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Updated: Jun 19 2020


  • 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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