Phaeochromocytoma

Phaeochromocytoma is a rare neuroendocrine tumor of the medulla of the adrenal glands (originating in the chromaffin cells), or extra-adrenal chromaffin tissue that failed to involute after birth, that secretes high amounts of catecholamines, usually norepinephrine, plus epinephrine to a lesser extent. Extra-adrenal paragangliomas (often described as extra-adrenal pheochromocytomas) are closely related, though less common, tumors that originate in the ganglia of the sympathetic nervous system and are named based upon the primary anatomical site of origin.

Signs and symptoms

The signs and symptoms of a pheochromocytoma are variable and are due to the effects of the excessive production of catecholamines, usually norepinephrine and epinephrine. The most common symptoms are palpitations, sweating, headache, and hypertension. Other symptoms can include anxiety, nausea, tremors, and abdominal pain.

Diagnosis

The diagnosis of pheochromocytoma is made through biochemical testing, and once the diagnosis is suspected, imaging studies are performed to locate the tumor. The most commonly used tests are plasma free metanephrines and urinary fractionated metanephrines.

Treatment

The treatment of choice for pheochromocytoma is surgical removal of the tumor. Prior to surgery, patients are typically treated with alpha-adrenergic blocking agents to prevent a hypertensive crisis during the operation.

Prognosis

The prognosis for benign pheochromocytomas that have been completely removed is excellent, with patients often going on to live normal lives. Malignant pheochromocytomas have a less favorable prognosis.

See also

• Adrenal gland
• Neuroendocrine tumor
• Paraganglioma

Phaeochromocytoma Resources
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