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Block 2 FCM > Pheochromocytoma > Flashcards

Pheochromocytoma Flashcards

1
Q

What is Pheochromocytoma?

A

Catecholamine secreting tumour

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2
Q

Pheochromocytoma Can precipitate life threatening ________.

A

hypertension, dysrhythmias

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3
Q

characteristics of Pheochromocytoma

A

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

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4
Q

Where are Pheochromocytomas usually located?

A
  • 98% in abdomen
  • 85% in adrenals (medulla)
  • 15% extra-adrenal (or paragangliomas)
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5
Q

where are Pheochromocytomas Paragangliomas usually located?

A

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

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6
Q

is the catecholamine secretion of Pheochromocytomas continuous or intermittent?

A

can be either

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7
Q

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

A

Norepinephrine

Norepinephrine > epinephrine > dopamine

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8
Q

Catecholamines effect Alpha adrenergic receptors, this results in?

A

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

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9
Q

Catecholamines effect Beta adrenergic receptors, this results in?

A

increased HR & contractility

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10
Q

Do alpha or Beta adrenergic receptors effect contractility?

A

they both do

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11
Q

Pheochromocytoma Triggers

A
  • 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
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12
Q

Common Pheochromocytoma metastasis sites

A

bone, liver, lymph nodes

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13
Q

malignancy percentage for Pheochromocytoma in adrenals?

A

10% malignancy

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14
Q

malignancy percentage for Pheochromocytoma in Extra-Adrenals?

A

Extra-Adrenals: 35% malignancy

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15
Q

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

A

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

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16
Q

0.05-0.2% of hypertensives are due to _________.

A

pheochromocytoma

17
Q

percentage of pheochromocytoma discovered at autopsy?

A

50%

18
Q

percentage of pheochromocytoma discovered at incidentally?

A

10%

19
Q

occurrence rate of pheochromocytoma

A

0.8/100k/year

20
Q

pheochromocytoma usual age of diagnosis?

A

Any age, peaks in 20-40 year-olds

21
Q

percentage of pheochromocytoma in children?

A

10%

22
Q

pheochromocytoma prognosis

A
  • 5 yr survival rate >95%
  • Malignant →5 yr survival rate <50%
  • Malignancy rate is 10%- dx is critical!
23
Q

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

A

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

24
Q

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

A
  • Tremors
  • Weakness
  • Nausea
  • Anxiety- easy to diagnose panic attack, know that being sick makes people anxious
  • Epigastric pain
  • Flank pain
  • Constipation
25
Q

Physical Exam Findings of pheochromocytoma

A
  • HTN: paroxysmal in 50%
  • Volume contraction: postural hypotension
  • Hypertensive retinopathy signs
  • Weight loss
  • Pallor
  • Fever
  • Tachyarrhythmias
  • Neurofibromas, Café au lait spots
26
Q

Complications of pheochromocytoma

A

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%

27
Q

Work up/DX of pheochromocytoma

labs

A
  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
28
Q

Work up/DX of pheochromocytoma imaging

A
  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
29
Q

Would you do genetic testing after being diagnosed with pheochromocytoma?

A

yes, because if it is familial you can pass on to children

need To r/o familial (MEN2A): normal PTH, calcium levels

30
Q

pheochromocytoma Management

A

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

Block 2 FCM (21 decks)

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