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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

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Pheochromocytoma Glossary
  • abdomen: Also referred to as the belly. It is the part of the body that contains all of the internal structures between the chest and the pelvis.

  • abdominal: in the abdomen, the cavity of this part of the body containing the stomach, intestines and bladder.

  • adrenal: Glands that reside above the kidneys composed of an inner medulla and an outer cortex.

  • adrenal cortex: The outer layer of the adrenal gland.

  • adrenal gland: One of a pair of small glands, each of which sits on top of one of the kidneys. These glands produce hormones that help control heart rate, blood pressure, the way the body uses food and other vital functions.

  • adrenal medulla: Inner portion of the adrenal gland.

  • adrenaline: Also known as epinephrine. Hormone secreted in the adrenal gland that raises blood pressure, produces a rapid heartbeat and acts a neurotransmitter when the body is subjected to stress or danger.

  • aldosterone: An adrenal hormone critical for salt (sodium) balance. Produced by the outer part of the adrenal cortex, its action causes a reabsorption of sodium and secretion of potassium and is critical for the maintenance of blood pressure.

  • alpha-adrenergic blockers: Also known as alpha-blockers. Drugs used to treat high blood pressure and other conditions like an enlarged prostate or stress urinary incontinence.

  • anesthesia: Loss of sensation in any part of the body induced by a numbing or paralyzing agent. Often used during surgery to put a person to sleep.

  • anxiety: A feeling of apprehension, often characterized by feelings of stress.

  • benign: Not malignant; not cancerous.

  • cancer: An abnormal growth that can invade nearby structures and spread to other parts of the body and may be a threat to life.

  • catecholamine: Type of organic compound.

  • cortex: The outer layer of an organ.

  • cortisone: A hormone secreted by the adrenal gland and used to treat rheumatoid arthritis and allergies.

  • CT scan: Also known as computerized tomography, computerized axial tomography or CT scan. A diagnostic imaging procedure that uses a combination of X-rays and computer technology to produce cross-sectional images of the body. Shows detailed images of any part of the body, including bones, muscles, fat and organs. CT scans are more detailed than general X-rays.

  • degradation products: Something made to decline to a lower condition, quality or level.

  • diagnosis: The process by which a doctor determines what disease or condition a patient has by studying the patient's symptoms and medical history, and analyzing any tests performed (e.g., blood tets, urine tests, brain scans, etc.).

  • endocrine: Relating to glands that secrete hormones internally directly into the lymph nodes or bloodstream.

  • endocrine glands: Any gland of the body that secretes hormones directly into the lymph nodes or bloodstream.

  • epinephrine: Also known as adrenaline. Hormone secreted in the adrenal gland that raises blood pressure, produces a rapid heartbeat and acts as a neurotransmitter when the body is subjected to stress or danger.

  • gland: A mass of cells or an organ that removes substances from the bloodstream and excretes them or secretes them back into the blood with a specific physiological purpose.

  • glucagons: A hormone responsible for helping maintain proper blood sugar levels.

  • high blood pressure: Medical term is hypertension.

  • histamine: A hormone transmitter involved in local immune response regulating stomach acid production and in allergic reactions.

  • hormone: A natural chemical produced in one part of the body and released into the blood to trigger or regulate particular functions of the body. Antidiuretic hormone tells the kidneys to slow down urine production.

  • hypertension: High blood pressure, which can be caused either by too much fluid in the blood vessels or by the narrowing of blood vessels.

  • hypertensive: Having high blood pressure.

  • incision: Surgical cut for entering the body to perform an operation.

  • invasive: Having or showing a tendency to spread from the point of origin to adjacent tissue, as some cancers do. Involving cutting or puncturing the skin or inserting instruments into the body.

  • invasive: Not just on the surface; with regard to bladder cancer, a tumor that has grown into the bladder wall.

  • ions: Electrically charged atoms.

  • kidney: One of two bean-shaped organs that filter wastes from the blood and discharge these waste products in urine. The kidneys are located on either side at the level of the 12th ribs toward the back. The kidneys send urine to the bladder through tubes called ureters.

  • kidneys: One of two bean-shaped organs that filter wastes from the blood and discharge these waste products in urine. The kidneys are located on either side at the level of the 12th ribs toward the back. The kidneys send urine to the bladder through tubes called ureters.

  • laparoscope: An instrument in the shape of a tube that is inserted through the abdominal wall to give an examining doctor a view of the internal organs.

  • laparotomy: An operation in which the abdominal cavity is opened and inspected directly.

  • medulla: The innermost area of an organ.

  • meta-iodobenzylguanidine: A radioisotope.

  • metastatic: Cancer that has metastasized, in other words, spread to other parts of the body.

  • MRI: Also referred to a magnetic resonance imaging. A diagnostic procedure that uses a combination of large magnets, radiofrequencies and a computer to produce detailed images of organs and structures within the body.

  • pharmacologic: Reaction to drugs.

  • pheochromocytoma: A tumor of the adrenal gland, which is typically benign (non-cancerous).

  • renal: Pertaining to the kidneys.

  • thyroid: An endocrine gland located in the neck that secretes the hormones responsible for controlling metabolism and growth.

  • tumor: An abnormal mass of tissue or growth of cells.

  • tyramine: Normal substance in the body that helps support blood pressure.

  • urge: Strong desire to urinate.

  • urine: Liquid waste product filtered from the blood by the kidneys, stored in the bladder and expelled from the body through the urethra by the act of urinating (voiding). About 96 percent of which is water and the rest waste products.

  • urologist: A doctor who specializes in diseases of the male and female urinary systems and the male reproductive system. Click here to learn more about urologists. (Download the free Acrobat reader.)

  • urology: Branch of medicine concerned with the urinary tract in males and females and with the genital tract and reproductive system of males.

  • vas: Also referred to as vas deferens. The cordlike structure that carries sperm from the testicle to the urethra.

Pheochromocytoma Anatomical Drawings

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