Updated: 6/19/2020

Pheochromocytoma

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Snapshot
  • A 40-year-old gentleman presents with episodic headaches and palpitations. Attributing it to his tendency to worry excessively, he put it off for several months until he began to sweat episodically too. In the clinic, his blood pressure is found to be elevated at 160/120 mmHg.  Plasma metanephrines are elevated and an abdominal CT scan reveals an adrenal mass.
Introduction
  • Nonmalignant tumor of adrenal medulla
    • most common adrenal tumor in adults (vs neuroblastoma, most common in children)
    • composed of chromaffin cells that are normally stimulated by acetylcholine 
    • secretes catecholamines causing episodic hypertension
  • Pathogenesis
    • from chromaffin cells of neural crest origin
  • Epidemiology
    • 40-50 years old
  • Rule of 10’s
    • 10% malignant
    • 10% bilateral
    • 10% kids
    • 10% extra-adrenal
      • bladder, organ of Zuckerkandl (bifurcation of aorta)
    • 10% calcify
  • Associated conditions
    • von Hippel-Lindau disease
    • MEN 2A
    • MEN 2B
    • neurofibromatosis type 1
Presentation
  • Symptoms are episodic  
    • 5 P’s
      • Pressure (↑ BP)
        Pain (headache)
      • Perspiration
      • Palpitations (tachycardia)
      • Pallor
    • mediated by tumor secretion of epinephrine, norepinephrine, and dopamine
  • Physical exam
    • ↑ BP (diastolic and systolic increase due to alpha adrenergic stimulation resulting in vasoconstriction)
Evaluation
  • Serology
    • ↑ free metanephrine level
  • 24-hour urine collection for diagnostic confirmation
    • ↑ vanillyl mandelic acid (breakdown of norepinephrine and epinephrine)
    • ↑ metanephrines (more sensitive than VMA)
  • Histology
    • chromaffin cells with enlarged dysmorphic nuclei
Differential Diagnosis
  • Hyperthyroidism
  • Carcinoid tumors
  • Panic disorder
  • Therapy resistant hypertension from obstructive sleep apnea
Treatment
  • In this sequence of events
    • α-antagonist phenoxybenzamine to prevent unopposed α-action if β receptors are blocked  
    • β-blocker
    • tumor resection
Prognosis, Prevention, and Complications
  • Prognosis
    • very good with resection
  • Complications
    • metastases
      • bones, lungs, and liver
 

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Questions (5)
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(M1.EC.17.4713) A 34-year-old woman comes to the emergency department complaining of severe headache and anxiety, diaphoresis, and palpitations for the last 30 minutes. She has had several similar episodes over the past few weeks. She has no significant past medical history and has a 10 pack-year smoking history. She takes no illicit drugs. Her blood pressure on arrival is 181/80 mmHg and her pulse is 134/min. If this patient was given metoprolol, how would her blood pressure respond? Tested Concept

QID: 108485
1

Hypotension due to beta-adrenergic receptor blockade

64%

(148/232)

2

Hypotension due to alpha-1-adrenergic receptor blockade

8%

(19/232)

3

Hypertension due to alpha-1-adrenergic receptor blockade

3%

(6/232)

4

Hypertension due to alpha-1-adrenergic receptor stimulation

20%

(46/232)

5

Hypertension due to alpha- and beta-adrenergic receptor blockade

2%

(4/232)

M 3 B

Select Answer to see Preferred Response

(M1.EC.13.64) A 34-year-old female presents to the emergency room with headache and palpitations. She is sweating profusely and appears tremulous on exam. Vital signs are as follows: HR 120, BP 190/110, RR 18, O2 99% on room air, and Temp 37C. Urinary metanephrines and catechols are positive. Which of the following medical regimens is contraindicated as a first-line therapy in this patient? Tested Concept

QID: 104389
1

Phenoxybenzamine

20%

(34/169)

2

Nitroprusside

20%

(33/169)

3

Propranolol

34%

(57/169)

4

Labetalol

7%

(12/169)

5

Lisinopril

10%

(17/169)

M 3 E

Select Answer to see Preferred Response

(M1.EC.12.110) A 40-year-old female presents with palpitations, headache, and episodic diaphoresis. On physical exam she has a blood pressure of 170/90 mmHg. Urine analysis shows elevated levels of vanillylmandelic acid. Figure A shows the histology of the adrenal gland. Hyperactivity of cells in which of the follow regions is likely responsible for this patient's symptoms? Tested Concept

QID: 100411
FIGURES:
1

Region 1

4%

(1/28)

2

Region 2

11%

(3/28)

3

Region 4

18%

(5/28)

4

Region 5

0%

(0/28)

5

Region 6

68%

(19/28)

M 1 E

Select Answer to see Preferred Response

(M1.EC.12.116) A 49 year-old-male presents with a primary complaint of several recent episodes of severe headache, sudden anxiety, and a "racing heart". The patient originally attributed these symptoms to stress at work; however, these episodes are becoming more frequent and severe. Laboratory evaluation during such an episode reveals elevated plasma free metanephrines. Which of the following additional findings in this patient is most likely? Tested Concept

QID: 100417
1

Decreased 24 hour urine vanillylmandelic acid (VMA) levels

0%

(0/41)

2

Episodic hypertension

78%

(32/41)

3

Anhidrosis

2%

(1/41)

4

Diarrhea

10%

(4/41)

5

Hypoglycemia

7%

(3/41)

M 2 E

Select Answer to see Preferred Response

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