Pheochromocytoma (Adrenal Medulla Tumor)
The early diagnosis of pheochromocytoma is important, not only because it offers the possibility of curing high blood pressure, but if left untreated it can become a lethal condition leading to heart attack or stroke. But what are its symptoms and how should it be treated? The following information should help you talk to a urologist about this condition.
What happens under normal conditions?
The adrenal glands are paired endocrine glands that are located on the top of both kidneys. The adrenal glands are triangular shaped and measure about one-half inch in height and three inches in length. Each gland consists of a medulla that is surrounded by the cortex. The medulla is responsible for producing epinephrine also known as adrenaline. The adrenal cortex produces other hormones necessary for fluid and salt balance in the body such as cortisone and aldosterone. Disorders of either the cortex or the medulla can result in hypertension.
What is pheochromocytoma?
Pheochromocytoma is a tumor of the adrenal medulla that produces excess adrenaline. It can be a deadly tumor because of the severe elevation in blood pressure it causes. It usually is not cancerous but may be associated with cancerous tumors in other endocrine glands such as the thyroid.
Who is at risk for pheochromocytoma?
Pheochromocytoma accounts for less than 1 percent of all hypertensive cases. There is no male to female sex preference and it can occur at any age, but is most common in people between the ages of 40 and 60. Ninety percent of cases are sporadic. However, one-third of the cases can be linked to hereditary causes. These are more common in children and include Multiple endocrine neoplasia type 2, Von Hipple-Lindau syndrome neurofibromatosis and hereditary paraganglioma syndrome.
What are the symptoms of pheochromocytoma?
The majority of patients with pheochromocytoma experience hypertension. Only 15 to 20 percent of patients have normal blood pressure. Many patients experience the classic triad of headache, profuse sweating and palpitations. In fact, hypertensive patients with this triad have more than a 90 percent chance of having a pheochromocytoma, whereas individuals with none of these characteristics have less than 1 percent incidence of pheochromocytoma. Other symptoms include anxiety, chest pain, abdominal pain, fatigue, weight loss, vision problems and seizures.
Hypertensive episodes can be sudden or long-lasting. Sudden attacks typically last less than one hour and can occur as frequently as several times a week or as rarely as a few times a year. These attacks are more commonly seen in women. Episodes can be triggered by physical, emotional or pharmacological stimuli including bending over, abdominal pressure, fear, tobacco, histamine and glucagons, as well as foods rich in tyramine (e.g., beer, wine and cheese).
How is pheochromocytoma diagnosed?
If pheochromocytoma is suspected, there are some standard tests that are initially performed, including the 24-hour blood and urine tests to measure the levels of catecholamines or their degradation products. Now there is also a biochemical analysis that tests plasma metanephrines. Traditionally there are three major imaging studies to locate a pheochromocytoma: CT scan, MRI and meta-iodobenzylguanidine (MIBG) scanning.
How is pheochromocytoma treated?
Laparoscopic surgical removal of the tumor is the standard treatment for pheochromocytoma. Before surgery, medications such as alpha-adrenergic blockers are given to block the effect of the hormones and normalize blood pressure. Laparoscopic surgery is a minimally invasive procedure performed under anesthesia. Three to four small incisions are made in the abdomen, the laparoscope and other instruments are inserted into the incisions and the tumor is removed. Hospital stay is one to two days if blood pressure is well controlled. Laparoscopic laparotomy enables the patient to return to normal activities with two weeks. Traditional laparotomy is performed in a hospital under anesthesia and requires five to seven days in the hospital and a four-week recovery period.
What can be expected after treatment for pheochromocytoma?
Hypertension is seen in about 25% of patients after the removal of pheochromocytoma. In the long run, it appears that response to surgical therapy depends on whether the hypertension was sporadic or long lasting. Up to 95 percent of patients with history of sporadic hypertension respond to surgery vs. 75 percent who suffered from long-lasting hypertension.
Long-term follow up of all patients is important because recurrent or metastatic disease may develop in the future. Initial follow up after surgical removal of sporadic pheochromocytoma is recommended at three and six months after surgery. The five-year survival rate after removal of benign pheochromocytoma has ranged from 84 to 96 percent.
Frequently asked question:
Who should be examined for pheochromocytoma?
People with the following may want to be examined:
- difficult to control hypertension
- more than four blood pressure medications being taken
- have suffered from hypertension before the age of 35 of after the age of 60
- have the following signs or symptoms: severe headaches, excessive sweating, racing heart, anxiety, nervous shaking, pain in the lower chest or upper abdomen, nausea, weight loss or intolerance to heat
Reviewed: January 2011
Last updated: April 2013