Fast Five Quiz: Adrenal Gland Disorders

Romesh Khardori, MD, PhD

Disclosures

December 11, 2019

Symptoms and signs of pheochromocytoma include the following:

  • Headache

  • Diaphoresis

  • Palpitations

  • Tremor

  • Nausea

  • Weakness

  • Pallor

  • Anxiety, sense of doom

  • Epigastric pain

  • Flank pain

  • Constipation

  • Weight loss

Clinical signs associated with pheochromocytoma include the following:

  • Hypertension

  • Postural hypotension (from volume contraction)

  • Hypertensive retinopathy

  • Weight loss

  • Pallor

  • Fever

  • Tremor

  • Tachyarrhythmias

  • Pulmonary edema

  • Cardiomyopathy

  • Diabetes mellitus

  • Ileus

Sinus tachycardia (presenting as palpitations) is the most common cardiac rhythm abnormality in patients with pheochromocytoma, but more serious ventricular arrhythmias or conduction disturbances may also occur. Other cardiac manifestations include reversible dilated or hypertrophic cardiomyopathy; Takotsubo cardiomyopathy has gained increasing recognition.

Guidelines from the North American Neuroendocrine Tumor Society recommend biochemical testing for pheochromocytoma in the following cases:

  • Symptomatic patients

  • Patients with an adrenal incidentaloma

  • Patients who have a hereditary risk for pheochromocytoma or paraganglioma (extra-adrenal pheochromocytoma)

The choice of diagnostic test should be based on clinical suspicion of a pheochromocytoma. Plasma metanephrine testing has the highest sensitivity for detecting a pheochromocytoma but has a lower specificity. In comparison, a 24-hour urinary collection for catecholamines and metanephrines has a lower sensitivity and a higher specificity.

General laboratory features of pheochromocytoma include the following:

  • Hyperglycemia

  • Hypercalcemia

  • Erythrocytosis

Imaging studies should be performed only after biochemical studies have confirmed the diagnosis of pheochromocytoma. CT or MRI can be used for detection of the disorder. Scintigraphy may be used when these techniques fail to localize the tumor. PET has shown promising results as an imaging modality for pheochromocytoma.

Surgical resection of the tumor is the treatment of choice for pheochromocytoma and usually results in cure of the hypertension. Careful preoperative management is required to control blood pressure, correct fluid volume, and prevent intraoperative hypertensive crises. If pheochromocytoma is found during pregnancy, initiate alpha-adrenergic blockade (with phenoxybenzamine) as soon as the diagnosis is confirmed. Remove the tumor by laparoscopic adrenalectomy as soon as possible during the first 2 trimesters, after proper preparation. Pregnancy need not be terminated. Spontaneous abortion is very likely, however.

Read more about pheochromocytoma.

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