Pheochromocytoma

Medical quality assurance by Dr. Albrecht Nonnenmacher, MD at April 28, 2016
StartDiseasesPheochromocytoma

A pheochromocytoma is a typically benign tumor that grows in the adrenal glands. There are two adrenal glands in the human body, each associated with one of the kidneys. These growths can occur at any age, but they most commonly affect adults between 20 and 50 years of age.

Contents

Definition & Facts

Pheochromocytomas are generally noncancerous, but in some rare cases can be malignant. In either case, the growth occurs in the center of the adrenal gland, which affects the gland and the way it functions within the body. The adrenal glands are responsible for the production of many hormones that instruct tissues and organs how to behave.

The presence of the tumor causes those hormones to be released incorrectly, which can lead to many dangerous symptoms, especially those related to cardiovascular health. In many cases, surgery is one of the only options for treating a pheochromocytoma; such procedures have a high success rate.

Symptoms & Complaints

Many of the symptoms associated with this type of condition are symptoms of other conditions, so further testing must be done once a doctor comes to suspect a pheochromocytoma. The most common symptoms brought on by these adrenal tumors are:

While the presence of any one of these symptoms doesn't necessarily point to a pheochromocytoma, several of these presenting at once might indicate a serious problem. For more extreme cases, or instances where the tumors have gone untreated for a long period of time, other symptoms might present as well, including:

These symptoms aren't as common and tend to occur in isolated spells, perhaps once or twice per day.

Causes

It is not known what causes these pheochromocytomas to develop. However, it is known that these tumors only grow within specialized cells located in the center of the adrenal gland. These cells are called chromaffin cells, and they are responsible for the release of some of the most important hormones in the human body: epinephrine and norepinephrine.

The primary function of these hormones is to induce a fight-or-flight response during a potential threat. These hormones enable the body systems to quickly react, acting on the body to increase heart rate, blood pressure, and metabolic rate. The presence of a tumor in the adrenal gland causes these hormones to be released in excessive amounts, which leads to a permanent state of agitation. Certain situations or substances can trigger symptoms; these include:

Diagnosis & Tests

There are a number of ways to search for the presence of a pheochromocytoma. Doctors typically order a blood test or urine test to look for the presence of adrenaline and noradrenaline in the bloodstream. Such tests might require fasting or skipping other medications to avoid tainting the test.

Once the initial tests have proven the possibility of a tumor, the doctor might order one of several imaging tests to actively search for a tumor within the adrenal glands. These tests might include a CT scan, an MRI, which uses magnetic resonance to produce highly detailed images, or even a PET scan which detects small traces of radioactive elements taken into the tumor.

It is surprisingly common for these tumors to be discovered during other procedures, especially imaging tests that are being conducted for unrelated purposes. This is due to the fact that many of the symptoms are often ignored, especially if other, more serious symptoms are plaguing a patient. If a doctor were to find a tumor during an unrelated scan, the next step would be to perform additional tests to ascertain the nature of the growth.

Once a pheochromocytoma is confirmed, the doctor might want to perform genetic testing to search for any other conditions that might arise in relation to the tumor. This is important because the presence of one of these tumors could indicate an underlying genetic issue that might lead to further medical problems in the future. Further knowledge regarding the source of the condition can indicate whether family members should be screened for possible occurrences as well.

Treatment & Therapy

The most effective treatment for a pheochromocytoma is to remove it with surgery. In some cases, a doctor might prescribe certain medicines to relieve some of the cardiovascular symptoms. Alpha blockers and beta blockers are the most common pre-surgery medications, but other drugs might be needed if blood pressure doesn't respond to the blockers.

Laparoscopic surgeries are usually employed to remove either the entire affected gland, or simply a small portion with the tumor. If the other gland is still in healthy condition, most doctors will remove the entire affected gland, and the remaining gland handles the load of both. However, if the second gland has already been removed, doctors will attempt to save some tissue from the affected gland.

Other treatments are available for the rare instances of a malignant tumor within the adrenal gland. Surgery is still used to remove the dangerous tissue, and chemotherapy or other targeted therapies are used to prevent recurrences.

Prevention & Prophylaxis

Since it isn't known what causes these tumors to form, there are no suggested ways to prevent them from forming. Once the tumor forms, symptom flare-ups can be avoided by treading carefully around the triggers that set off spells. Once treated, there is a slight risk of recurrence, but properly maintaining a generally healthy lifestyle will help prevent relapses.