Pheochromocytoma

Diagnosis

To diagnose pheochromocytoma, your doctor may order a series of tests.

Lab tests

These exams check your body’s levels of adrenaline, noradrenaline, or chemicals that are produced as a result of those hormones:

• 24-hour urine test. Every time you urinate over the course of a 24-hour period, you must collect a urine sample for this test. Request written directions on how to label, store, and send back the samples.
• Blood test. Blood will be drawn for laboratory testing.

It is important to consult with your doctor regarding any necessary preparations for both types of tests. For example, you may be advised to fast (abstain from food) or skip a medication dose before the test. However, it is crucial to never miss a prescription dose without your doctor’s permission.

Imaging tests

If the lab test results indicate the presence of a paraganglioma or pheochromocytoma, your doctor will likely request one or more imaging tests to search for a possible tumor. These tests may include:

• Computed tomography (CT) scan, an advanced form of X-ray technology
• Magnetic Resonance Imaging (MRI) combines a magnetic field and radio waves to create detailed images.
• M-iodobenzylguanidine (MIBG) imaging, a scanning technique that can find minute amounts of injected radioactive material absorbed by paragangliomas or pheochromocytomas
• Positron emission tomography (PET), a scanning technique that is also capable of identifying radioactive materials absorbed by a tumor

Imaging tests performed for other purposes may detect a tumor in an adrenal gland. If that occurs, your doctor will typically request additional testing to learn more about the tumor.

Genetic testing

To ascertain whether a pheochromocytoma is connected to an inherited condition, your doctor may advise genetic testing. Information on potential genetic influences may be crucial for a variety of reasons, including:

• Since some genetic abnormalities can result in a number of conditions, test results may indicate the need for additional medical screenings.
• Because some illnesses are more likely to recur or be malignant, your test results may have an impact on the course of your therapy or the long-term plans you make to monitor your health.
• Test results may indicate that other family members should undergo screenings for pheochromocytoma or disorders that are similar.

You can better comprehend the outcomes of your genetic testing with the aid of genetic counseling.

Treatment

The primary treatment for pheochromocytoma is surgery to remove the tumor. Prior to the procedure, your doctor may recommend certain blood pressure medications. These medications suppress the high-adrenaline hormones and help reduce the risk of dangerously high blood pressure during surgery.

Preparations before surgery

Before surgery, you’ll probably need to take drugs for 7 to 10 days to decrease your blood pressure. Other blood pressure medications you now use will either be replaced by these medications or added. A high-salt diet may also be suggested for you.

Smaller veins and arteries are kept open and relaxed by medications including alpha blockers, beta blockers, and calcium channel blockers. Blood flow is enhanced, and blood pressure is reduced. Your heart may beat more slowly and with less force as a result of certain of these drugs. The blood pressure may drop even lower as a result.

These drugs reduce blood vessel fluid because they enlarge the blood vessels, which results in reduced blood vessel fluid. When standing, this might result in potentially fatal reductions in blood pressure. In order to prevent low blood pressure during and after surgery, a high-salt diet will draw more fluid into the blood vessels.

Surgery

Laparoscopic surgery is typically used to execute the procedure to remove an adrenal tumor. To complete the procedure, your surgeon will make a few minor cuts (incisions) in your abdomen. These wounds will be used to introduce wand-like instruments that are fitted with cameras and small tools.

With a pheochromocytoma, your surgeon often removes the entire adrenal gland. Your surgeon, however, might merely remove the tumor and leave some healthy adrenal gland tissue. When the other adrenal gland has also been removed or when both adrenal glands have tumors, this may be done.

The tumor and any surrounding malignant tissue will be removed if a tumor is cancerous. Even if all of the malignant tissue is not removed, medical treatment and surgery may lessen hormone production and help to regulate blood pressure.

After surgery

The remaining healthy adrenal gland is capable of performing the tasks typically handled by two glands. Normal blood pressure levels usually recover. For the rest of your life, you’ll require routine checkups with your doctor to monitor your health, spot issues, or determine whether the tumor has returned.

Cancer treatments

Malignant pheochromocytomas that are cancerous are rare, and as a result, the most effective treatments have received limited attention in research. However, treatment options for malignant tumors related to pheochromocytoma and cancer that has spread throughout the body may include:

• Targeted therapies combining a radioactive substance that kills cancer cells with a medicine or substance that targets cancer cells for destruction. There may potentially be drug therapies that target certain aberrations in cancer cells that enable their survival.
• Chemotherapy employing strong medications that kill cancer cells that develop quickly.
• Radiation therapy may ease the pain-causing symptoms of malignancies that have progressed to the bone.