Week 5 Crash Course

Gilbert Sterling O
Pheochromocytoma
 Definition
Rare catecholamine secreting tumour derived
from chromaffin cells of the sympathetic system
 Epidemiology
• Most commonly a single tumour of adrenal
medulla
• Rare cause of HTN (<0.2% of all hypertensives)
 Etiology & Patof
• most cases sporadic (80%)
• familial: associated with multiple endocrine
neoplasia II (MEN IIA and IIB) (50% penetrance;
i.e.
• 50% of people with the mutation get
pheochromocytoma), von Hippel-Lindau (10-20%
penetrance),
• paraganglioma (20% penetrance), or
neurofibromatosis type 1 (0.1-5.7% penetrance)
• tumours, via unknown mechanism, able to
synthesize and release excessive catecholamines
 Clinical Features
• 50% suffer from paroxysmal HTN; the rest have
sustained HTN
• classic triad (not found in most patients): episodic
“pounding” headache, palpitations/tachycardia,
diaphoresis
• other symptoms: tremor, anxiety, chest or abdominal
pain, visual blurring, weight loss, polyuria, polydipsia
• other signs: orthostatic hypotension, papilledema,
hyperglycemia, dilated cardiomyopathy
• symptoms may be triggered by stress, exertion,
anesthesia, abdominal pressure, certain foods
(especially tyramine containing foods)
 Lab Test
urine catecholamines (most sensitive)
• increased catecholamine metabolites (metanephrines)
and free catecholamines
• plasma metanephrines if available (most sensitive)
• cut-off values will depend on assay used
Urine VMA
CT abdomen
• if negative, whole body CT and meta-iodo-
benzoguanidine (MIBG) scintigraphy, Octreoscan, or
MRI
 Tx
• surgical removal of tumour (curative) with careful pre- and post-
operative ICU monitoring
adequate pre-operative preparation
• α-blockade for BP control: doxazosin or calcium channel blockers
(10-21 d pre-operative), IV phentolamine (perioperative, if
required)
• β-blockade for HR control once α blocked for a few days
• metyrosine (catecholamine synthesis inhibitor) +
phenoxybenzamine or prazosin
• volume restoration with vigorous salt-loading and fluids
• rescreen urine 1-3 mo post-operatively
• screen urine in first degree relatives; genetic testing in patients <50
yr old