Pathophys, Test Iv - Pheochromocytoma, Etc.

Front 1

pheochromocytoma is

Back 1

a tumor of the adrenal medulla

Front 2

pheochoromocytoma tumors are made of what type of cells

Back 2

chromaffin

Front 3

pheochoromocytoma tumors do what

Back 3

produce store and secrete catecholamines

Front 4

what is the typical presentation of pheochoromocytoma tumors

Back 4

severely hypertensive patients

Front 5

what age does pheochoromocytoma tumors occur most commonly

Back 5

usually 3rd to 5th decades of life (equally men and women)
10 - 12% are kids (males)

Front 6

what % of pheochormocytoma tumors have metastasis

Back 6

10%

Front 7

what % of pheochromocytoma tumors are solitary tumors

Back 7

85 - 90% are unilateral - right side

Front 8

do pheochromocytoma tumors tend to be on the left or right adrenal medulla

Back 8

right

Front 9

what percent of adults and children with pheochromocytoma tumors have bilateral tumors

Back 9

10% adults
25% children

Front 10

Le Papier

Back 10

paper

Front 11

characteristics of pheochromocytoma tumors

Back 11

highly vascular with areas of focal hemorrhage and calcification

Front 12

pheochromocytoma occurs in conjunction with what other disorder

Back 12

multiple endocrine neoplasia type IIB (MEN IIB)

Front 13

in addition to pheochromocytoma, people with MEN IIB also tend to have

Back 13

thyroid cancer and tumors of the nerves in the lips, mouth, eyes, and digestive tract

Front 14

pheochromocytoma can progress along

Back 14

the sympathetic nerves

Front 15

do the adrenal glands normally secrete more epi or norepi

Back 15

epi

Front 16

a patient with pheochromocytoma will have adrenal glands that secrete

Back 16

more norepi than epi - but more of both

Front 17

pheochromocytoma tumors secrete

Back 17

epi and norepi

Front 18

what regulates tumor secretion

Back 18

nothing - no neuronal control so tumors release catecholamines whenever they feel like it

Front 19

how does size of tumor affect levels of circulating catecholamines

Back 19

no correlation - but other tumors generally have an inverses relationship between size and amount released

Front 20

triad of pheochromocytoma

Back 20

diaphoresis, tachycardia, headache

Front 21

if someone doesn't have the triad of symptoms for pheochromocytoma

Back 21

they probably don't have pheochromocytoma - the triad is diagnostic and highly specific

Front 22

most common symptom of pheochromocytoma

Back 22

hypertension - 90% of patients

Front 23

50% of patients with pheochromocytoma will have what symptom

Back 23

paroxysmal hypertension (periods of hypertension and hypotension with syncope)

Front 24

are the adrenergic receptors up or down regulated in pheochromocytoma

Back 24

downregulated

Front 25

mechanisms of hypertension associated with pheochromocytoma are

Back 25

both neural and hormonal - increased central sympathetic activity, downregulation of adrenergic receptors, increased plasma renin levels

Front 26

excessive production of a hormone causes what to happen to the receptors

Back 26

downregulation of receptors

Front 27

top 10 symptoms of pheochromocytoma

Back 27

hypertension, headache, tachycardia, diaphoresis, palpatations, weight loss, pallor, nausea, tremors, weakness

Front 28

10 less common symptoms of pheochromocytoma

Back 28

exhaustion, nervousness, anxiety, epigastric pain, chest pain, dyspnea, flushing, numbness, blurred vision, tightness in throat

Front 29

Proxysms are

Back 29

acute catecholamine attacks

Front 30

Proxysms may mimic

Back 30

MH

Front 31

triggers that may cause Proxysms include

Back 31

physical stress, abdominal palpation, defecation, hypotension, activation of the SNS, urination

Front 32

things that are not triggers for Proxysms

Back 32

mental or psychological stress

Front 33

what to think about if surgeon is attempting to remove pheochromocytoma tumor

Back 33

Proxysms- like symptoms of acute cathecholamine release from tumor manipulations

Front 34

10 - 20% of people with pheochromocytoma experience

Back 34

Proxysms

Front 35

when a patient experiences Proxysms - the symptoms can last

Back 35

from minutes to DAYS and are followed by exhaustion

Front 36

death from pheochromocytoma is often due to

Back 36

CHF, MI, and intracerebral hemorrhage

Front 37

symptoms of paroxysms are

Back 37

severe headache, profuse sweating, palpitations, sense of doom, anxiety, orthostatic hypotension

