Pheochromocytoma - Encyclopedia Information
Encyclopedia for Pheochromocytoma
Pheochromocytoma is a tumor of the adrenal gland that causes excess release of epinephrine
and norepinephrine, hormones that regulate heart rate and blood pressure.
Alternative Names: Chromaffin tumors
Causes, incidence, and risk factors:
Pheochromocytoma may occur as a single tumor or as multiple growths. It usually develops
in the medulla (center or core) 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 malignant (cancerous), with 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.
A common clinical feature is a paroxysm (an attack of symptoms listed below) that
may be frequent but sporadic (occurring at unpredictable intervals). The paroxysms
may increase in frequency, duration and severity as the tumor grows.
- Severe headache
- Rapid heart rate
- Chest pain
- Abdominal pain
- Increased appetite
- Loss of weight
- Additional symptoms that may be associated with this disease:
- Sleeping difficulty
- Hand tremor
- High blood pressure
Signs and tests:
Vital signs (temperature, pulse, rate of breathing, blood pressure) reveal high blood
pressure (hypertension) that may be sustained or episodic, rapid heart rate, and
- An adrenal biopsy that shows pheochromocytoma
- A MIBG scintiscan that shows tumor
- An MRI of abdomen that shows adrenal mass
- An abdominal CT scan that shows mass.
- Elevated urine metanephrine
- Elevated urine catecholamines
- Abnormal results of a glucose test
- Abnormal levels of catecholamines in the blood
The definitive treatment is removal of the tumor by surgery. Stabilization of the
person's vital signs with medication prior to surgery is important, and may require
hospitalization. After surgery, it is necessary to continually monitor all vital
signs in an intensive care unit. In the case of an inoperable tumor, management with
medication is necessary. Radiation therapy or chemotherapy have not been effective
in curing this kind of tumor.
For patients who have non-cancerous tumors that are removed with surgery, 95% of
these patients are still alive after 5 years. The tumors come back in less than 10%
of these patients. Hormone secretion of norepinephrine and epinephrine returns to
normal after surgery.
For patients who have cancerous tumors that are removed with surgery, less than
50% of patients are alive after 5 years.
High blood pressure may not be cured in 25% of patients after surgery, yet control
is usually achieved in these people with standard treatments for hypertension. Recurrence
of tumor may occur in 10% of the cases.
Calling your health care provider:
Call your health care provider if you have symptoms of pheochromocytoma or if you
have had a pheochromocytoma in the past and symptoms recur.
Review Date: 8/3/2004
Reviewed By: Marcia S. Brose, M.D., Ph.D., Assistant Professor, Hematology/Oncology,
The University of Pennsylvania Cancer Center, Philadelphia, PA. Review provided by
VeriMed Healthcare Network.
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