirm biochemically. Before surgery, alpha-blockade comes first; beta-blockade before it can precipitate crisis.

Classic presentation

Episodes of headache, palpitations and sweating, with pallor, anxiety or hypertension; some patients instead have sustained hypertension, an adrenal incidentaloma or few symptoms.

Key points

  • The symptom triad is a prompt to test, not a diagnostic rule: PPGL can be asymptomatic or present as an adrenal incidentaloma.
  • Use plasma free or urinary fractionated metanephrines through the local laboratory pathway; do not rely on a catecholamine level taken between attacks.
  • Confirm biochemical excess before anatomical imaging, then localise and stage with specialist-directed imaging.
  • Establish alpha-blockade before adding a beta-blocker; the reverse sequence risks unopposed alpha-mediated vasoconstriction.
  • Consider genetic assessment for early-onset, bilateral, extra-adrenal, recurrent or metastatic disease, SDH abnormalities or a relevant family history.
  • High-salt and fluid intake after alpha-blockade helps reverse blood-volume contraction before surgery, with specialist monitoring for hypotension.
  • After resection, monitor blood pressure, heart rate and glucose, confirm biochemical remission and arrange long-term specialist surveillance.

First-line investigation

Plasma free or 24-hour urinary fractionated metanephrines via the local specialist laboratory; biochemical confirmation should precede imaging.

Management

Recognise crisis and refer urgently

  • Treat severe hypertension with chest pain, dyspnoea, neurological symptoms, arrhythmia or acute heart failure as an emergency and involve specialist endocrine or neuroendocrine services urgently.1,4

Confirm biochemically before localisation

  • Use plasma free or urinary fractionated metanephrines through the local laboratory pathway, address pre-analytical factors and obtain specialist interpretation of borderline or discordant results before imaging.2,3

Prepare safely for surgery

  • Under specialist supervision, establish alpha-blockade, then add a beta-blocker only if tachycardia persists; increase salt and fluid intake after alpha-blockade when appropriate to reverse blood-volume contraction.4,3,5

Use an experienced PPGL surgical team

  • Proceed to definitive resection only after specialist pre-operative preparation; operative planning depends on tumour site, size, multifocality, local extension and metastatic disease.4,3,1

Monitor after resection and lifelong when risk is high

  • Monitor blood pressure, heart rate and glucose after surgery, confirm biochemical remission after recovery and arrange specialist long-term surveillance; lifelong annual follow-up is especially important for younger, hereditary, large-tumour or paraganglioma presentations.3,6,1

Exam traps

  • Never start a beta-blocker before adequate alpha-blockade: this is a classic exam trap that precipitates a hypertensive crisis.
  • A normal catecholamine level taken outside an attack does not exclude PPGL; use metanephrines through the laboratory pathway.
  • Imaging should follow, not precede, biochemical confirmation unless a specialist team identifies an exceptional reason.
  • An adrenal incidentaloma still needs the appropriate endocrine assessment; absence of symptoms does not make PPGL safe to ignore.

Illustrations

Catecholamine surge and the paroxysmal triadDiagram linking episodic adrenaline/noradrenaline release from a chromaffin cell tumour to headache, palpitations and sweating.PassFinals · original
Alpha- before beta-blockade sequenceTimeline diagram showing establishment of alpha-blockade followed by beta-blockade before surgery, contrasted with the hypertensive crisis risk of reversing the order.PassFinals · original

Key sources

  1. NHS Genomics Education Programme GeNotes: Inherited phaeochromocytoma and paraganglioma (UK genomic testing criteria, hereditary patterns, specialist surveillance and cascade-testing principles; last reviewed August 2025.)
  2. North West London Pathology: Metanephrines (urine) (NHS laboratory guidance on first-line urinary metanephrines, indications, pre-analytical considerations and interpretation pathway; last updated September 2025.)
  3. Endocrine Society: Pheochromocytoma and Paraganglioma clinical practice guideline (2014) (Specialist guideline for biochemical testing, imaging after biochemical confirmation, peri-operative preparation, post-operative monitoring and biochemical follow-up where no more specific UK guidance is available.)
  4. Society for Endocrinology: Peri-operative preparation of patients with phaeochromocytoma/paraganglioma (updated 2017) (UK specialist position statement for pre-operative alpha blockade, beta-blocker sequencing, volume expansion and peri-operative targets.)
  5. BNF online: adrenergic blockers and peri-operative prescribing (Current UK prescribing, contraindication, interaction and monitoring cross-check for alpha- and beta-adrenoceptor blockers; consult the live monographs before prescribing.)
  6. European Society of Endocrinology: Long-term follow-up after surgery for phaeochromocytoma or paraganglioma (2016) (Specialist follow-up guideline supporting risk-stratified and lifelong surveillance after PPGL surgery.)

This page is exam revision material, not medical advice, and must not be used for patient care. Always check drug doses against the BNF and current guidance. Full disclaimer.