PHEOCHROMOCYTOMA (PH or PCC)

i. Rare (benign) tumours arising from the chromaffin cells of the adrenal medulla.
ii. They make, store metabolize and usually but nor always release catecholamines
iii. Extra-adrenal paraganglio mas are closely related through less common tumours that originate in the ganglia of the
sympathetic nervous system.
SIGNS AND SYMPTOMS
Sympathetic nervous system hyperactivity
iv. Diaphoresis (excessive sweating
v. Headaches
vi. Elevated heart rate
a. Skin sensations
b. Flank pain
c. Elevated blood pressure ( including paroxysmal high blood pressure)
d. Orthostatic hypotension ( a fall in systolic bp greater than 20mm Hg or a fall in diastolic pressure greater than 10mm
Hg upon standing.
e. Anxiety resembling a panic attack
f. Pallor
g. Weight loss
h. Elevated blood glucose level ( due to catecholamine stimulation of lipolysis -> high level of free fatty acids
1. Resistant arterial HTN-> hypertensive emergency (one or more organ systems are affected
PHEOCHROMOCYTOMA CRISIS
1. Rare and potentially life-threatening emergency can occur spontaneously or be precipitated
by drugs tumour manipulation micturition or food that aggravate the release of catecholamines.
COMPLICATIONS
1. Damage to heart cells (compromised microcirculation or direct toxic effects
2. HTN, sudden death, heart attack, pulmonary oedema and arrhythmias
CAUSES
1. Genes
a. NF, of neurofibromatosis type 1
b. Neuro endocrine tumours multiple endocrine neoplasia 1 and 2
DIAGNOSIS
1. Measuring catecholamine and metanephines in
Plasma or
24hour urine collection
2. Other causes of adrenaline – like excess should be ruled out- hypoglycemia exercise and
drugs (stimulants, methyldopa dopamine agonists) or ganglion – block >anti – hypertensive.
Various food stuffs (e.g coffee, tea, bananas chocolate, citrus fruits and vanilla
3. Measure HMA and VMA
4. Imaging
i. Computed tomography
ii. MRI
iii. MIBG scan (which is scintigraphy using iodine 123 – marked marked
metaohenzylguanidine
iv. PET scan
5. Pheochromocytomas occur during young and adult life
6. They can form a pattern with other endocrine gland cancers (men1 or men 2) occur in
association with von Hippel landau patients
OTHERS

1. Clonidine – centrally acting alpha – 2 – agonist -> suppression of catecholamine release

2. Chronogram A is elevated

TUMOUR LOCATION

a. 80% are unilateral and solitary, 10% are bilateral and 10% are extra – adrenal
b. In children 25% are bilateral and 25% are extra adrenal
c. Solitary lesions favor the right size
d. Tumour cells – large polyhedral pleomorphic chromaffin cells
e. Extra adrenal – abdomen thorax urinary bladder, neck

DIFFERENTIAL DIAGNOSIS

i. Anxiety disorders
ii. Paragangliomas
iii. Essential HTN
iv. Hyperthyroidism
vi. Insulinoma
vii. Paroxysmal supraventricular or tachycardia
viii. Carcinoid
TREATMENT

ix. Surgical resection

x. Pretreatment with phenoxybenzamine (alpha adrenergic blocker) or short acting
alpha antagonist (prasozin, terazosin or doxazosin)