Patient will present as → a 43-year-old female with high blood pressure unresponsive to therapy. She complains of headaches, palpitations, and sweating. She has a history of neurofibromatosis type 1, though without any neurological deficits. She has multiple café-au-lait spots on her body. The ECG demonstrates sinus tachycardia. She is found to be hypertensive to 154/121 mmHg. Her 24-hour urine metanephrines and VMA come back elevated. Her abdominal CT demonstrates an adrenal mass.
Pheochromocytoma is a catecholamine-secreting adrenal tumor that secretes norepinephrine and epinephrine autonomously and intermittently, causing hypertension, palpitations, headaches, excessive sweating
- This is rare and accounts for only 0.1% of patients with HTN
- Over 90% are benign, while 10% are malignant
- Associated with Neurofibromatosis type 1, MEN 2A/2B, and Von Hippel-Lindau disease
Symptoms are episodic: 5 P’s
- Pressure (↑ BP)
- Pain (headache)
- Perspiration
- Palpitations (tachycardia)
- Pallor
Diagnose with 24-hour catecholamines, including metabolites (metanephrine and vanillylmandelic acid)
- MRI or CT of the abdomen to visualize catecholamine-secreting adrenal tumor
Treat with complete adrenalectomy
- Preoperative nonselective α-blockade: phenoxybenzamine or phentolamine x 7-14 days followed by beta blockers to control HTN. DO NOT initiate therapy with beta-blockade to prevent unopposed alpha constriction during catecholamine release triggered by surgery, which could lead to life-threatening HTN.
Osmosis | |
Pheochromocytoma is caused by a tumor in the adrenal medulla. The tumor affects the chromaffin cells and increases the release of catecholamines. Symptoms of pheochromocytoma include episodic hypertension, diaphoresis, and abdominal or chest pain. Interventions include surgery to remove the adrenal tumor and medications, such as alpha-adrenergic blockers, beta-adrenergic blockers, and metyrosine (Demser). Since pressure to the area may release catecholamines and cause severe hypertension, avoid palpating the patient’s abdomen.
Play Video + QuizQuestion 1 |
Supraventricular tachycardia | |
Acute coronary syndrome | |
Aortic dissection | |
Pheochromocytoma
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Question 2 |
Pituitary gland | |
Liver | |
Adrenal gland | |
Testicle | |
Kidney |
List |
References: Merck Manual · UpToDate