Pheochromocytoma

Learning objectives

• Describe pheochromocytoma
• Recognize the symptoms and signs of pheochromocytoma
• Anesthetic management of a patient with a pheochromocytoma

Definition and mechanisms

• Pheochromocytomas are neuroendocrine tumors arising from chromaffin cells in the adrenal medulla
• Pheochromocytomas synthesize and secrete catecholamines (i.e., dopamine, norepinephrine, and epinephrine)
• The clinical presentation depends on the profile of the catecholamine secretion
  • Norepinephrine: Hypertension
  • Epinephrine: Tachycardia and tachydysrhythmias
• Neuroendocrine chromaffin tumors arising outside of the adrenal medulla are called paragangliomas

Signs and symptoms

• Hypertension
• Headache
• Heavy sweating
• Tachycardia
• Tachydysrhythmias
• Tremors
• Pallor
• Shortness of breath
• Panic attack-type symptoms
• Hyperglycemia
• Intravascular volume depletion (hypovolemia)
• Abdominal pain

Less common symptoms

• Anxiety
• Weight loss
• Blurred vision
• Constipation

Risk factors

• Family history of pheochromocytoma
• Family history of related genetic disorders
  • Multiple endocrine neoplasia, type 2 (MEN 2)
  • Von Hippel-Lindau disease
  • Neurofibromatosis 1
  • Hereditary paraganglioma syndromes

Complications

Hypertension can damage other organs, particularly the cardiovascular system, brain, and kidneys. This damage may result in the following critical conditions:

• Cardiovascular disease
• Stroke
• Kidney failure
• Problems with the nerves of the eye

Treatment

• Primary treatment: Surgery to remove the tumor

Management

Suggested reading

• Connor D, Boumphrey S. Perioperative care of phaeochromocytoma. BJA Education. 2016;16(5):153-158.
• Domi R, Sula H. Pheochromocytoma, the Challenge to Anesthesiologists. Journal Of Endocrinology And Metabolism. 2011;1(3):97-100.

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