Pheochromocytoma

Question 1

What is Pheochromocytoma?

Answer 1

Catecholamine secreting tumour

Question 2

Pheochromocytoma Can precipitate life threatening ________.

Answer 2

hypertension, dysrhythmias

Question 3

characteristics of Pheochromocytoma

Answer 3

Weigh 2g-3kg (avg 100g)
well encapsulated,
highly vascular,
reddish brown

Question 4

Where are Pheochromocytomas usually located?

Answer 4

• 98% in abdomen
• 85% in adrenals (medulla)
• 15% extra-adrenal (or paragangliomas)

Question 5

where are Pheochromocytomas Paragangliomas usually located?

Answer 5

in nervous system anywhere from brain to bladder: bladder wall, heart, mediastinum, carotid, organ of Zuckerkandl (in mesenteric arteries)

Question 6

is the catecholamine secretion of Pheochromocytomas continuous or intermittent?

Answer 6

can be either

Question 7

What is the most common catecholamine secreted in Pheochromocytomas- Norepinephrine, epinephrine, or dopamine?

Answer 7

Norepinephrine

Norepinephrine > epinephrine > dopamine

Question 8

Catecholamines effect Alpha adrenergic receptors, this results in?

Answer 8

HTN, increased cardiac contractility, glycogenolysis, gluconeogenesis, intestinal relaxation

Question 9

Catecholamines effect Beta adrenergic receptors, this results in?

Answer 9

increased HR & contractility

Question 10

Do alpha or Beta adrenergic receptors effect contractility?

Answer 10

they both do

Question 11

Pheochromocytoma Triggers

Answer 11

• Anesthesia induction
• Opiates
• Dopamine antagonists (Reglan)
• Cold/Congestion/Cough OTC medications
• Beta-blockers- unopposed alpha condition, if you treat with beta blockers it can make it worse.
• Drugs that inhibit catecholamine reuptake- TCAs, cocaine
• Childbirth

Question 12

Common Pheochromocytoma metastasis sites

Answer 12

bone, liver, lymph nodes

Question 13

malignancy percentage for Pheochromocytoma in adrenals?

Answer 13

10% malignancy

Question 14

malignancy percentage for Pheochromocytoma in Extra-Adrenals?

Answer 14

Extra-Adrenals: 35% malignancy

Question 15

Pheochromocytoma has 30% Association with familial syndromes, such as?

Answer 15

MEN-2, von Hippel-Lindau, Neurofibromatosis type 1

Question 16

0.05-0.2% of hypertensives are due to _________.

Answer 16

pheochromocytoma

Question 17

percentage of pheochromocytoma discovered at autopsy?

Answer 17

50%

Question 18

percentage of pheochromocytoma discovered at incidentally?

Answer 18

10%

Question 19

occurrence rate of pheochromocytoma

Answer 19

0.8/100k/year

Question 20

pheochromocytoma usual age of diagnosis?

Answer 20

Any age, peaks in 20-40 year-olds

Question 21

percentage of pheochromocytoma in children?

Answer 21

10%

Question 22

pheochromocytoma prognosis

Answer 22

• 5 yr survival rate >95%
• Malignant →5 yr survival rate <50%
• Malignancy rate is 10%- dx is critical!

Question 23

Classic Presentation of pheochromocytoma, what four things do you need to have to diagnose this disease?

Answer 23

o Headaches
o Palpitations
o Diaphoresis
o Severe HTN – at some point in history, doesn’t have to be all the time

Question 24

Other Symptoms of pheochromocytoma (not the main 4 for diagnosis)

Answer 24

• Tremors
• Weakness
• Nausea
• Anxiety- easy to diagnose panic attack, know that being sick makes people anxious
• Epigastric pain
• Flank pain
• Constipation

Question 25

Physical Exam Findings of pheochromocytoma

Answer 25

- HTN: paroxysmal in 50% - Volume contraction: postural hypotension - Hypertensive retinopathy signs - Weight loss - Pallor - Fever - Tachyarrhythmias - Neurofibromas, Café au lait spots

Question 26

Complications of pheochromocytoma

Answer 26

Cardiac complications: 1. Myocarditis 2. Cardiomyopathy- increase in musculature of heart 3. Pulmonary edema- if heart is failing fluid will back up into lungs Pregnancy extremely rare: but prognosis if undiagnosed→ Mortality rates mother 48%, fetus 55%

Question 27

Work up/DX of pheochromocytoma | labs

Answer 27

1. Plasma metanephrine: 96 sensitivity/85% specificity 2. 24 hr urinary collection catecholamines/metanephrines: 88% sensitivity, 99.7% specificity 3. Vanillylmandelic acid in urine 4. Guidelines by North American NeuroEndocrine Tumor Society recommend biochemical testing for pheochromocytoma if: symptomatic, adrenal incidentaloma or hereditary risk

Question 28

Work up/DX of pheochromocytoma imaging

Answer 28

1. CT Abdomen 2. MR: preferred in children, pregnant women 3. Scintigraphy: if confirmed with labs but not picked up on CT/MR 4. PET

Question 29

Would you do genetic testing after being diagnosed with pheochromocytoma?

Answer 29

yes, because if it is familial you can pass on to children need To r/o familial (MEN2A): normal PTH, calcium levels

Question 30

pheochromocytoma Management

Answer 30

TOC: surgical resection Alpha and beta blockade required pre-op to prevent intra-op hypertensive crisis - Alpha given a few days before beta blockade so you don’t get unopposed blockade from beta blockade because that can throw pt into crisis Test for plasma metanephrines 2 weeks post op- if within ref range→ resection success