Phaeochromocytoma

Original article by Daan de Costa | Last updated on 28/5/2014
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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

Life-long follow up

  • Malignant recurrence may present late