Pheochromocytoma

Signs and Symptoms

Pheochromocytoma is a rare tumor that usually develops from one of the body's two adrenal glands, which are located above each kidney in the back of the upper abdomen. The cancer usually develops in the central part of the adrenal glands called the adrenal medulla, which is responsible for producing important hormones such epinephrine, adrenalin, norepinephrine and dopamine. Pheochromocytomas often secrete too much hormone, which can cause high blood pressure, palpitations and headaches. High blood pressure that is left untreated can cause heart disease, stroke and other major health problems. Pheochromocytoma may occur as a single tumor or as multiple growths. Those that develop outside the adrenal glands are called paragangliomas.

About 10 percent to 15 percent of pheochromocytomas are malignant, or cancerous, with the potential to spread to other parts of the body. The condition may occur at any age, but is most common in young and middle-aged adults.

Symptoms of pheochromocytoma result from excessive hormone production and include:

• Headaches that typically are severe
• Excessive sweating
• Accelerated or racing heartbeat, called tachycardia, and palpitations
• Anxiety and/or nervousness, such as feelings of impending death
• Nervous shaking or tremors
• Pain in the lower chest or upper abdomen
• Nausea with or without vomiting
• Weight loss
• Heat intolerance

Diagnosis

Standard tests available to diagnose pheochromocytoma include:

• 24-Hour Urinary Catecholamines and Metanephrines Test -- This measures the production of the different types of hormonal compounds made by the adrenal glands. Since the body excretes these hormones in the urine, a patient's urine is examined after 24 hours to check for increased levels of the hormones. This test measures different types of adrenaline, including epinephrine, norepinephrine and dopamine, as well as the compounds discarded by the liver and kidney.

• Serum Catecholamines -- This study measures the adrenalin compounds in the blood and can detect elevated adrenalin levels

• Computed Tomography (CT) Scans and I-MIBG Scans -- These scans help locate the tumor. If a pheochromocytoma is suspected, the CT scan should be without contrast unless hypertension is controlled.

Treatment

Treatment of pheochromocytoma involves surgery, chemotherapy and radiation therapy.

• Surgery -- Once a diagnosis of pheochromocytoma has been established and the tumor located, the tumor will be surgically removed. One or both adrenal glands may be removed in an operation called adrenalectomy. Your doctor will examine the inside of the abdomen to make sure all the cancer is removed. If the cancer has spread, lymph nodes or other tissues also may be taken out.

• Chemotherapy -- This therapy uses drugs to kill cancer cells. It may be taken orally in pill form or may be administered intravenously, through an IV. Chemotherapy is called a systemic treatment because the drug enters the bloodstream, travels through the body and can kill cancer cells throughout the body.

• Radiation Therapy -- This treatment uses high-energy X-rays to kill cancer cells and shrink tumors. Since radiation comes from a machine outside the body, it is called external radiation therapy.

• Radioisotope Therapy -- This treatment uses high doses of I-MIBG, which cancerous pheochromocytomas may absorb, resulting in shrinkage or even disappearance of the tumors.