pheochromocytoma
PHEOCHROMOCYTOMA
Pheochromocytoma is a rare adrenal gland tumor that may precipitate life-threatening hypertension.
It can occur at any age but usually occurs during young to mid-adult life.
Majority of patients presents with ≥1 of the following hypertension resistant to standard antihypertensive treatment, hypertensive crisis with malignant hypertension, hypertensive encephalopathy, aortic dissection, or myocardial infarction and paroxysmal symptoms which suggest seizure disorder, anxiety attacks or hyperventilation.

Introduction

  • Pheochromocytomas are catecholamine-secreting tumors arising from chromaffin cells
  • Adrenal medulla is the most common site of origin (approximately 90%)
    • However, tumors may develop anywhere along the abdominal sympathetic chain & are likely to be located near the aorta at the level of the inferior mesenteric artery or at its bifurcation

Etiology

  • Most cases of pheochromocytoma have no known cause
  • In a substantial minority of cases, genetic abnormalities have been identified in some familial forms of pheochromocytoma, as well as those associated with MEN2A and MEN2B, von Hippel-Lindau disease, neurofibromatosis type 1, & paraganglioma syndromes

Signs and Symptoms

  • Pheochromocytoma can occur at any age
    • Usually occurs during young to mid-adult life
  • 10% of patients are asymptomatic

Majority of patients present w/ ≥1 of the following:

  • Hypertension resistant to standard antihypertensive treatment
    • May be associated w/ headaches, excessive sweating &/or palpitations
  • Hypertensive crisis w/ malignant hypertension, hypertensive encephalopathy, aortic dissection or myocardial infarction (MI)
  • Paroxysmal symptoms which suggest seizure disorder, anxiety attacks or hyperventilation
    • Occurs in half of patients
    • Usually sudden in onset but variable in length (eg min-several hr)
    • Associated w/ headache, sweating, palpitations, apprehension, chest or abdomen pain, N/V
    • Pallor may occur
    • Very high BP usually w/ tachycardia

Other Symptoms

  • Increased metabolic rate
    • Mild-moderate wt loss
  • Orthostatic hypotension
    • Results from decreased plasma volume & dulled sympathetic reflexes
  • Cardiac symptoms
    • Sinus tachycardia, sinus bradycardia & other arrhythmias
    • Acute MI or angina may occur even in the absence of coronary artery disease
    • ECG changes
    • Cardiomyopathy
  • Multi-organ system failure w/ noncardiogenic pulmonary edema
  • Carbohydrate intolerance
  • Elevated hematocrit secondary to decreased plasma volume
  • Hypercalcemia
  • Fever, elevated erythrocyte sedimentation rate (ESR)
  • Polyuria, occasionally rhabdomyolysis w/ myoglobinuric renal failure
  • Hypokalemia
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