Terms in this set (15)
What is Pheochromocytoma?
Tumor of the adrenal MEDULLA and sympathetic ganglion (from chromaffin cell lines) that produces catecholamines (norepinephrine > epinephrine)
What is the incidence?
Cause of hypertension in 1/500 hypertensive patients ( 10% of U.S. population has hypertension)
Which age group is most likely to be affected?
Any age (children and adults); average age is 40 to 60 years
What are the associated risk factors?
MEN-II, family history, von Recklinghausen disease, von HippelLindau disease
What are the signs/symptoms?
"Classic" triad: 1. Palpitations 2. Headache 3. Episodic diaphoresis
Also, hypertension (50%), pallor S flushing, anxiety, weight loss, tachycardia, hyperglycemia
How can the pheochromocytoma SYMPTOMS triad be remembered?
Think of the first three letters in the word PHEochromocytoma: Palpitations Headache Episodic diaphoresis
What is the most common sign of pheochromocytoma?
What is the differential diagnosis?
Renovascular hypertension, menopause, migraine headache, carcinoid syndrome, preeclampsia, neuroblastoma, anxiety disorder with panic attacks, hyperthyroidism, insulinoma
What diagnostic tests should be performed?
Urine screen: VanillylMandelic Acid (VMA), metanephrine, and normetanephrine (all breakdown products of the catechols)
Urine/serum epinephrine/ norepinephrine levels
What are the other common lab findings?
Hyperglycemia (epinephrine increases glucose, norepinephrine decreases insulin) Polycythemia (resulting from intravascular volume depletion)
What is the classic pheochromocytoma "rule of 10's"?
10% malignant 10% bilateral 10% in children 10% multiple tumors 10% extra-adrenal
What is the preoperative/ medical treatment?
Increase intravascular volume with B-blockade (e.g., phenoxybenzamine or prazosin) to allow reduction in catecholamine-induced vasoconstriction and resulting volume depletion; treatment should start as soon as diagnosis is made ± -blockers
What is the surgical treatment?
Tumor resection with early ligation of venous drainage (lower possibility of catecholamine release/crisis by tying off drainage) and minimal manipulation
What are the possible perioperative complications?
Anesthetic challenge: hypertensive crisis with manipulation (treat with nitroprusside), hypotension with total removal of the tumor, cardiac dysrhythmias
In the patient with pheochromocytoma, what must be ruled out?
MEN type II (almost all cases are bilateral)
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