Pheochromocytoma is an abnormal group of cells (tumor) that secretes hormones that may raise blood pressure. Pheochromocytomas may appear singly or in groups. In about 90 percent of cases, these tumors develop in the abdomen, most often in the adrenal glands near the kidneys. They may also appear in the chest or neck. About 90 percent of pheochromocytomas are benign. The remaining tumors are cancerous and may spread throughout the body.

Pheochromocytomas are rare. They are estimated to occur in less than 1 percent of patients with high blood pressure. About 800 new cases are diagnosed each year in the United States, with most occurring in patients between the ages of 30 and 50.  Doctors may test for this condition if people are not responding to blood pressure medication or are experiencing certain symptoms (e.g., headaches, dizziness, palpitations).

Any diagnosis of pheochromocytoma will be immediately followed by tests to exactly locate the tumor(s). After the tumor is located, the physician will attempt to determine if it is benign or cancerous.

The standard treatment for pheochromocytoma is surgery after the patient has been stabilized with medication. Stabilization usually includes blood pressure medications to prevent medical emergencies related to elevated blood pressure.  Patients with cancerous tumors may also receive chemotherapy and/or radiation therapy.

About 75 percent of patients will no longer have high blood pressure after treatment of the pheochromocytoma. For the remaining 25 percent, their high blood pressure is usually controllable with medications and other conventional treatments.

Pheochromocytoma is a rare and specialized type of tumor of the chromaffin cells. Chromaffin cells are primarily found in the body’s two adrenal glands, and about 90 percent of pheochromocytomas occur in the adrenal glands. Located above each kidney in back of the upper abdomen, the adrenal glands produce catecholamines, a group of hormones including norepinephrine, adrenaline and dopamine, which help regulate blood pressure and heart rate.

If too many catecholamines are released into the body, the patient will develop severe high blood pressure. Left untreated, high blood pressure can lead to heart attack, stroke or other major health problems.

Pheochromocytoma is sometimes referred to as the “10 percent tumor” because of the number of factors that appear in only about 10 percent of cases, including:

Little is known about the causes of pheochromocytoma. However, there are factors known to be associated with the development of this condition, although the exact mechanisms of their involvement have not been determined. They include:

• Familial pheochromocytoma. About 10 percent of cases occur in members of the same family. This percentage rises to 50 percent if the pheochromocytoma is occurring as part of multiple endocrine neoplasia syndrome (MEN). MEN is a condition in which tumors occur in other glands in the body (such as the thyroid gland) as well as other hormonal disorders.
  
  
• High manganese diets. Excessive amounts of the mineral manganese in the diet may encourage the growth of pheochromocytoma cells. Manganese rich foods include bananas, pineapples, blueberries, green vegetables, legumes, nuts, eggs, rice, whole grains and ginger. Too much manganese can also reduce the amount of iron in the body, which is needed by the adrenal glands to produce hormones, and the amount of copper, which the body needs to break down certain hormones.
  
  
• Iron deficiency. The adrenal glands use iron to produce catecholamines (a type of hormone). If there is too little iron in the body, then catecholamine levels become too low. Because pheochromocytoma tumors also produce catecholamines, low iron levels may actually encourage tumor growth because the tumors are seen as another site for producing catecholamines.
  
  
• Copper deficiency. Copper is used by the body to break down catecholamines. Therefore too little copper in the blood will result in too many circulating catecholamines. The exact relationship between copper deficiency and tumor development is unknown.

Pheochromocytomas excrete hormones that regulate blood pressure and heart rate. Thus, the classic symptoms of pheochromocytoma include tachycardia (rapid heart rate), headache and sweating.

Most patients will also suffer from high blood pressure. The hypertension may be episodic, with rapid spikes in blood pressure, or essential hypertension. Many patients with pheochromocytoma experience sudden attacks of symptoms, which may increase in intensity, frequency and duration.

Besides the classic triad of symptoms, other symptoms associated with elevated catecholamines include:

Signs that may be detected by a physician include:

• Elevated blood pressure (severe, but may come and go)
• Rapid pulse
• Elevated temperature

The first step in diagnosing pheochromocytoma is usually a series of blood tests and/or urine tests to determine if there is an excess of adrenaline hormones in the body. These tests include:

• 24-hour urine testing for catecholamines (a group of hormones produced by the adrenal glands near the kidneys) and their byproducts (metanephrines). The body rids itself of hormones like adrenaline through the urine. Measuring the levels of these substances in the patient’s urine over a 24-hour period (through repeated testing) can determine if the body is producing too much adrenaline. The test can also measure levels of hormone byproducts (substances that are left behind when the hormone is processed by the liver). Because hormone compounds and byproducts are most concentrated in urine, this test is more accurate than blood testing alone in diagnosing pheochromocytoma.
  
  
• Serum blood testing for catecholamines (including metanephrines). The blood test is the fastest detection method available and will usually be one of the first steps in diagnosing pheochromocytoma. However, hormone levels vary greatly and a blood test may only provide a “snapshot” of the body’s hormone levels. Repeat testing and urine testing help clarify the levels.

If a pheochromocytoma is confirmed, the physician will locate the tumor. About 90 percent of tumors are located in the adrenal glands, but they may also be located elsewhere in the abdomen or in the chest or neck. Pheochromocytomas may appear singly or in groups. Tests used to located tumors include:

• CAT (computed axial tomography) scan. A painless type of x-ray that uses a computer to produce detailed cross-sectional images, or “slices,” of parts of the body. Of all imaging tests that are currently available, the CAT scan is best able to produce images of bone, blood and soft tissues. It is the most common and most accurate test available for diagnosing pheochromocytoma and can also give precise measurements (e.g., size and position of tumor) that will assist the physician in treating this disease.
  
