Phaeochromocytoma Nursing science class year 3, university of Zimbabwe, nursing science department

1. Phaeochromocytoma
Sasha Bondi

2. Objectives
 Define Phaeochromocytoma.
 Describe the Anatomy and Physiology of the Adrenal glands.
 Discuss Phaeochromocytoma
 Aetiology
 Assessment
 Diagnosis
 Treatment and perioperative care

3. Definition

4. Anatomy and Physiology
 The adrenal glands are small, triangular glands located on top of each kidney.
(AKA suprarenal glands)
 Each gland has cortex (outer region) and a medulla (inner region).
 Cortex secrets glucocorticoids (cortisol), mineralocorticoids (aldosterone), and
small amount of androgens and oestrogens.
 The medulla has Chromaffin Cells via which catechol amines are secreted
(norepinephrine, epinephrine, small amounts of dopamine)
 It is here in the Chromaffin cells where the tumour develops
 Catechol amines secreted by Chromaffin cells stimulate the sympathetic system
 This increases the heart rate, BP, respiratory rate, and amount of energy available
to the body

6. CONT……
 Catechol amines play a huge role in how organs and tissues work, from the
heart to glucose secretion or storage.
 These hormones have good effects when secreted in normal amounts and at
the right time
 E.g. increases our heart rate and bp why because we need all the extra blood
flow rich in oxygen to flow to our muscles to run and avoid danger
 increasing respiratory rate to ensure enough oxygen in times of trouble
 increase our glucose, the hepatic cells are stimulated to release their stores of
glucose by processes such as glycogenolysis, gluconeogenesis
 our cells will require the extra glucose to enable us to fight or fly
 Cause increase in fat metabolism which is needed for fuel
 increases our basal metabolic rate to increase the amount of energy needed
by the body, also increase in thermogenesis to increase the body
temperature,
 they also make us feel fear and anxiety, which Is a good response in times of
trouble, catechol amines determine how we respond to
danger

7. Aetiology
 1/3 of PCC cases occur when patients inherit a mutated gene from their parents
 Studies have linked several genes to the disease but researchers don’t know how
these genes contribute to formation of the tumour. Associated genetic conditions
 Von Hippel-Lindau disease, cysts and tumours grow in CNS, kidneys, adrenal
glands e.t.c.
 Neurofibroblastoma type 1, tumours on skin and optic nerves
 Multiple endocrine neoplasia type 2, a form of thyroid cancer, develops in
conjunction with PCC
 also believed that hypoxia is linked to development of PCC, conditions that
cause hypoxia;
 Severe hypertension
 Congenital heart disease

8. Assessment
 With PCC, catechol amines are secreted without the appropriate stimulation.
 On assessment patient continuously presents with;
• high bp,
• feelings of impending doom,
• anxiety,
• scared, which might be attributed to an anxiety attack

12. Assessment cont…..
 Fight and flight
 F facial flushing, could be from excessive HTN, big problem may go into a
hypertensive crisis, palpitations,
 I increases heart rate and BP biggest tell-tale sign,
 G glucose will be high, hyperglycaemia why, catechol amines increase release of
glucose into blood stream
 H head aches, tend to be severe due to the increase in BP
 T Tremors

13. Flight and fight continued
 F frequent sweating, because of the increased thermogenesis, increased
metabolic rate and the HBP, episodes of sweating for no apparent reason
 L loss of weight, catechol amines increase metabolism
 I increased anxiety and fear even though nothing is stimulating them
 G growing tumour in adrenal that will put pressure on abdomen which will cause
pain or increased pressure in the abdomen
 H heat intolerance
 T tired and weak, because of the constant stimulation, the anxiety, the HBP, the
tachycardia
 6 Ps of Phaeochromocytoma
 Pressure/paroxysmal bursts, pounding headaches, perspiration, pallor, panic,
palpitations,

14.  These S/S can come in episodes and can be triggered by certain things
 like eating foods rich in tyramine, which plays role in blood pressure regulation,
 lfoods that are aged,
 fermented or pickled like cheese wines specifically red wine, smoked, dried meats
 bananas, sauerkraut, chocolate can trigger these resposnes,
 any type of surgery or trauma to the body, injury, emotional stress or medications
like MAOI can trigger S/S and cause the tumour to start secreting excessive
amounts of catechol amines

