Pheochromocytoma – Diagnosis and Treatment

Diagnosis

Tests that Measure Hormone Levels

Doctors usually start by checking hormone levels in people who may have symptoms like high blood pressure, severe headaches, tremors, or rapid heartbeat.

These symptoms may be linked to a pheochromocytoma, a rare tumor of the adrenal medulla or sometimes in paragangliomas.

Most often, professionals look for changes in catecholamines such as epinephrine and norepinephrine, as well as their byproducts, metanephrines.

The main tests are:

• 24-Hour Urine Collection: The patient saves all urine for a full day. The lab analyzes this sample for substances like free metanephrine, normetanephrine, and catecholamines.
• Blood Draw for Plasma Tests: Doctors check blood samples to see if hormone levels are higher than usual.

Factors like certain foods, stress, or medications can affect results. Patients should follow all instructions, such as fasting or adjusting medicine, only as told by their healthcare provider.

Test Type	What It Measures	Why It’s Used
Urine test	Metanephrines, catecholamines	Higher levels are common in tumors.
Blood test	Plasma free metanephrines	An efficient way to identify elevated hormone levels.

Both urine and blood results guide decisions about further checks.

Scans and Studies for Tumor Location

When doctors find higher hormone levels, they use imaging tests to locate the tumor. These tests are crucial for both pheochromocytoma and paraganglioma because tumors sometimes develop outside the adrenal glands.

Common imaging tests include:

• Computed Tomography (CT) Scanning: This imaging method uses X-rays to show detailed pictures. CT helps doctors find tumors in the adrenal glands or nearby tissues.
• Magnetic Resonance Imaging (MRI): MRI scans provide clear views of soft tissues without X-rays. MRI is helpful for seeing tumors that might not show up well on CT.
• M-iodobenzylguanidine (MIBG) Scan: Doctors inject a small amount of radioactive material. Tumor cells, like those in pheochromocytoma, absorb the compound and show up on images.
• Positron Emission Tomography (PET) Scanning: Doctors use a radioactive substance to highlight abnormal cell activity.

Sometimes, these scans reveal a tumor by accident while looking for something else. If so, doctors test hormone levels next. Imaging studies help check for spread and plan for removal or other treatment.

Looking for Genetic Changes

Doctors often recommend genetic tests for patients with pheochromocytoma or paraganglioma, especially if the patient is young, has a family history, or tumors on both sides.

These tests can find gene changes (mutations) linked to syndromes like multiple endocrine neoplasia type 2, neurofibromatosis type 1, or von Hippel-Lindau syndrome.

Knowing about genetic mutations helps in several ways:

• Doctors can check for other neuroendocrine tumors or related health problems.
• Some gene changes mean the tumors might come back, become cancer, or run in families.
• Results can show if other relatives need tests or regular checks.

Doctors often suggest meeting with a genetic counselor. This expert explains results and what they mean for the patient and family’s health.

Treatment

Getting Ready Before the Operation

Doctors often prescribe medicine to control blood pressure before removing a pheochromocytoma.

This step helps lower the risk of sudden and dangerous blood pressure spikes during surgery. Medicines may include alpha blockers, beta blockers, and calcium channel blockers.

These medicines relax and widen blood vessels to allow for smoother blood flow. Doctors usually start alpha-blockers first.

They often add beta blockers after alpha blockers to slow the heart rate if needed. Sometimes, calcium channel blockers help control blood pressure more effectively.

Doctors may recommend a high-sodium diet before surgery. This increases fluid in the blood vessels and keeps blood pressure from dropping too low once the vessels are widened by medication. The care team monitors and adjusts doses as needed.

Summary Table of Pre-Surgery Steps:

Step	Purpose	Example Medicines
Alpha blocker	Relaxes blood vessels, lowers BP	Phenoxybenzamine, doxazosin
Beta blocker	Slows heart rate, lowers BP	Atenolol, metoprolol
Calcium channel blocker	Further controls blood pressure	Amlodipine
High-sodium diet	Expands blood volume	Salty foods as advised

Doctors and nurses check blood pressure several times a day and may perform blood tests to keep patients safe before surgery.

