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. 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 which 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)
- Palpitations (tachycardia)
Diagnose with 24 hour catecholamines including metabolites (metanephrine and vanillylmandelic acid)
- MRI or CT of 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, could lead to life threatening HTN.
Acute coronary syndrome