15. Diagnosis
 From nursing stand point, need to be aware of disease one way to diagnose is thru
a 24hr urine
 if patient is going home they need education on how to do it.
 In the urine we look for catecholamines and metanephrines which are metabolites
formed when the body breaks down catecholamines
 Blood test can also be conducted to check for plasma metanephrine levels
 if urine/blood has excessive amounts of those chances are there is a tumour and
likely Phaeochromocytoma
 When found high, MRI may be ordered or a CT scan of the adrenal glands to look
for the tumours

16.  High levels of norepinephrine cld cause heart cells to stop functioning-
cause MI, stroke, arrhythmias and organ failure.
 High levels of epinephrines also upset the RAAS system equilibrium,
 this affects blood volume regulation and water balance,
 this causes release of aldosterone, angiotensin 2, renin, all this leads to
vasoconstriction and retention of water increasing blood volume.
 The body of people suffering PCC suppresses the hormones in RAAS system
in order to off set the HTN being caused by the excess norepinephrine in
circulation,
 this results in a large amount of fluid loss in an attempt to keep BP low.

17. Treatment
 Laparascopic surgery-90% success rate
 Surgery has risk and as earlier alluded patients with PCC have extremely
high levels of norepinephrine circulating in their bloodstream,
 recalling that the body will suppress the RAAS system in order to off set the
HTN caused by excess epinephrine,
 sudden removal of the source of all the extra epinephrine without allowing
the body to recover wld cause catastrophically low blood pressure post-op.
 in preps for surgery patients are treated with high levels of sodium in order
to increase blood volume and to prevent the sudden crash of the BP.

18. Treatment……
What to do for the patient
1st thing, monitor vital signs very closely,
patients are hypertensive and tachycardic.
Monitor for hypertensive crisis SBP greater than 180 or DBP greater then 120,
for long period of time this causes damage to the eyes, the brain, the kidneys, and the
heart
signs and symptoms cld be a headache vision impairment/ changes, neuro status changes
and seizures, this increases risk for MI
keep watch and make sure they report chest pain if they have any,
keep watch for neuro status changes cz they are at risk of strokes,
 also monitor for EKG changes and monitor for Hyperglycaemia,

19. Treatment……
 because the tumours constantly releases catechol amines they cause patient to be
heat intolerant,
 they feel anxious,very fearful, they need a calm cool environment to keep em from
being overstimulated otherwise their BP will shoot up even higher and their heart rate
too,
 another thing the alpha adrenergic blocker pre-op, works by blocking norepinephrine
from connecting with the receptor which helps to decrease blood pressure
preventing hypertensive crisis during surgery,
 but u also shud watch for reflex tachycardia,
 when the blood pressure decreases, the body tries to compensate for this, causing
reflex tachycardia as a response, can also cause orthostatic hypotension.
 When BP is under control doctor may also order a beta blocker along with a-blocker
to help control the tachycardia that myt develop also helping to decrease the blood
pressure,
 nurse monitors the blood pressure and HR to make sure they’re responding well to the
meds

20. Treatment…..
 while theyre hospitalised make sure they get a good high calorie diet cz theyre
burning calories and fats at a very high rate so need to make their body Is given
proper nutruition,
 no caffeine or energy drinks or smoking bcz these cause vasoconstriction which
cld exacerbate the vasoconstriction being caused by the catechol amines
already,
 if they re going for the surgery make sure they are aware that if its bilateral or
unilateral, they myt need to be in hormone replacemtn therapy

21. Post-operative care
 PCC are highly vascular tumours
 Surgery causes significant blood loss and possible hypovolaemia
 Patient may need I.V. fluids, colloids and vasopressors
 Close monitoring of BP, electrolytes
 Monitor signs for acute haemorrhage
 Monitor patients pain level and give meds as prescribed
 Clear fluid diet on first post-op day, diet advanced as tolerated
 Bilateral adrenolectomy they’ll have to take glucocorticoids,
mineralcorticoids, for life,
 if its unilateral, they’ll have to be on glucocorticoids for up to 2 years.

22. Nursing Diagnosis
Risk for decreased cardiac output related to increased afterload,
vasoconstriction.
Ineffective tissue perfusion: cerebral, renal, cardiac, related to impaired
circulation.
Decreased cardiac output related to serious dysrhythmia as evedinced by
altered heart rate/rhythm (tachycardia)
Knowledge, deficient regarding condition, treatment plan and lifestyle
changes related to unfamiliarity with information resources.

23. References
 Eunice Kennedy Shriver National Institute of Child Health and Human
development. The proper diagnosis, treatment, genetics and research of
Pheochromocytoma and paraganglioma: genetic screening. Retrieved 6
September 2018
 Registerednursern.com