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Pheochromocytoma
1.
2. FUNCTIONS OF HORMONES SECRETED
BY ADRENAL GLAND
Adrenal medulla {Fight-or-flight response}
Adrenaline (epinephrine) (Primarily)
Boost the supply of oxygen and glucose to the brain
and muscles (by increasing heart rate and stroke
volume, vasodilation, increasing catalysis of glycogen
in liver, breakdown of lipids in fat cells)
Dilate the pupils
Suppress non-emergency bodily processes (e.g.,
digestion)
Suppress immune system
3. FUNCTIONS OF HORMONES SECRETED
BY ADRENAL GLAND
Noradrenaline (norepinephrine)
Boost the supply of oxygen and glucose to the brain
and muscles (by increasing heart rate and stroke
volume, vasoconstriction and increased blood pressure,
breakdown of lipids in fat cells)
Increase skeletal muscle readiness.
4. PHEOCHROMOCYTOMA
Rare neuroendocrine tumor of the medulla of the adrenal
glands 85-95% cases(originating in the chromaffin cells),
or extra-adrenal chromaffin tissue that secretes excessive
amounts of catecholomines (epinephrine and
norepinephrine) -- hormones that regulate heart rate and
blood pressure.
Rare disorder.
Equally common in women and men.
Disease can occur at any age and rarely occurs after age
60 yrs.
4
5. INCIDENCE
Pheochromocytoma tumors occur in less than 1% of
hypertensive patients and 90% of them are benign.
May occur as a single tumor or as more than one growth.
It usually develops in the center (medulla) of one or both
adrenal glands.
Sometimes this kind of tumor occurs outside the adrenal
gland.
6. Factors associated with
pheochromocytoma include:
A family history of pheochromocytoma.
Tumors in other glands of the body.
Other hormonal disorders.
Genetic diseases.
6
7. Clinical Manifestations
Elevated blood glucose and glucosuria( glycogen is converted into
glucose in liver by catecholamines).
Hypertension : principal manifestation , can be persistent,
fluctuating, intermittent or paroxysmal ( rapid onset and abrupt
cessation) .
Headache.
Increased metabolic rate, diaphoresis, agitation, rapid pulse,
palpitation, emotional outbursts.
Emotional instability.
Nausea, vomiting.
Acute attacks (profuse diaphoresis, dilated pupils, cold
extremities, severe hypertension which can precipitate stroke or
sudden blindness).
8. Diagnosis
HISTORYAND PHYSICAL EXAMINATION
Symptomatic attacks over weeks, months or even yrs
BP change with exertion or emotional stress
Complications in longstanding intractable HTN ( visual
disturbances, manifestations of Heart disease (dyspnea,
edema), manifestations of Kidney damage (
albuminuria,Increase BUN)
9. Diagnosis
Chemical tests of Urinary catecholamines and their
metabolites (metanephrines and Vanillylmandelic acid )
and of plasma catecholamines
Normal range of urinary catecholamines (upto 14mg/100ml
of urine)
Normally amount of VMA (vanillylmandelic acid) is less
than 7mg in 24 hrs
Increase level of these chemicals in pheochromocytoma
10. Diagnosis
Direct assay of catecholamines in the blood
epinephrine : 0.02- 0.2mg/dl
norepinephrine :0.1-0.5mg/dl
X Ray, CT scan, MRI
To confirm adrenomedullary tumor
Nonspecific lab tests
Increase BMR, Blood glucose rises to abnormal levels,
glycosuria
11. Medications
Alpha blockers.
Alpha blockers, also called alpha-adrenergic blocking agents or
alpha-adrenergic antagonists, relax certain muscles and help small
blood vessels remain open. They work by keeping the hormone
noradrenaline (norepinephrine) from stimulating the muscles in
the walls of smaller arteries and veins. This stimulation makes the
vessel walls constrict. Blocking that effect causes the vessels to
remain open and relaxed. This improves blood flow and lowers
blood pressure. Examples of alpha blockers include
phenoxybenzamine (Dibenzyline), doxazosin (Cardura), prazosin
(Minipress) and terazosin (Hytrin).
12. Medications
Beta blockers.
Beta blockers, also known as beta-adrenergic blocking agents, make
the heart beat more slowly and with less force. They work by blocking
the effects of the hormone norepinephrine. This action slows down the
nerve impulses that travel through the heart. Consequently, the heart
doesn't pump as hard because it needs less oxygen and blood. Beta
blockers also slow down the release of the enzyme renin from kidneys,
helping keep blood vessels dilated. Examples of beta blockers include
Atenolol (Tenormin), Metoprolol (Lopressor, Toprol XL) and
Propranolol (Inderal LA).
13. Medications
For a pheochromocytoma, alpha blockers are used first to
return blood pressure back to within normal limits.
Once blood pressure is lowered, beta blockers can help
control a rapid or irregular heartbeat.
Both alpha and beta blockers are used in preparation for
surgery.
17. Nursing management ( pre-op)
Risk for injury related to excessive release of
catecholamines preoperatively
Desired outome is to prevent attacks of acute
paroxysmal HTN
Promote rest and relief from stress
Sedatives
Prohibiting beverages containing caffeine such as
coffe, tea, cola
monitor vitals
18. Nursing management (post op)
Risk for injury related to postop hypotension, hemorrhage
and shock
During immediate postop pd, monitor for shock and hemorrhage
Give IV fluids ( blood,plasma, dextran, glucose in water to maintain
blood vol
monitor BP. Give vasopressors to MAINTAIN BP
Carefully measure urine output ( oliguria indicates shock)
Assess client for manifestations of hemorrhage (dressing for bloody
drainage)
If cortical tissue is resected during sx, assess client for
manifestations of adrenal insufficiency. If both glands removed.
Client must receive cortical replacement for life