2. Case B
36 year old woman is seen at the outpatient clinic
because of intermittent high blood pressure.
She complains about periodic sweating and
panic attacks.
there is intermittent tremor, palpitation and
elevated blood pressure.
During these attacks blood pressure is 210/110
4. Treatment
is surgical excision of the tumor,
following preoperative treatment
of hypertension, which is usually
curative (unless metastatic)
5. Treatment Options for Patients With Pheochromocytoma
Pheochromocytoma Standard Treatment Options
Localized Pheochromocytoma Surgery
Regional Pheochromocytoma Surgery
Metastatic Pheochromocytoma Surgery
Palliative therapy
Recurrent Pheochromocytoma Surgery
Palliative therapy
6. Preoperative Medical Preparation
• Alpha-adrenergic blockade
• should be initiated at the time of diagnosis and
maximized preoperatively to prevent potentially life-threatening
cardiovascular complications, which can
occur as a result of excess catecholamine secretion
during surgery.
Complications may include the following:
• Hypertensive crisis.
• Arrhythmia.
• Myocardial infarction.
• Pulmonary edema.
7. Phenoxybenzamine
(a nonselective alpha-antagonist)
is the
usual drug of choice
prazosin, terazosin, and
doxazosin (selective alpha-1-
antagonists) are alternative
choices
Prazosin, terazosin,
and doxazosin are
shorter acting than
phenoxybenzamine
The duration of
postoperative
hypotension is
theoretically less
than with
phenoxybenzamine
• A preoperative treatment
period of 1 to 3 weeks
is usually sufficient
8. • If tachycardia develops
or if blood pressure
control is not optimal
with alpha-adrenergic
blockade
• a beta-adrenergic blocker
(e.g., metoprolol or
propranolol) can be added
but only after alpha-blockade.
• Beta-adrenergic
blockade must never be
initiated before alpha-adrenergic
blockade
9. Sodium nitroprusside and
phentolamine ( rapid acting
alpha blocker ) should be
available in cases sudden
sever hypertension develops.
Propranolol
120-240 mg
daily
Treatment
Phenoxybenzamine (
20 -80 mg daily
initially in divide
doses )
Common side effects include headache,
palpitation, orthostatic hypotension and
tachycardia
10. surgery
• Laparoscopic surgery is being used
more often for tumors smaller than 6
than 6 cm but for larger tumors, an
an open operation is probably safer.
safer.
• Both anterior transabdominal
laparoscopic adrenalectomy as well
well as posterior retroperitoneoscopic
retroperitoneoscopic adrenalectomy
adrenalectomy have been
demonstrated to be safe for the
majority of patients with a modestly
modestly sized.
11. Surgical outcome and post-operative follow-up
Following surgical removal of Pheochromocytoma 80% of patients
are expected to become normotensive.
Around 20% of patients will remain hypertensive without
biochemical evidence of residual tumor, however, due to associated
essential hypertension or due to acquired renovascular changes.
Plasma catecholamine or urinary metanephrines should be
measured two weeks after surgery. If the biochemical tests are still
diagnostically high, residual or metastatic tumor should be
suspected.
Plasma catecholamines or urinary metanephrines should be
measured every three months for the first year and then annually
even in normotensive patients.
12. Summary:
Alpha blockade with phenoxybenzamine is started at least 7 to 10 days
before operation to allow for expansion of blood volume.
Only once this is achieved is beta blockade considered. If beta blockade
is started too soon, unopposed alpha stimulation can precipitate a
hypertensive crisis.
Laparoscopic surgery is being used more often for tumors smaller than 6
cm but for larger tumors, an open operation is probably safer.
Catecholamines are hormones that increase the heart rate, blood pressure, rate of breathing and amount of energy available to the body. Adrenaline is the most common and well-known catecholamine. The adrenal medulla releases extra adrenaline in response to stress. This increase is known as the "fight or flight response"—i.e. the body is ready to fight or run. Pheochromocytomas are rare tumors that make too much adrenaline.
Adequate α-blockade is defined as supine arterial pressure not greater than 160/90
Beta-adrenergic receptor blockade with proranolol is contraindicated until alpha-adrenergic receptor blockade is complete, to avoid unopposed α-
Vasoconstriction
doing so blocks beta-adrenergic receptor-mediated vasodilation and results in unopposed alpha-adrenergic receptor-mediated vasoconstriction, which can lead to a life-threatening crisis