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Endocrinology

Pheochromocytoma

Catecholamine-secreting tumour of adrenal medulla chromaffin cells (paraganglioma if extra-adrenal). Causes episodic hypertension, headache, palpitations and sweating; up to 40% are hereditary.

Last reviewed 19 Jul 2026 - MedicoMedics editorial team

Adrenal medulla pheochromocytoma releasing catecholamines

Pathophysiology

Autonomous secretion of noradrenaline, adrenaline and dopamine drives alpha- and beta-adrenergic overstimulation. Associations: MEN2A/2B (RET), von Hippel-Lindau, neurofibromatosis type 1, SDHB/SDHD mutations.

Sources: Endocrine Society CPG Pheochromocytoma/Paraganglioma (J Clin Endocrinol Metab 2014;99:1915); NANETS Consensus on Perioperative Management (Endocr Relat Cancer 2020;27:T1)

Clinical presentation

Classic triad: episodic headache, sweating, palpitations. Sustained or paroxysmal hypertension, orthostasis, pallor, anxiety, weight loss. Precipitants: anaesthesia induction, contrast, beta-blocker alone, tyramine foods.

Sources: Endocrine Society CPG Pheochromocytoma/Paraganglioma (J Clin Endocrinol Metab 2014;99:1915); NANETS Consensus on Perioperative Management (Endocr Relat Cancer 2020;27:T1)

Diagnosis

Plasma free metanephrines (highest sensitivity) or 24-h urinary fractionated metanephrines. Localise with CT/MRI adrenal; if negative, 123I-MIBG or 68Ga-DOTATATE PET. Genetic testing for all patients.

Sources: Endocrine Society CPG Pheochromocytoma/Paraganglioma (J Clin Endocrinol Metab 2014;99:1915); NANETS Consensus on Perioperative Management (Endocr Relat Cancer 2020;27:T1)

Management

Preoperative alpha blockade (phenoxybenzamine 10-14 days) FIRST, then add beta-blocker only after adequate alpha-blockade to prevent hypertensive crisis. High-salt diet and volume expansion. Definitive: laparoscopic adrenalectomy. Lifelong biochemical follow-up (10% malignant, 10% bilateral, 10% extra-adrenal - the 10% tumour).

Sources: Endocrine Society CPG Pheochromocytoma/Paraganglioma (J Clin Endocrinol Metab 2014;99:1915); NANETS Consensus on Perioperative Management (Endocr Relat Cancer 2020;27:T1)

Sample USMLE-style MCQs

Try 5 questions on this topic. Practice hundreds more free with a trial.

Question 1

Highest-yield initial screening test?

Question 2

A patient with pheochromocytoma is scheduled for adrenalectomy. Which sequence is correct?

Question 3

Which syndrome is NOT associated with pheochromocytoma?

Question 4

Classic triad of pheochromocytoma?

Question 5

Percentage of pheochromocytomas that are malignant?

References

Primary guidelines and peer-reviewed sources used for this entry. Reviewed 19 Jul 2026 by MedicoMedics editorial team.

  1. Endocrine Society CPG Pheochromocytoma/Paraganglioma (J Clin Endocrinol Metab 2014;99:1915)
  2. NANETS Consensus on Perioperative Management (Endocr Relat Cancer 2020;27:T1)

Frequently asked

Why give alpha-blockade before beta-blockade?

Unopposed alpha stimulation from beta-blockade can precipitate life-threatening hypertensive crisis and pulmonary oedema.

Which test has best diagnostic yield?

Plasma free metanephrines - sensitivity >95%. Urinary fractionated metanephrines are an alternative.

Which syndromes predispose?

MEN2 (RET), VHL, NF1, familial paraganglioma syndromes (SDHB/SDHC/SDHD).

Is pheochromocytoma always benign?

About 10% are malignant - defined by presence of metastases (not histology). SDHB mutations carry highest malignant risk.

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