questions 4

Pheochromocytoma

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Topic updated on 12/17/16 2:56pm

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)
    • 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)
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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Qbank (3 Questions)

TAG
(M1.EC.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? Topic Review Topic

1. Phenoxybenzamine
2. Nitroprusside
3. Propranolol
4. Labetalol
5. Lisinopril

PREFERRED RESPONSE ▶
TAG
(M1.EC.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? Topic Review Topic
FIGURES: A          

1. Region 1
2. Region 2
3. Region 4
4. Region 5
5. Region 6

PREFERRED RESPONSE ▶
TAG
(M1.EC.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? Topic Review Topic

1. Decreased 24 hour urine vanillylmandelic acid (VMA) levels
2. Episodic hypertension
3. Anhidrosis
4. Diarrhea
5. Hypoglycemia

PREFERRED RESPONSE ▶
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