Pheochromocytoma

Answer 1

Definition

Pheochromocytoma is a rare tumor of the adrenal gland that causes too much release of epinephrine and norepinephrine -- hormones that regulate heart rate and blood pressure.

Alternative Names

Chromaffin tumors; Paraganglionoma

Causes, incidence, and risk factors

Pheochromocytoma may occur as a single tumor or as more than one growth. It usually develops in the center (medulla) of one or both adrenal glands. Sometimes this kind of tumor occurs outside the adrenal gland, usually somewhere else in the abdomen.

Less than 10% of pheochromocytomas are cancerous (malignant), meaning they have the potential to spread to other parts of the body.

The tumors may occur at any age, but they are most common from early to mid-adulthood. About 10% of the time there is a family history of the disease.

Symptoms

• Abdominal pain
• Chest pain
• Flushing
• Increased appetite
• Irritability
• Nervousness
• Palpitations
• Rapid heart rate
• Severe headache
• Sweating
• Weight loss

Other symptoms that can occur with this disease:

• Hand tremor
• High blood pressure
• Sleeping difficulty

There may be an attack of the symptoms listed above (paroxysm). It may occur at unpredictable intervals (sporadic). The paroxysms may increase in frequency, length, and severity as the tumor grows.

Signs and tests

An examination of vital signs can show high blood pressure, rapid heart rate, and fever when taken during an attack of symptoms. These signs can be normal at other times.

Tests include:

• Abdominal CTscan
• Adrenal biopsy
• Catecholamines in the blood
• Glucose test
• MIBG scintiscan
• MRI of abdomen
• Urine catecholamines

Treatment

Treatment involves removing the tumor with surgery. It is important to stabilize blood pressure and pulse with medication before surgery. You may need to stay in the hospital.

After surgery, it is necessary to continually monitor all vital signs in an intensive care unit. When the tumor cannot be surgically removed, medication is needed to manage it. Radiation therapy and chemotherapy have not been effective in curing this kind of tumor.

Expectations (prognosis)

About 95% of patients who have noncancerous tumors that are removed with surgery are still alive after 5 years. The tumors come back in less than 10% of these patients. Release of the hormones norepinephrine and epinephrine returns to normal after surgery.

Less than 50% of patients who have cancerous tumors that spread to the bones, liver, or lung are alive after 5 years.

Complications

High blood pressure may not be cured in 25% of patients after surgery. However, standard treatments can usually control high blood pressure. In about 10% of people, the tumor may return.

Calling your health care provider

Call your health care provider if:

• You have symptoms of pheochromocytoma
• You had a pheochromocytoma in the past and your symptoms return

References

Ferri FF. Ferri's Clinical Advisor. 1st ed. Philadelphia, Pa: Mosby Elsevier; 2009.

Answer 2

Definition

Pheochromocytoma is a rare tumor of adrenal gland tissue. It results in the release of too much epinephrine and norepinephrine, hormones that control heart rate, metabolism, and blood pressure .

Alternative Names

Chromaffin tumors; Paraganglionoma

Causes, incidence, and risk factors

Pheochromocytoma may occur as a single tumor or as more than one growth. It usually develops in the center (medulla) of one or both adrenal glands. Rarely, this kind of tumor occurs outside the adrenal gland, usually somewhere else in the abdomen.

Very few pheochromocytomas are cancerous.

The tumors may occur at any age, but they are most common from early to mid-adulthood.

Symptoms

• Abdominal pain
• Chest pain
• Irritability
• Nervousness
• Pallor
• Palpitations
• Rapid heart rate
• Severe headache
• Sweating
• Weight loss

Other symptoms that can occur with this disease:

• Hand tremor
• High blood pressure
• Sleeping difficulty

Symptom attacks may occur at unpredictable intervals and usually last 15 to 20 minutes. The attacks may increase in frequency, length, and severity as the tumor grows.

Signs and tests

The doctor will perform a physical exam. You may have high blood pressure, rapid heart rate, and fever during an attack of symptoms. Your vital signs can be normal at other times.

Tests include:

• Abdominal CTscan
• Adrenal biopsy
• Catecholamines blood test
• Glucose test
• Metanephrine blood test
• MIBG scintiscan
• MRI of abdomen
• Urine catecholamines

Treatment

Treatment involves removing the tumor with surgery. It is important to stabilize blood pressure and pulse with medication before surgery. You may need to stay in the hospital with close monitoring of your vital signs.

After surgery, it is necessary to continually monitor all vital signs in an intensive care unit. When the tumor cannot be surgically removed, medication is needed to manage it. This usually requires a combination of medications to control the effects of the excessive hormones. Radiation therapy and chemotherapy have not been effective in curing this kind of tumor.

Expectations (prognosis)

Most patients who have noncancerous tumors that are removed with surgery are still alive after 5 years. The tumors come back in less than 10% of these patients. Levels of the hormones norepinephrine and epinephrine return to normal after surgery.

Complications

High blood pressure may not be cured in 25% of patients after surgery. However, standard treatments can usually control high blood pressure. In about 10% of people, the tumor may return.

Calling your health care provider

Call your health care provider if:

• You have symptoms of pheochromocytoma
• You had a pheochromocytoma in the past and your symptoms return

References

Hande KR. Adrenal medulla, catecholamines, and pheochromocytoma. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 246.

Reviewed By

Review Date: 09/26/2010

David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.