Introduction

Phaeochromocytoma is a rare catecholamine secreting tumour.

Epidemiology

  • Incidence: 1.00 cases per 100,000 person-years
  • Peak incidence: 40-50 years
  • Sex ratio: 1:1
Condition Relative
incidence
Fibromuscular dysplasia10.00
Phaeochromocytoma1
<1 1-5 6+ 16+ 30+ 40+ 50+ 60+ 70+ 80+

Pathophysiology

bout 10% are familial and may be associated with MEN type II, neurofibromatosis and von Hippel-Lindau syndrome

Basics
  • bilateral in 10%
  • malignant in 10%
  • extra-adrenal in 10% (most common site = organ of Zuckerkandl, adjacent to the bifurcation of the aorta)

Clinical features

Features are typically episodic

Investigations

  • 24 hr urinary collection of metanephrines (sensitivity 97%*)
  • this has replaced a 24 hr urinary collection of catecholamines (sensitivity 86%)

*BMJ 2012; 344 doi: http://dx.doi.org/10.1136/bmj.e1042 (Published 20 February 2012)

Management

Surgery is the definitive management. The patient must first however be stabilized with medical management:
  • alpha-blocker (e.g. phenoxybenzamine), given before a
  • beta-blocker (e.g. propranolol)