Pheochromocytoma

Also called Chromaffin Tumor

A pheochromocytoma is a rare tumor of the adrenal gland that results in the body's receiving too much epinephrine and norepinephrine. These are hormones that control your metabolism, your blood pressure and your heart rate. Some tumors secrete just norepinephrine while other tumors secrete epinephrine. Still others will secrete both hormones.

The causes of pheochromocytoma are not really known. It involves a single tumor or multiple different tumors in the adrenal gland tissue so that too much of the epinephrine or norepinephrine hormones are secreted. It develops in the inner aspect or medulla of the adrenal gland. Rarely, this can be a tumor that occurs outside the adrenal gland. In such a situation, the tumor usually ends up within the abdomen somewhere. Both adrenal glands can be involved or just one. Some pheochromocytomas are cancerous but this is a rare occurrence. The tumors can show up at any age but are more common in adults who are middle aged.

The symptoms of pheochromocytoma include abdominal pain, pain in the chest and irritability. The person always feels nervous and has a pale skin. There are palpitations of the heart or a rapid heart rate. There can be severe headaches, unusual sweating and weight loss without trying to lose weight. In other cases, there can be a hand tremor, high blood pressure and sleeping difficulties.

The symptoms can come and go and can be highly unpredictable. The spells last about fifteen to twenty seconds and may increase in frequency, severity and length as the tumor gets bigger and puts out more hormones.

Doctors can diagnose pheochromocytoma once it is anticipated. Anyone with refractory high blood pressure or spells of rapid heart rate, fever or anxiety associated with physical symptoms should be considered to have a pheochromocytoma so that the tests for the disease can be performed.

The main test is a CT scan or MRI scan of the abdomen which will show the pheochromocytoma on the film. It looks like one or more round defects in the otherwise normal adrenal tissue. An adrenal biopsy can show the pheochromocytoma and can be done under CT or ultrasound guidance. A glucose test or a catecholamine blood test can reveal the presence of a pheochromocytoma. A metanephrine blood test can show the presence of the disease and a test called a MIBG scinticscan can be performed. The urine can be tested for the presence of catecholamines, or breakdown products of epinephrine or norepinephrine.

Treatment of a pheochromocytoma includes removing the tumor using surgery. The first thing that is done is to stabilize the heart rate and blood pressure with medications so that the patient can tolerate the surgery. This may take a hospitalization with medications that are helpful in returning the vital signs to a normal state. After the surgery, you are carefully monitored in the intensive care unit so that the blood pressure and heart rate can maintain themselves in the normal position. If the tumor is unable to be removed, the individual needs medications to control the symptoms on a long term basis. The use of chemotherapy and radiation has not been found to be effective in the cure of this type of tumor.

The prognosis of this type of tumor is fairly good. Most people who have a pheochromocytoma that is noncancerous will survive past the five year mark. The tumor recurs or comes back in a different place in less than ten percent of the cases. The levels of norepinephrine and the hormone epinephrine usually return to a normal state after the surgery is over with.

Complications include persistent high blood pressure in about twenty five percent of cases, even after successful surgery. The drug treatments usually control the blood pressure after pheochromocytoma surgery and ten percent recur.