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Definition:
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.
Symptoms:
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 elevated temperature.
Tests include:
Treatment:
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.
Expectations (prognosis):
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.
Complications:
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.
