Thursday, 28 October 2010

MRCP revision battle 36.7: Phaeochromocytoma

Phaeochromocytomas are rare, catecholamine (=adrenaline, noradrenaline, dopamine ) producing tumours.

Presentations are variable and can include:
  • headaches
  • visual disturbances
  • chest tightness
  • palpitations
  • hypertension (70% persistent, 30% episodic)
  • hypotension/postural hypotension is also possible
  • faints
  • abdominal pain

The triad of headache, sweating and palpitations is 90% predictive.

Lots of facts to remember about phaeochromocytomas follow the rule of 10%:
10% are familial (= MEN II, neurofibrosis, Von Hippel Lindau)
10% are bilateral, 10% are malignant and10% are extraadrenal

The commonest extraadrenal site is the organ of Zuckerkandl, which is adjacent to the bifurcation of the aorta.

Diagnosis is by VMA in urine and CT/MRI (show up bright white on T2 weighted MRI)

Treatment is surgery with pre-op alpha and beta blockade (alpha blocker of choice = phenoxybenzamine)

Recurrence is possible so these patients should be followed up lifelong.

Thats all for today, hopefully more tomorrow!