phaeochrmocytoma Flashcards
which familial conditions is pheochromocytoma related to?
10% of cases, phaeochromocytoma is associated with familial conditions such as
- MEN 2
- neurofibromatosis 1,
- Von-Hippel-Lindau disease.
what are the rules of 10 for pheochromocytoma?
10% extra-adrenal (paraganglioma)*:
10% bilateral
10% malignant
10% familial
where is the most common extradrenal site for a pheochromocytoma?
organ of Zuckerkandl located at the bifurcation of the aorta.
what are the clinical features?
- episodic headache
- palpitations
- anxiety
- sweating
what are the primary investigations for pheochromocytoma?
- urinary and plasma metanephrines are the first-line investigation; will both be elevated* (97% sensitivity)
- CT abdomen and pelvis: if there is biochemical evidence of a phaeochromocytoma, then CT imaging can be performed to look at the adrenals
what is the first line management?
- laparoscopic adrenalectomy
- but patients have to be medically managed before the surgery:
1. alpha blocker is offered 7-14 days before the surgery for blood pressure and heart rate to normalise- phenoxybenzamine
2. beta blocker- added after to give additional control of blood pressure and arrhythmias. it is not given before alpha blocker as this can precipitate hypertensive crisis
what is second line management?
patients not suited for surgery should be treated with long term antihypertensives
what are the complications of pheochromocytoma?
Hypertensive crisis: patients can present with severe hypertension and evidence of end-organ damage e.g. cerebral haemorrhage, encephalopathy, arrhythmias, myocardial infarction, and renal failure. Requires IV anti-hypertensive therapy e.g. labetalol
why is labetalol the preferred antihypertensive to use for patients with phaeochromocytoma?
Labetalol blocks both alpha and beta receptors
I remember this because it’s lABetalol
