Pediatric Pheochromocytoma Differential Diagnoses

Updated: Sep 20, 2019
  • Author: Patricia Myriam Vuguin, MD, MSc; Chief Editor: Max J Coppes, MD, PhD, MBA  more...
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Diagnostic Considerations

Misdiagnosis of pheochromocytoma is not uncommon. A 2009 study revealed that over a 10-year period, the overdiagnosis rate was 23% and the underdiagnosis rate was 25%. [15] The most common causes of overdiagnosis were misinterpretation of borderline biochemical test results and overzealous imaging. The most common cause of underdiagnosis was failure to consider and test for pheochromocytoma.

Overdiagnosis subjected patients to unnecessary adrenalectomy and its complications. Underdiagnosis resulted in dangerous adrenal biopsy or adrenalectomy with hypertensive crisis and nearly doubled the length of stay in hospital.

Pregnancy in women with pheochromocytoma is associated with a maternal and fetal mortality rate of 40-56%. Antenatal diagnosis reduces maternal and fetal mortality rate to 0% and 15%, respectively.

Adrenal incidentalomas are defined as asymptomatic adrenal masses occasionally discovered during high-resolution imaging procedures, such as CT scanning or MRI. Pheochromocytoma must be excluded before any invasive diagnostic or therapeutic procedure.

All adrenal tumors with suggestive radiological findings (attenuation values expressed in Hounsfield units), most functional tumors, and all tumors larger than 4 cm that lack characteristic benign imaging features should be surgically excised. All patients should undergo hormonal evaluation for subclinical Cushing syndrome and pheochromocytoma, and those with hypertension should also be evaluated for hyperaldosteronism.

Annual biochemical follow-up of most patients with adrenal incidentalomas, especially if the tumor is larger than 3 cm, for up to 5 years has been proposed as a safety measure. Patients with adrenal masses smaller than 4 cm and a noncontrast attenuation value of more than 10 Hounsfield units should have a repeat computed tomography study in 3-6 months and then yearly for 2 years. Adrenal tumors with indeterminate radiological features that grow to at least 0.8 cm over 3-12 months may be considered for surgical resection. [16]

Other problems to be considered in the differential diagnosis of pheochromocytoma include the following:

  • Ganglioneuromas

  • Severe anxiety states

  • Autonomic epilepsy

  • Hypertensive crisis associated with paraplegia, tabes dorsalis, lead poisoning, acute intermittent porphyria

  • Monoamine oxidase inhibitor toxicity

Differential Diagnoses