A Disease Unlike Any Other

Pheo and para are unique because these tumors produce hormones that can cause over 100 different signs and symptoms. Controlling tumors and reducing these symptoms are the two goals of pheo and para treatment.

What Makes Pheo and Para Different?

Pheo and para cells produce the hormones epinephrine (also called adrenaline) and norepinephrine (also called noradrenaline).
Pheo and para tumor cells are unique because of the hormones they produce: epinephrine and norepinephrine.

Epinephrine and norepinephrine play an important role in the fight or flight response. Normally, these hormones get triggered during times of stress and prepare the body for increased activity. One of the main ways they do this is by boosting how quickly and how powerfully the heart beats.

Pheo and para tumors can produce a large amount of these hormones when they are not needed. This excess production of hormones is what causes the many symptoms of pheo and para.

What are the Signs & Symptoms of Pheo and Para?

Over 100 different signs and symptoms are associated with pheo and para.

  • Common signs and symptoms include:
    1. High blood pressure
    2. Strong, fast, or irregular heartbeat
    3. Headaches
    4. Heavy sweating
    5. Pain in the chest or abdomen
    6. Uncontrollable shaking
    7. Anxiety and panic attacks
    8. Fatigue and weakness
    9. Nausea, vomiting, or weight loss
    10. Paleness
  • Symptoms can come and go without warning, and not every patient has every symptom. When they do happen, symptoms can be sudden, intense, and even life-threatening. If pheo and para tumors are not diagnosed and appropriately treated, the disease will likely be fatal.

Tumor-produced hormones cause over 100 different signs and symptoms.

How are Pheo and Para Diagnosed?

Because symptoms come and go and can mimic other, more common diseases, diagnosing pheo and para is not easy.

On average, it takes 3 years from the time symptoms start before a correct diagnosis is reached. Some pheo and para cases never get diagnosed.

Over 50% of pheo and para found at autopsy were unsuspected.

 

It’s important for people with pheo and para to be their own best advocates

Did you know?

In fact, President Dwight D. Eisenhower died in 1969 from the severe cardiovascular symptoms he suffered for much of his adult life. Upon autopsy, his physicians found a pheochromocytoma in his adrenal gland that was undetected in his lifetime. Now, 50 years later, reaching a timely and accurate diagnosis remains a serious challenge in the pheo and para community.

25% of pheo and para cases are discovered while looking for an unrelated disorder.

One way to test for pheo and para is to check for extra epinephrine or norepinephrine in the bloodstream. A blood test or a urine test can measure this.

Another way to find pheo and para is with imaging, like CT or MRI. Over half of cases are identified from imaging performed when a doctor is looking for a different disease.

What is MIBG?

MIBG is a molecule that can be used to find pheo and para.

MIBG enters pheo and para cells using the same transporter recognized by norepinephrine. In other words, the tumor cells absorb the MIBG like a hormone. MIBG can be made with a single radioactive atom that acts like a beacon for doctors to track (in fact, doctors sometimes refer to MIBG as a “tracer”). When enough MIBG builds up in a tumor, that tumor will show up on an MIBG imaging test. Once the tumor is identified, doctors can decide how to treat it.

MIBG is a molecule that can be used to find pheo and para.

How are Pheo and Para Treated?

The two goals of treatment are controlling tumors and reducing symptoms.
Goals of pheo and para treatment are tumor control and symptom reduction.

Some tumors can be removed using surgery. Even if the pheo or para tumor is cancerous and has metastasized, your doctor may recommend surgery for tumors that can be removed. If a tumor is too close to other organs, it may be too dangerous to remove. Any surgery to remove a pheo or para tumor can trigger a release of hormones, so doctors may give drugs to stabilize heart rate and blood pressure ahead of time.

For treating cancerous pheo or para tumors, there are two classes of radiation-based therapy that are frequently used. External beam radiation therapy is the most common type of radiation therapy used to treat cancer. For this therapy, a machine is used to aim high-energy rays from outside the body into the tumor. In contrast, systemic radiation therapy uses targeted molecules injected into the blood to find the tumor inside the body and treat it from within.

The most commonly used systemic radiation therapy is called I-131 MIBG therapy (also known as iobenguane I 131 or MIBG therapy), which uses the targeting ability of MIBG to find tumors and treat them with powerful radioactivity. There are two different types of MIBG therapy, referred to as high and low specific activity I-131 MIBG, that differ by how many of the MIBG molecules are radioactive. MIBG therapy is an option if the pheo or para tumor (and any metastases) are positive on an MIBG imaging test.

Other treatments that may be considered, although they are not approved by the Food and Drug Administration (FDA), include chemotherapy, peptide receptor radiotherapy (PRRT), or somatostatin analog therapy.

None of these treatments are a cure, but they may help control tumor growth and reduce symptoms.

Doctors may also prescribe drugs to help manage the symptoms of pheo and para. These drugs work to counteract the extra hormones produced by tumors, and thus lower blood pressure, and lower heart rate.

What Happens After Treatment?

Long-term follow-up is essential for people with pheo or para.

Even if you’ve only had surgery to remove a solitary tumor, you should continue to visit your doctor regularly for testing. These routine visits can help detect early signs of the tumor returning. If you’ve had genetic testing that identified a genetic mutation, long-term management is especially important because your risk for additional tumors growing over your life may be high.

Start a conversation with your doctor about this disease and its treatment options.