Phaeochromocytoma
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Introduction

Phaeochromocytoma is a neuroendocrine tumour of the medulla of the adrenal glands. It secretes large quantities of  the catecholamines noradrenaline, and to a lesser extent, adrenaline.

 

Epidemiology

Rule of 10: 10% familial, 10% bilateral, 10% extra-renal, 10% malignant

 

Presentation

  • Severe headaches
  • Severe hypertension (eg 210/100) and orthostatic hypotension, both fluctuating
  • Cardiac tachyarrythmias (eg SVTs)
  • A plethora of other symptoms, including flushing+unwell+wt loss (like in hyperthyroid), panic/anxiety, sweating, pallor, hyperglycaemia, pyrexia, abdominal pain …
  • May be worsened by TCA (if you thought it was ps-somatic), beta-blockers, IV contrast

 

Complications

MI, heart failure, dilated cardiomyopathy, arrhythmias, stroke, coma, death

 

Associations

MEN 2a,2b; neurofibromatosis; Von Hippel-Lindau syndrome.

 

Diagnosis

  • Initial best test to rule out phaeochromocytoma: metadrenaline from blood.
  • Confirmed by 3x 24-hour collection of urine catecholamines.
  • CT abdo.
  • Nuclear imaging: MIBG: chrommafin-seeking isotope scan, for extra-renal tumours

 

Management

Medical

  • Stabilise patient: ALPHA-blockers (phenoxybenzamine) BEFORE beta-blockers, to prevent hypertensive crisis from unopposed alpha-stimulation.

Surgery

  • Definitive Rx: surgical resection of adrenal gland.

Life-long follow up

  • Malignant recurrence may present late

References

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Dr Tom Leach

Dr Tom Leach MBChB DCH EMCert(ACEM) currently works as a GP Registrar and an Emergency Department CMO in Australia. He is also a Clinical Associate Lecturer at the Australian National University. After graduating from his medical degree at the University of Manchester in 2011, Tom completed his Foundation Training at Bolton Royal Hospital, before moving to Australia in 2013. He started almostadoctor whilst a third year medical student in 2009. Read full bio

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