Adrenal Pheochromocytoma

What is adrenal pheochromocytoma?

• Adrenal (ah-DREE-nal) pheochromocytoma (fee-o-kro-mo-si-TO-mah) is a rare tumor of the adrenal glands. The adrenal glands are triangular-shaped glands that sit on top of the kidneys. They produce hormones and chemicals that maintain and control how different parts of your body work. This includes helping your body respond to stress, and keeping your blood sugar and blood pressure levels normal.
  
  
• Normally, cells divide (split) in a planned way, making more cells only when needed. In adrenal pheochromocytoma, cells grow and divide without control or order, making too much tissue. This increased amount of tissue causes more hormones to be released in the body, which increases blood pressure. Most adrenal pheochromocytomas are benign (noncancerous) and can be treated easily. They may also be malignant (cancerous). Cancer cells from this type of pheochromocytoma may spread to nearby healthy tissue and other areas of the body. Once a malignant pheochromocytoma spreads, the cancer is harder to control. The chances of curing an adrenal pheochromocytoma are better when it is found and treated as soon as possible.

What causes adrenal pheochromocytoma?

An adrenal pheochromocytoma may be sporadic or familial .

• Sporadic: Sporadic means that it occurred for the first time in a family. Sporadic adrenal pheochromocytoma may be due to changes or problems with your genes. A gene is a little piece of information that tells the body what to do or what to make. This is the most common type of adrenal pheochromocytoma and is usually seen among those 40 to 50 years of age.
  
  
• Familial (hereditary): Caregivers think that having a family member with certain disorders may increase your risk for adrenal pheochromocytoma. These disorders may include neurofibromatosis, multiple endocrine neoplasia type 2 syndrome, or von Hippel-Landau disease. A hereditary adrenal pheochromocytoma is most often diagnosed before 40 years of age.

What are the signs and symptoms of adrenal pheochromocytoma?

Sudden and repeated attacks of hypertension (high blood pressure) are the most common sign of adrenal pheochromocytoma. The hypertension is usually accompanied by palpitations (fast and pounding heartbeats), increased sweating, and a severe (bad) headache. These attacks may occur as often as every day or every few months. Usually, the symptoms are the same with each attack. You may also have any of the following:

• Fatigue (more tired than usual) and body weakness.
  
  
• Flushed or pale skin.
  
  
• Nausea (upset stomach), vomiting (throwing up), or a fever.
  
  
• Nervousness and anxiety.
  
  
• Pain in the abdomen (stomach), chest, lower back, or groin (area between the thighs).
  
  
• Trouble breathing.
  
  
• Weight loss.

What may trigger a hypertensive attack of adrenal pheochromocytoma?

The following conditions or substances may trigger your blood pressure to shoot up:

• Certain medicines, such as anesthesia (numbing medicine) or those used for depression, diabetes (high blood sugar), or seizures (convulsions).
  
  
• Contrast material or dye used during tests.
  
  
• Food or beverages, such as cheese and red wine.
  
  
• Injuries or trauma.
  
  
• Physical exertion or stress.
  
  
• Touching the adrenal tumor during surgery.

How is adrenal pheochromocytoma diagnosed?

You may have any of the following:

• Blood and urine tests: Samples of your blood and urine are sent to a lab for tests. These tests measure the amount of hormones and other chemicals in your body.
  
  
• Genetic screening: Genetic screening may be done to confirm if you or other family members have adrenal pheochromocytoma. This will also give your caregiver more information on how to treat your adrenal pheochromocytoma.
  
  
• Imaging tests: You may be given dye through an IV in your vein during one of your tests. The dye helps cancer show up better in the pictures. People who are allergic to shellfish (lobster, crab, or shrimp) may be allergic to this dye. Tell your caregiver if you are allergic to any of these.
  
  
  • Computerized tomography scan: This is also called a CT scan. A special x-ray machine uses a computer to take pictures of your chest, abdomen, or hip area.
    
    
  • Magnetic resonance imaging test: This test is also called an MRI. An MRI uses magnetic waves to take pictures of your chest, abdomen, and adrenal glands. You will need to lie still during a MRI. Never enter the MRI room with an oxygen tank, watch, or any other metal objects. Doing this can cause serious injury. Tell your caregiver if you have any metal implants in your body.
    
    
  • Positron emission tomography scan: This test is also called a PET scan. A PET scan shows how much blood and oxygen is flowing to an area of the adrenal gland and other parts of the abdomen. It may also show areas where there is cancer.
    
    
  • Ultrasound: An ultrasound is a test that looks inside your abdomen. Sound waves are used to show pictures of your adrenal glands, kidneys, or other organs on a TV-like screen.
    
    
  • Scintigraphy: This is a test that looks at the flow or movement of blood through the adrenal glands. You will be given a small amount of dye in an IV. Pictures are then taken of your adrenal glands, abdominal organs, and blood vessels.

How is adrenal pheochromocytoma treated?

Treatment of adrenal pheochromocytoma depends on the signs, symptoms, and type of tumor that you have. You may have any of the following:

• Medicines: Caregivers may give you certain medicines to dilate (widen) your blood vessels to treat high blood pressure. You may also be given medicines to relieve your symptoms, such as an abnormal heartbeat, pain, nausea, or vomiting.
  
  
• Surgery: Caregivers may do open or laparoscopic surgery to remove a part or all of your adrenal gland. Open surgery is done by making an incision (cut) in your abdomen (stomach). Laparoscopic surgery is done by inserting a scope through several small cuts. The scope is a long tube with a magnifying glass, a camera, and a light on the end.
  
  
• Additional treatment: If you have a malignant adrenal pheochromocytoma, you may also have the following:
  
  
  • Chemotherapy:
    
    
    • This medicine, often called chemo, is used to treat cancer. It works by killing tumor cells. Chemotherapy may also be used to shrink lymph nodes that have cancer in them. Once the tumor is smaller, you may need surgery to cut out the rest of the cancer.
      
      
    • Many different chemotherapy medicines are used to treat cancer. You may need blood tests often. These blood tests show how your body is doing and how much chemotherapy is needed. Chemotherapy can have many side effects. Caregivers will watch you closely and will work with you to decrease side effects. Chemotherapy can cure some cancers. Even if the chemotherapy does not cure your cancer, it may help you feel better or live longer.
    
    
  • Radiation: Radiation shrinks tumors and kills cancer cells with x-rays or gamma rays. Radiation may be given after surgery to kill cancer cells that were not removed. It may also be given alone or with chemotherapy to treat cancer.

Where can I get support and more information?

Adrenal pheochromocytoma may be a life-changing condition for you and your family. Accepting that you have an adrenal pheochromocytoma is hard. You and those close to you may feel scared, angry, or sad. These are normal feelings. Talk to your caregivers, family, or friends about your feelings. You may also want to join a support group. This is a group of people who also have adrenal pheochromocytoma. Contact the following for more information:

• American College of Surgeons
  633 N. Saint Clair St.
  Chicago , IL 606113211
  Phone: 1- 312 - 2025000
  Phone: 1- 800 - 6214111
  Web Address: http://www.facs.org
  

• National Cancer Institute
  6116 Executive Boulevard, Room 3036A
  Bethesda , MD 20892-8322
  Phone: 1- 800 - 422-6237
  Web Address: http://www.cancer.gov
  

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The above information is an educational aid only. It is not intended as medical advice for individual conditions or treatments. Talk to your doctor, nurse or pharmacist before following any medical regimen to see if it is safe and effective for you.

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