  
• MRI (magnetic resonance imaging). A safe and painless test that produces very clear cross-sectional or three-dimensional images of the body’s tissues, even through bone and other obstructions. An MRI works by placing the patient in a chamber surrounded by a magnetic field. The center (nucleus) of every cell of the patient’s body responds to the magnetic force in characteristic ways, allowing a computer to produce the images. It is similar to the CAT scan in terms of accuracy and the type of information it provides. In addition, MRI testing may give a clearer indication of whether the tumor is malignant (cancerous) or benign. However, a biopsy to confirm the tumor type is usually performed.
  
  
• MIBG scintiscan (or adrenal medullary imaging). A radionuclide-imaging test for diagnosing adrenal tumors that is used if the CAT scan or MRI are negative, but the urine and blood tests remain positive for pheochromocytoma. The test is named for the chemical used during the scan (iodine-131–meta-iodobenzylguanidine, MIBG). Radionuclide imaging is a technique in which tiny amounts of radioactive materials (called “tracers”) are introduced into the patient’s body. The tracers emit a certain type of energy called gamma rays, which are detected by special machines. For the MIBG scan, the tracers are attached to substances that the adrenal glands need to produce adrenaline. After they are injected into the body, the tracers will be concentrated in the adrenal glands, making them visible to the special cameras used during the scan.

Finally, the physician will determine if the tumor is cancerous or benign. If cancerous, the physician will find out what stage the malignant tumor has reached. Tests used for this purpose include:

• PET scan (positron emission tomography). This is a form of nuclear imaging that can measure the metabolic rate of organs. It can be helpful in detecting tumors because they operate at a different metabolic rate than the surrounding organ tissue.
  
  
• Biopsy. The surgical removal of a small piece of the tumor for analysis. A biopsy is the best method for determining if a tumor is benign or malignant. The procedure is usually performed under general anesthesia.

Stages of malignant pheochromocytoma are as follows:

• Localized pheochromocytoma. The tumor is present in only one area of the body and has not spread to other tissue.
  
  
• Regional pheochromocytoma. The cancer has spread beyond its original area, usually into the lymph nodes or tissue surrounding the original tumor. Lymph nodes are small structures found throughout the body that produce and store infection–fighting cells.
  
  
• Metastatic pheochromocytoma. The cancer has spread beyond the lymph nodes and tissue immediately surrounding the original tumor into other areas of the body.
  
  
• Recurrent pheochromocytoma. The cancer has returned after being successfully treated in the past. It may occur in the same location as the original pheochromocytoma or in other areas of the body. Recurrent pheochromocytoma is rare.

The standard treatment for pheochromocytoma is surgical removal of the tumor. Before treatment, the patient’s condition will be stabilized. This often includes the use of medications, including alpha blockers and beta blockers, which are used to control blood pressure. These drugs have been shown to reduce the levels of stress hormones. Among patients who cannot tolerate alpha blockers, alternative antihypertensives such as calcium channel blockers may be used.

A few days in the hospital may be required while the patient is being stabilized. After the blood pressure is under control, surgery can be scheduled.

The choice of surgeries depends on the location and number of pheochromocytomas. If the adrenal glands are removed as a part of this process, the procedure may be called an adrenalectomy (surgical removal of an adrenal gland).

Two surgical approaches are used to remove the tumors: standard and laparoscopic. During standard surgery, the physician uses a large incision to gain entry to the tumors. This approach allows the physician to view all the tumors.

In recent years, however, laparoscopic techniques have gained popularity because they reduce trauma for the patient.  Laparoscopic surgery uses three or four small incisions (ports) in the patient’s abdomen rather than one long incision (opening the abdomen). Carbon dioxide is pumped into the abdomen through a port to create more room for the surgery. A fiber optic camera is placed through one port to allow the surgeon to “see” the tumor area. The other ports are used to admit long surgical instruments into the abdomen that the surgeon will use to remove the tumor. Following surgery, no additional treatment is generally necessary for a benign tumor. 

Cancerous pheochromocytomas are treated the same as benign tumors, by surgical removal. However, depending on the stage of the tumor, additional treatment may be necessary, including:

Chemotherapy or radiation therapy may be recommended instead of (or in addition to) surgery for some patients. Chemotherapy may be delivered by pill or through an injection. The drug enters the bloodstream and travels throughout the body. It is considered a systemic treatment because it can kill cancer cells anywhere in the body. Radiation therapy is delivered through a machine (outside the body) that directs high-energy x-rays into the cancer cells to shrink them. Radiation is administered to shrink the tumors and relieve localized symptoms.

After the underlying condition is treated, most patients will no longer show signs of high blood pressure. However, about one–fourth of all pheochromocytoma patients will need to take antihypertensive medications to treat ongoing high blood pressure after their treatment.

With little understanding of the cause of pheochromocytomas, there is no way to prevent them.

Preparing questions in advance can help patients have meaningful discussions with their physicians about their conditions. Patients may wish to ask their doctors the following questions about pheochromocytomas:

1. Can anything other than a pheochromocytoma be causing my high blood pressure?
   
   
2. I had high blood pressure before. How can you be sure it is now related to this tumor?
   
   
3. Will surgery return my blood pressure to normal and end my other symptoms?
   
   
4. Will I notice changes after surgery if you remove my adrenal gland?
   
   
5. Will chemotherapy instead of surgery lower my blood pressure?
   
   
6. If my blood pressure remains high after surgery will I need medication?
   
   
7. Could this pheochromocytoma have caused my stroke?
   
   
8. Why do my symptoms come and go?
   
   
9. If I had a relative with a pheochromocytoma are there symptoms I should watch for?
   
   
10. Will I need to make any lifestyle changes after surgery?