Pheochromocytoma

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Pheochromocytoma is a rare disease in which tumors form in chromaffin cells of the body. Most pheochromocytomas start inside the adrenal gland (the adrenal medulla) where most chromaffin cells are located.

Adrenal Glands

There are two adrenal glands, one above each kidney in the back of the upper abdomen. Cells in the adrenal glands make important hormones that help the body work properly. Usually pheochromocytoma affects only one adrenal gland. Pheochromocytoma may also start in other parts of the body, such as the area around the heart or bladder.

Most tumors that start in the chromaffin cells do not spread to other parts of the body and are not cancer. These are called benign tumors. If a tumor is found, the doctor will need to determine whether it is cancer or benign.

Pheochromocytomas often cause the adrenal glands to make too many hormones called catecholamines. The extra catecholamines cause high blood pressure (hypertension), which can cause headaches, sweating, pounding of the heart, pain in the chest, and a feeling of anxiety. High blood pressure that goes on for a long time without treatment can lead to heart disease, stroke, and other major health problems.

Diagnosis

If there are symptoms, a doctor may order blood and urine tests to see if there are extra hormones in the body. A patient may also have a special nuclear medicine scan. A CT scan, an x-ray that uses a computer to make a picture of the inside of a part of the body or an MRI scan, which uses magnetic waves to make a picture of the abdomen, may also be done.

Pheochromocytoma is sometimes part of a condition called multiple endocrine neoplasia syndrome (MEN). People with MEN often have other cancers (such as thyroid cancer) and other hormonal problems.

Treatment

Different types of treatment are available for patients with pheochromocytoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.

Three kinds of standard treatment are used:

  • Surgery (taking out the cancer).
  • Radiation therapy (using high- dose x-rays or other high-energy rays to kill cancer cells).
  • Chemotherapy (using drugs to kill cancer cells).
  • Surgery is the most common treatment of pheochromocytoma. A doctor may remove one or both adrenal glands in an operation called adrenalectomy. The doctor will look inside the abdomen to make sure all the cancer is removed. If the cancer has spread, lymph nodes or other tissues may also be taken out.

Chemotherapy uses drugs to kill cancer cells. Chemotherapy may be taken by pill, or it may be put into the body by a needle in the vein or muscle. 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 uses high-energy x-rays to kill cancer cells and shrink tumors. Radiation comes from a machine outside the body (external radiation therapy).

Localized Benign Pheochromocytoma

Treatment will probably be surgery to remove one or both adrenal glands (adrenalectomy). After surgery the doctor will order blood and urine tests to make sure hormone levels return to normal.

Regional Pheochromocytoma

Treatment may be one of the following:

  • Surgery to remove one or both adrenal glands (adrenalectomy) and as much of the cancer as possible. If cancer remains after surgery, drugs will be given to control high blood pressure.
  • External radiation therapy to relieve symptoms (in rare cases).
  • Chemotherapy. 

Metastatic Pheochromocytoma

Treatment may be one of the following:

  • Surgery to remove as much of the cancer as possible. If cancer remains after surgery, drugs will be given to control high blood pressure.
  • External radiation therapy to relieve symptoms.
  • Chemotherapy.

Recurrent Pheochromocytoma

Treatment may be one of the following:

  • Surgery to remove as much of the cancer as possible. If cancer remains after surgery, drugs will be given to control high blood pressure.
  • External radiation therapy to relieve symptoms.
  • Chemotherapy.

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1 The text and images were reproduced or adapted from the websites of the National Endocrine and Metabolic Diseases Information Service, a service of the National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, http://endocrine.niddk.nih.gov/,.

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