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Pheochromocytoma Causes, Symptoms, and Treatments

Pheochromocytoma is a rare tumor that forms in the adrenal glands. It's often not cancerous but can lead to other severe health problems.

The tumor causes the adrenal glands to overproduce hormones that control heart rate and blood pressure.

Pheochromocytoma treatment may include surgery and medicine.

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What Is Pheochromocytoma (PCC)?

Doctors define PCC as a tumor that grows in an adrenal gland.

The adrenal glands are two small organs, one on top of each kidney.

They make the hormones epinephrine (adrenaline) and norepinephrine that control:

  • Blood pressure.
  • Heart rate.
  • Metabolism.

Pheochromocytoma symptoms include:

  • Headaches.
  • High blood pressure.
  • Racing, fluttering, or pounding heartbeats.

PCCs are rare. About less than eight people in a million in the U.S. have them.

Doctors don't know exactly how many people have PCCs because many are never diagnosed.

PCCs often occur in people who are between 30 and 50. Most are benign, meaning they don't spread to other body parts.

But about 10% of pheochromocytomas are cancerous and can spread to other body parts.

Doctors usually treat PCC with surgery. If surgery isn't possible, they treat the tumors with medicine.

What are the types of pheochromocytoma?

Most PCCs (80 to 85%) grow in the inner layer of the adrenal gland. This part of the gland is the adrenal medulla.

A smaller number (15 to 20%) of PCCs grow outside the adrenal medulla. Doctors call them extra-adrenal PCCs or paragangliomas.

Both PCCs and paragangliomas can be cancerous or non-cancerous. Most are non-cancerous.

What causes pheochromocytoma?

Doctors don't know what causes PCC.

In some cases, it may run in families.

It occurs equally in men and women.

What are pheochromocytoma risks and complications?

Scientists are still learning about PCC causes, risk factors, and complications. But there isn't evidence that environmental and lifestyle factors affect the disease.

Pheochromocytoma risk factors

Some genetic conditions may raise your risk of getting PCC.

These include:

  • Carney triad.
  • Carney-Stratakis dyad.
  • Hereditary paraganglioma syndrome.
  • Multiple endocrine neoplasia 2 syndrome, types A and B.
  • Neurofibromatosis type 1.
  • Von Hippel-Lindau syndrome.

Complications of pheochromocytoma

Having PCC can lead to serious health problems.

Most PCCs aren't cancerous. But the tumor can release hormones that cause extremely high blood pressure.

Untreated, PCC and the resulting high blood pressure can lead to:

  • Heart attack.
  • Stroke.
  • Death.

If the tumor is malignant, the cancer can spread to other parts of your body.

How can I prevent pheochromocytoma?

There are no known ways to prevent PCC.

But if you have the tumor, you should have genetic counseling. This can help you find out your risk of getting other cancers.

Some genetic conditions that cause PCC can also raise the risk for thyroid and parathyroid cancer.

Your doctors may suggest genetic testing for close family members. They may be at risk for PCC or other tumors.

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Pheochromocytoma Symptoms and Diagnosis

What are the signs and symptoms of PCC?

People with PCC may get sudden symptoms or an "attack."

These sudden attacks may:

  • Happen at seemingly random times.
  • Occur a couple of times a week or only a few times a year.
  • Last from a few minutes to several hours.
  • Get longer, more severe, and closer together as the tumor grows.

Pheochromocytoma symptoms include:

  • Bad headaches.
  • Chest pain.
  • Excessive anxiety, worry, or nervousness.
  • Excessive sweating.
  • Fatigue.
  • Feeling overheated.
  • Heart palpitations or racing heartbeat.
  • High blood pressure.
  • Insomnia.
  • Nausea and vomiting.
  • Pallor or paleness.
  • Panic attacks.
  • Seizures.
  • Shortness of breath.
  • Stomach pain.
  • Weight loss.

What triggers a PCC attack?

Triggers that can bring on PCC symptoms include:

  • Abdominal pressure.
  • Anesthesia for surgery.
  • Bending over.
  • Childbirth.
  • Eating certain foods high in tyramine (red wine, chocolate, and cheese).
  • Emotional stress.
  • Fear.
  • Hard physical activity.
  • Injury.
  • Smoking.

How do you diagnose pheochromocytoma?

PCC symptoms can mimic some stress-related disorders.

To arrive at a pheochromocytoma diagnosis, doctors will:

  • Do a physical exam.
  • Ask about your medical history.
  • Run blood and urine tests. These lab tests will help the doctor check your hormone levels.
  • Order imaging tests.

Imaging tests will help the pheochromocytoma doctor (often an endocrinologist) see where and how big the tumor is.

These tests may include CT, MRI, or PET scans of the belly.

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How Do You Treat Pheochromocytoma?

A pheochromocytoma specialist is the best doctor to diagnose and treat this rare tumor.

UPMC doctors can access the latest equipment and research. They're experts at treating rare endocrine diseases like PCC.

High-tech imaging studies and lab testing can help find the exact size and location of the tumor.

Treatment for pheochromocytoma

PCC treatment is a step-by-step process.

Step 1: blood pressure medicine

The first step starts with controlling your blood pressure.

This may include drugs to:

  • Block the effect of extra hormones produced by the tumor.
  • Keep your blood pressure normal.
  • Keep your heart rate normal.

Step 2: surgery to treat PCC

At UPMC, we use the latest surgical techniques to remove as much of the tumor as possible.

Sometimes, doctors will remove the entire adrenal gland. If so, you'll need life-long hormone therapy to replace the adrenal glands' hormones.

If your PCC is cancerous, you may need radiation or chemo.

Pheochromocytoma prognosis

If your adrenal glands are still intact, your hormone levels should return to normal after pheochromocytoma treatment.

Even after PCC treatment, your blood pressure may not return to normal. In that case, you may need to keep taking medicine.

After treatment, you'll have testing from time to time to make sure the tumor hasn't come back. Your doctor can tell you how often you'll need testing.

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