PANCE Blueprint Endocrinology (6%)

Adrenal neoplastic disease (pheochromocytoma)

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)
  • Perspiration
  • Palpitations (tachycardia)
  • Pallor

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.

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Question 1
A 21-year-old woman presents to the clinic with complaints of palpitations and a headache. On physical examination, the patient is anxious and diaphoretic with a blood pressure of 175/105 mm Hg and heart rate of 122 bpm. ECG demonstrates sinus tachycardia. Based on this presentation, what is a likely diagnosis?
A
Supraventricular tachycardia
B
Acute coronary syndrome
C
Aortic dissection
D
Pheochromocytoma
Question 1 Explanation: 
Pheochromocytoma is related to an increase in catecholamine release with symptoms of palpitations, anxiety, sweating, and headache. Supraventricular tachycardia can present with anxiety and palpitations but is normally not associated with severe hypertension. This patient is not complaining of chest pain and is a young adult, so it is unlikely that the patient has acute coronary syndrome (ACS). The patient is not complaining of chest pain and does not list a history of trauma or connective tissue disease, so it is less likely she has aortic dissection.
Question 2
A 38-year-old man presents to the emergency department experiencing a severe headache and heart palpitations. He appears to be anxious and perspiring heavily. On examination, he is found to be tachycardic and his blood pressure is 158/102 mm Hg. His urine catecholamines are increased. If imaging were performed, what is the most likely location where a lesion would be found?  
A
Pituitary gland
B
Liver
C
Adrenal gland
D
Testicle
E
Kidney
Question 2 Explanation: 
Pheochromocytomas produce, store, and secrete catecholamines. They are usually derived from the adrenal medulla, although they may be found in other locations.
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