Pheochromocytoma

Diagnosis

Your doctor might order a number of tests.

Laboratory tests

Your doctor will likely order the following tests that measure levels of adrenaline, noradrenaline or byproducts of those hormones in your body:

• 24-hour urine test. You will be asked to collect a urine sample every time you urinate during a 24-hour period. Ask for written instructions about how to store, label and return the samples.
• Blood test. You will have blood drawn for laboratory work. Talk to your doctor about special preparations, such as fasting or skipping a medication. Don't skip a dose of medication without instructions from your doctor.

Imaging tests

If the results of laboratory tests indicate the possibility of a pheochromocytoma or paraganglioma, your doctor will order one or more imaging tests to locate a possible tumor. These tests may include:

• CT scan, a specialized X-ray technology
• MRI, which uses radio waves and a magnetic field to produce detailed images
• M-iodobenzylguanidine (MIBG) imaging, a scanning technology that can detect tiny amounts of an injected radioactive compound taken up by certain tumors
• Positron emission tomography (PET), a scanning technology that can also detect radioactive compounds taken up by a tumor

Incidental discovery

A tumor in an adrenal gland might be found during imaging studies conducted for other reasons. In such cases, your doctor would order additional tests to determine the nature of the tumor.

Genetic testing

Your doctor might recommend genetic tests to determine if a pheochromocytoma is related to an inherited disorder. Information about possible genetic factors can be important for a number of reasons:

• Because some inherited disorders can cause multiple conditions, test results may indicate the need to screen for other medical problems.
• Because some disorders are more likely to be recurrent or cancerous (malignant), your test results may affect treatment decisions or long-term plans to monitor your health.
• Results from your tests may indicate that other family members should be screened for pheochromocytoma or related conditions.

Ask your doctor about genetic counseling services that can help you understand the benefits and implications of genetic testing.

Treatment

The primary treatment for a pheochromocytoma is surgery to remove the tumor. Before you undergo surgery, your doctor will prescribe specific blood pressure medications that block the actions of the high-adrenaline hormones to lower the risk of developing dangerously high blood pressure during surgery.

Pre-operative medications

You will likely take two drugs for seven to 10 days that help lower blood pressure before surgery. These drugs will either replace or be added to other blood pressure drugs you take.

• Alpha blockers keep smaller arteries and veins open and relaxed, improving blood flow and decreasing blood pressure. Alpha blockers include phenoxybenzamine (Dibenzyline), doxazosin (Cardura), and prazosin (Minipress). Side effects might include irregular heartbeat, dizziness, fatigue, vision problems, sexual dysfunction in men and swelling in your limbs.

• Beta blockers cause your heart to beat more slowly and with less force. They also help keep blood vessels open and relaxed. In preparing for surgery a beta blocker is added several days after starting the alpha blocker.

  Beta blockers include atenolol (Tenormin), metoprolol (Lopressor, Toprol-XL) and propranolol (Inderal, Innopran XL). Possible side effects include fatigue, upset stomach, headache, dizziness, constipation, diarrhea, irregular heartbeat, difficulty breathing and swelling in the limbs.

• High-salt diet Alpha and beta blockers widen (dilate) the blood vessels, causing the amount of fluid within the blood vessels to be low. This can cause dangerous drops in blood pressure with standing. A high-salt diet will draw more fluid inside the blood vessels, preventing the development of low blood pressure during and after surgery.

Surgery

In most cases, the entire adrenal gland with a pheochromocytoma is removed with laparoscopic, or minimally invasive, surgery. Your surgeon will make a few small openings through which he or she inserts wandlike devices equipped with video cameras and small tools.

The remaining healthy adrenal gland carries out the functions normally performed by two, and blood pressure usually returns to normal.

In some unusual situations, such as when the other adrenal gland has been removed, surgery might remove only the tumor, sparing some healthy tissue.

If a tumor is cancerous (malignant), the effectiveness of surgery might rely on removing the tumor and all cancerous tissue. However, even if all of the cancerous tissue isn't removed, surgery might limit hormone production and provide some control of blood pressure.

Cancer treatments

As cancer is rare among cases of pheochromocytoma, the research about the best treatments is relatively limited. Treatments for malignant tumors and metastasized cancer related to a pheochromocytoma might include:

• Radionuclide treatment. This radiation therapy combines MIBG, a compound that attaches to adrenal tumors, with a type of radioactive iodine. The treatment goal is to deliver radiation therapy to a specific site and kill cancerous cells.
• Chemotherapy. Chemotherapy is the use of powerful drugs that kill fast-growing cancer cells.
• Targeted cancer therapies. These medications inhibit the function of naturally occurring molecules that promote the growth and spread of cancerous cells.

Preparing for your appointment

You're likely to start by seeing your primary care provider. Then you might be referred to a doctor who specializes in hormonal disorders (endocrinologist).

Here's some information to help you get ready for your appointment.

What you can do

When you make the appointment, ask if there's anything you need to do in advance, such as fasting before having a specific test. Make a list of:

• Your symptoms, including any changes from normal, when they began and how long they last
• Key personal information, including major stresses, recent life changes and family medical history
• All medications, vitamins and other supplements you take, including doses
• Questions to ask your doctor

Take a family member or friend along, if possible, to help you remember the information you get.

For a pheochromocytoma, questions to ask your doctor include:

• What's likely causing my symptoms?
• Other than the most likely cause, what are other possible causes for my symptoms?
• What tests do I need?
• Is my condition likely temporary or chronic?
• What's the best course of action?
• What are the alternatives to the primary approach you're suggesting?
• I have these other health conditions. How can I best manage them together?
• Are there restrictions I need to follow?
• Should I see a specialist?
• Are there brochures or other printed material I can have? What websites do you recommend?

Don't hesitate to ask other questions.

What to expect from your doctor

Your doctor is likely to ask you questions, including:

• Have your symptoms been continuous or intermittent?
• Does anything seem to improve your symptoms?
• What, if anything, appears to bring on or worsen your symptoms?
• Have you been diagnosed with other medical conditions? If so, what treatment are you getting?