EM@3AM: Pheochromocytoma

Author: Brit Long, MD (@long_brit, EM Attending Physician, San Antonio, TX) // Reviewed by: Alex Koyfman, MD (@EMHighAK)

Welcome to EM@3AM, an emDocs series designed to foster your working knowledge by providing an expedited review of clinical basics. We’ll keep it short, while you keep that EM brain sharp.

A 23-year-old male presents with recurrent episodes of global headache, flushing, sweating, and palpitations. These episodes first started 1 month ago. They occur several times per day and last less than one hour. He has also noted a 10-pound weight loss since these episodes started. He has no past medical or surgical history.

Exam reveals blood pressure 183/108 mm Hg, HR 1114 beats per minute, RR 18, temperature 98.4 C, saturation 98% on room air. His skin is flushed. Your cardiovascular, pulmonary, abdominal, and motor and sensory exams are otherwise normal.

What is the likely diagnosis?

Answer: Pheochromocytoma

Background: Pheochromocytoma is a rare adrenal gland tumor arising from chromaffin cells in the adrenal medulla, but it may arise from other paraganglia. These extra-adrenal tumors have a greater likelihood of being malignant and metastasize.

  • The prevalence approaches 0.6% in adults who present with hypertension, but the overall incidence is 0.05%.
  • The majority of cases are sporadic.
    • Up to 10% of cases have an underlying condition associated with the tumor: multiple endocrine neoplasia type II, von Hippel-Lindau disease, Sturge-Weber syndrome, neurofibromatosis type I, tuberous sclerosis.


10% Rule

  • 10% arise in locations other than the adrenal gland
  • 10% are malignant (higher rates of metastases to bone, liver, and lung)
  • 10% are bilateral
  • 10% are familial
  • 10% occur in pediatric patients
  • 10% are not associated with hypertension
  • 10% have calcifications



  • Recurrent episodes or paroxysms lasting < 1 hour with headache, anxiety, diaphoresis, flushing, alternating elevated and normal blood pressure, palpitations, tachycardia, tremor
  • End organ injury
    • Cardiac dysfunction (myocardial infarction, dissection, arrhythmia, shock, myocarditis, cardiomyopathy, acute peripheral ischemia)
    • Pulmonary (cardiogenic or non-cardiogenic edema)
    • Gastrointestinal (may prevent with several abdominal pain, acute GI bleeding)
    • Renal (acute kidney injury/failure)
    • Neurologic complications (visual changes, hemorrhagic stroke, seizures, hypertensive encephalopathy)
  • Weight loss



  • Hypertensive emergency, myocardial infarction, sympathetic crashing acute pulmonary edema, stroke, intracranial hemorrhage, PRES, polycystic kidney disease, renal injury/glomerulonephritis, urinary obstruction, Cushing’s syndrome, hyperthyroidism, hyperaldosteronism, volume overload, autonomic dysreflexia, preeclampsia/eclampsia


ED Studies and Assessment:

  • Based on differential and patient presentation, hemodynamic, and respiratory status: CBC, TSH, renal and liver function, troponin, VBG, extended electrolytes, CK, urinalysis, hCG in females
  • Laboratory testing typically includes erythrocytosis, hyperglycemia, hypercalcemia
  • Electrocardiogram (ECG) to evaluate for myocardial ischemia
  • Chest x-ray if respiratory symptoms are present
  • Head imaging (CT) if neurologic symptoms present
  • Abdominal imaging can localize tumor



  • Includes plasma free and fractionated metanephrines; urinary fractionated metanephrines/catecholamines
  • Imaging does not typically differentiate malignant from benign pheochromocytomas
    • CT of the abdomen and pelvis can be used to evaluate for the tumor, with sensitivity approximating 90%
      • Tumors tend to be > 3 cm at presentation; most tumors are present in the abdomen (98%)
      • Tumors tend to enhance most in the portal venous phase
      • Modern day contrast may be used in evaluation and is not a contraindication
    • Ultrasound may demonstrate a sold or cystic mass
    • Magnetic resonance imaging (MRI) has a sensitivity of 98%
    • Other imaging includes nuclear medicine and PET scans


Treatment: Definitive treatment includes surgery, and if the tumor is completely resected, hypertension resolves.  Treatment depends on whether the patient is in hypertensive crisis.

  • Hypertensive crisis
    • Alpha blockade
      • Phentolamine (5 mg IV every 2-4 hours) or phenoxybenzamine
      • Nicardipine may be used
    • Beta blockade several days later
      • This must be started after alpha blockade, as unopposed alpha activity may result in hypertensive emergency
    • Surgical resection



  • Consult endocrinology if suspecting the disease.
  • If disease confirmed and the patient presents with hypertensive crisis, admission is warranted.
  • If the disease is suspected but not confirmed and the patient is stable, discharge with follow up for definitive testing is possible.


  1. Tsirlin A, Oo Y, Sharma R, et al. Pheochromocytoma: a review. Maturitas. 2014;77 (3): 229-38.
  2. Blake MA, Kalra MK, Maher MM, et al. Pheochromocytoma: an imaging chameleon. Radiographics. 2004;24(suppl1): S87-99.
  3. Leung K, Stamm M, Raja A, et al. Pheochromocytoma: the range of appearances on ultrasound, CT, MRI, and functional imaging. AJR Am J Roentgenol. 2013;200 (2): 370-8.
  4. Zuber SM, Kantorovich V, Pacak K. Hypertension in pheochromocytoma: characteristics and treatment. Endocrinol Metab Clin North Am. 2011;40 (2): 295-311
  5. Bessell-Browne R, O’Malley ME. CT of pheochromocytoma and paraganglioma: risk of adverse events with i.v. administration of nonionic contrast material. AJR Am J Roentgenol. 2007;188 (4): 970-4.
  6. Baid SK, Lai EW, Wesley RA, et al. Brief communication: radiographic contrast infusion and catecholamine release in patients with pheochromocytoma. Annals of internal medicine. 2009;150 (1): 27-32.

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