Pheochromocytoma

A pheochromocytoma is a chromaffincell tumor of the adrenal medulla that secretes an excessive amount of the catecholamines epinephrine and norepinephrine, which results in severe hypertension, increased metabolism, and hyperglycemia. This disorder is potentially fatal, but the prognosis is generally good with treatment.

A pheochromocytoma can develop at any age, but most commonly occurs in people between ages 40 and 60. Most of the time, a pheochromocytoma is noncancerous (benign), and treatment can return blood pressure to normal.

Causes

According to some estimates, about 0.1 % of newly diagnosed patients with hypertension have pheochromocytoma. Although this tumor is usually benign, it may be malignant in as many as 10% of these patients. It affects all races and both sexes, occurring primarily between the ages of 30 and 40. A pheochromocytoma may result from an inherited autosomal dominant trait.

Signs and symptoms

The most common symptom of pheochromocytoma is high blood pressure, which is sometimes extreme. Each child may experience symptoms differently. Other symptoms may include:

• rapid pulse
• heart palpitations
• headache
• dizziness
• poor weight gain despite good appetite
• growth failure
• nausea
• vomiting
• abdominal pain
• pale skin
• clammy skin
• sweating

The symptoms of pheochromocytoma may resemble other problems or medical conditions. Always consult your child's physician for a diagnosis.

Diagnosis

Your doctor will examine you and take a complete medical history. He or she will order blood tests to check for elevated hormone levels and will do a urine screening. Because pheochromocytomas are usually large, they may show up on an x-ray. However, a CT scan is the most sensitive test for locating these types of tumors. An MRI may also show signs of pheochromocytomas.

Treatment

Surgical removal of the tumor is the treatment of choice. To decrease blood pressure, alpha-adrenergic blockers or metyrosine is given from 1 to 2 weeks before surgery. A beta-adrenergic blocker (propranolol) may also be used after achieving alpha blockade.

Postoperatively, I.V. fluids, plasma volume expanders, vasopressors and, possibly, transfusions may be required for hypotension. Persistent hypertension in the immediate postoperative period can occur.

If surgery isn't feasible, alpha and beta-adrenergic blockers-such as phenoxybenzamine and propranolol, respectively - are beneficial in controlling catecholamine effects and preventing attacks.

Management of an acute attack or hypertensive crisis requires I.V. phentolamine (push or drip) or nitroprusside to normalize blood pressure.

Self-care tips

While there is nothing you can do to prevent pheochromocytomas, in most cases the outlook for recovery is good with early treatment.