Pheochromocytoma

Causes and Risks:
The tumor may be single or multiple and usually develops in the medulla (center or core) of one or both adrenal glands . Sometimes the tumors occurs outside the adrenal gland, usually within the abdomen. Less than 10% of the tumors are malignant (cancerous). The tumors may occur at any age, but they are most common in young to mid-adult life. A common clinical feature is a paroxysm (attack of symptoms) that may be frequent but sporadic, and may increase in frequency, duration, and severity.

Prevention:
There is no known prevention.

Symptoms:

Additional symptoms that may be associated with this disease:



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. Continuous monitoring of all vital signs is necessary in the postoperative period in an intensive care unit. Stabilization of the person's vital signs with medication prior to surgery is important and may require hospitalization. In the case of an inoperable tumor, management with medication is necessary. Radiation therapy or chemotherapy have not been effective in destroying the tumor.

Prognosis:
With surgery, the 5-year survival rate is 95%, with recurrence less than 10%. Hormone secretion of norepinephrine and epinephrine returns to normal after surgery. In malignant tumors, the 5-year survival after surgery is less than 50%.

Complications:
High blood pressure may not be cured in one-fourth of the people 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.

Call Your Healthcare Provider:
Call your health care provider if you have symptoms of pheochromocytoma.

Call your health care provider if you have had a pheochromocytoma in the past and symptoms recur.


The endocrine system is the chemical control center of the body. The pituitary gland is often considered the master control; sending out hormones that regulate the function of the thyroid, adrenal glands, ovaries, and testis. Over- or under-production of hormones from any of these glands is associated with many different types of disease.




This CT scan of the upper abdomen shows an adrenal metastasis (spreading of a tumor to the adrenal gland, above the kidney) in a person with lung cancer.




CT scan of the upper abdomen in a person with a right adrenal mass. The adrenal glands are located above the kidneys.




This X-ray shows a tumor of the adrenal gland, which is located above the kidney. A dye that is visible on X-rays has been injected into the blood stream (the procedure is called an intravenous pyelogram; IVP).




Flushing on the chest and shoulders: Patchy redness is apparent across the chest and upper shoulders. Flushing may occur in response to diseases such as carcinoid, pheochromocytoma, mastocytosis; emotional changes; and other conditions.