Pheochromocytoma

Overview

Pheochromocytoma is a rare tumor that usually develops from one of the body's two adrenal glands, located above each kidney in the back of the upper abdomen. The cancer typically 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, and may occur as a single tumor or as multiple growths.

These tumors 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.

Tumors that develop outside the adrenal glands are called paragangliomas. About 10 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.

Our approach to pheochromocytoma

UCSF is an international leader in endocrinology care. We provide comprehensive consultations, evaluations and treatments for a wide range of adrenal hormone conditions, such as pheochromocytoma.

Surgery is the primary treatment for this disorder. The tumors are usually benign, but in the rare cases of a cancerous tumor, we may use a combination of surgical removal, chemotherapy and radiation therapy. We coordinate patients' surgical care at our adrenal surgery center, which is one of the largest programs of its kind in the United States. The center's team has pioneered innovative methods for diagnosing and treating adrenal disorders, and our surgeons are experts in minimally invasive techniques.

Awards & recognition

Among the top hospitals in the nation
Best in Northern California for diabetes care & endocrinology

Signs & symptoms

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.

Treatments

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.

UCSF Health medical specialists have reviewed this information. It is for educational purposes only and is not intended to replace the advice of your doctor or other health care provider. We encourage you to discuss any questions or concerns you may have with your provider.