Tumor Removal Surgery

Surgeons treat pheochromocytoma by removing the tumor. They often use minimally invasive tools for this.

Laparoscopic surgery involves small cuts in the belly area. Through these cuts, surgeons use special tools and cameras to remove the tumor.

Some surgeons use robotic technology, where robotic arms assist with the operation while the surgeon controls them nearby.

In most cases, the surgeon removes the adrenal gland containing the tumor. If needed, the surgeon might only remove the tumor itself and leave healthy adrenal tissue.

This approach can help if both adrenal glands are affected or if a person cannot lose both glands.

For people with metastatic disease—meaning the cancer has spread—surgeons may not be able to remove everything.

In these cases, they take out as much of the tumor as possible and use medical therapy to help ease symptoms and make blood pressure easier to manage.

Types of Surgery Used:

• Laparoscopic procedure (small cuts, fast recovery)
• Robotic-assisted surgery (precise, less invasive)
• Open surgery (larger incision, usually for bigger tumors)

Care Following Surgery

When one healthy adrenal gland remains after tumor removal, it usually can do the job for both. For most patients, blood pressure returns to a safer range quickly.

The care team schedules regular checkups to track blood pressure, hormones, and overall health. Blood tests help catch early signs if the tumor returns.

If surgeons remove both adrenal glands during surgery, patients need lifelong steroid medicine. These medicines replace the main hormones made in the adrenal glands, helping the body function normally.

Post-surgery care checklist:

• Scheduled visits with the healthcare team
• Blood test monitoring
• Checking for symptom changes
• Taking medications as prescribed

Advanced Cancer and Other Treatments

In rare situations, pheochromocytomas spread to other organs. These cases are called metastatic pheochromocytoma. Doctors choose treatments based on the patient’s needs and the places cancer has moved.

Common options include:

• Targeted Therapy: Doctors use medicine linked to radioactive substances to attack cancer cells while sparing healthy tissue.
• Chemotherapy: Doctors use drugs to kill fast-growing cancer cells if the disease spreads or cannot be fully removed with surgery.
• Radiation Therapy: Doctors direct strong beams of energy at tumors to help with pain or to shrink tumors, especially those that reach the bones.
• Ablation: Doctors use extreme cold, heat, or alcohol injections to destroy cancer tissue.

Doctors create a treatment plan for each patient. It may involve one or a combination of these therapies. The choice depends on tumor type, location, and overall health.

Getting Ready for Your Visit

Steps You Can Take

Being prepared for a medical appointment helps make the most of time with the care team. Bringing a friend or family member can help with remembering details and sharing important information.

Things to do before your visit:

• Ask about special instructions. Some appointments might need fasting or adjusting medications. Contact the clinic to find out if these steps apply.
• List your symptoms. Note any new, changed, or long-lasting signs you have noticed. Write down when they started and how often they occur.
• Personal history. Record big changes in your life, main sources of stress, and family medical history—especially if anyone has had similar health problems.
• Medication list. Make a table or list with all medicines, vitamins, and supplements you take. Include the name and dose for each.

Name of Medicine/Supplement	Dose	How Often Taken
Example: Lisinopril	10mg	daily

Question ideas for your care provider:

• What might be causing my symptoms?
• Are there other possible reasons for these changes?
• What tests should I expect?
• Is this condition likely to last, or will it go away?
• What treatment plans are available?
• Are there other treatment choices?
• How should I handle other health issues I have?
• What should I not do while being tested or treated?
• Should I see an endocrinologist or another specialist?

You can ask anything else that concerns you during the visit.

Questions Your Doctor May Ask

Doctors need clear details to give the best care. Expect questions, such as:

• Are your symptoms always present, or do they come and go?
• What makes your symptoms better or worse?
• Are there clear triggers for your symptoms?
• Do you have other health problems? If so, what treatment are you on?
• Is there any family history of adrenal or other gland tumors?