Front 38

people with pheochromocytoma develop CHF because

Back 38

of increased SVR

Front 39

people with pheochromocytoma get orthostatic hypertension because (Theory II that she talked about most)

Back 39

pressure diuresis because of hypertension - so intravascularly dry - but can have flash pulmonary edema - so check the HCT

Front 40

people with pheochromocytoma get orthostatic hypertension because (Theory I)

Back 40

decreased responsiveness by the alpha adrenergic receptors and down regulation of receptor numbers

Front 41

tools for diagnosis of pheochromocytoma

Back 41

urine screen, plasma screen, CT, MRI

Front 42

what do you look for in a urine screen to diagnose pheochromocytoma

Back 42

vanillylmandelic acid (VMA), metanephrines, norepinephrine, catecholamines (elevated levels in a 24 hour urine screen)

Front 43

are plasma or urine screens more sensitive for a pheochromocytoma diagnosis

Back 43

urine scree

Front 44

what do you look for in a plasma screen to diagnose pheochromocytoma

Back 44

epi or norepi levels

Front 45

if you have elevated catecholamine levels but are normotensive you probably have

Back 45

a downregulation of receptors in response to the excessive amounts of catecholamines

Front 46

a single pheochromocytoma tumor is treated

Back 46

laparascopically

Front 47

if the pheochromocytoma tumor has metastasis or multiple tumor sites, then treatment entails

Back 47

open surgery, chemotherapy, radiation

Front 48

if the pheochromocytoma tumor is not excisable treatment is to

Back 48

ablate their blood supply in fluoroscopy

Front 49

if you are going to give your patient an alpha blocker and a beta blocker - which one do you want to give first

Back 49

always give the alpha blocker before the beta blocker - because you want to decrease the SVR before you decrease the HR or else you'll get decreased CO and increased risk of MI

Front 50

preoperative concerns for patients with pheochromocytoma

Back 50

normal blood pressure - blood sugar - heart rate - orthostatic hypotension - MI?- alpha and beta blockers

Front 51

examples of alpha adrenergic blocking agents

Back 51

Phenoxybenzamine (dibenzyline)
doxazosin (cardura)
prozosin (minipres)
trazosin (hytrin)

Front 52

when you give an alpha blocker to your patient, it causes

Back 52

the vasular smooth muscle to relax by preventing release of norepi and therefore improving blood flow and lowering blood pressure. Intravascular plasma volume and hyperglycemia also improve

Front 53

phenoxybenzamine is usually administered to a patient

Back 53

10 - 14 days before surgery to help control preop symptoms of pheochromocytoma

Front 54

beta blockers work by

Back 54

blocking norepis attachment to beta receptors resulting in a slowing of the heart rate and reducing renin release from the kidney

Front 55

intraoperative concerns for a pt with pheochromocytoma

Back 55

chronic hypovolemia
decreased RBC mass and plasma volume
underlying anemia with volume expansion and blood loss
art line
good IV access
CVP is not a bad idea
PA cath with cardiomyopathy
intubate deeply sedated so SNS is completely blocked

Front 56

when Gayle's had pts with pheochromocytoma she's used what but not what

Back 56

art lines and CVP lines, but not PA catheters

Front 57

drugs to avoid in pheochromocytoma

Back 57

ephedrine, ketamine, histamine releasing agents (anything that increases catecholamine secretion)

Front 58

what else should you avoid in surgery with a patient with pheochromocytoma

Back 58

acidosis or alkalosis - ie - keep normoventilation - acidosis would cause increases SNS output and alkalosis would cause increased vasoconstriction

Front 59

how do you treat intraoperative hypertension in a patient with pheochromocytoma

Back 59

phentolamine, nitroprusside, nicardipine

Front 60

what type of drug is nicardipine

Back 60

calcium channel blocker

Front 61

characteristics of nitroprusside

Back 61

rapid onset, short duration, commonly used

Front 62

phentolamine is characterized by

Back 62

slow onset, long duration, associated with tachyphylaxis

Front 63

tachyphylaxis means

Back 63

a rapidly decreasing response to a drug after a few doses have been given

Front 64

would GA or SAB be more effective on a patient with pheochromocytoma

Back 64

either or neither

Front 65

do you want direct or indirect acting drugs to help the patient deal with the tumor removal

Back 65

direct acting

Front 66

what happens to the patient when the pheochromocytoma tumor is removed

Back 66

Fluid resuscitation needed, decreased BP, CVP, urine output - watch bleeding and 3rd space loss of fluids - will need to compensate for loss of catecholamines