Rare, but Not Alone

Pheo and para may be rare, but patients are not on their own. Specialized hospitals have teams of experienced doctors with extensive experience treating pheo and para. Getting treatment at a specialized hospital is the best option for patients with pheo or para.

What are Pheo and Para?

Pheochromocytoma and paraganglioma (pheo and para) are tumors that come from specific cells inside the body.

Pheo and para originate from neuroendocrine cells.
Neuroendocrine cells communicate with muscles and organs by producing different types of hormones. For example, if your body needs your heart to beat faster, your brain can send a signal to neuroendocrine cells telling them to produce a hormone called epinephrine (also called adrenaline). The epinephrine would signal to your heart that it should beat faster.

Pheo form in the adrenal glands. Para form in cells outside the adrenal glands.

Pheo and para form throughout the body.
Pheo form in the adrenal glands, just above the kidneys. The adrenal gland produces the hormones epinephrine and norepinephrine. Para form from cells outside the adrenal glands, which also produce epinephrine and norepinephrine.

Pheo and para produce “fight or flight” hormones.
Because of where they form, pheo and para tumors are able to produce epinephrine and norepinephrine on their own, and they can also take up these hormones.

All pheo and para tumors have the potential to be cancerous.

Most pheo and para can be removed with surgery unless they are metastatic (spread to another part of the body). In other types of cancer, you can sometimes tell if a tumor may be cancerous, even if it has not spread. However, with pheo and para, the only way to know if a tumor is cancerous is if it spreads.

As many as 1 in 3 pheo and para cases are metastatic malignant.
16.4% of apparently benign pheo and para cases come back after initial surgery.

About 1 in 6 patients have a tumor that comes back after surgery.
Tumors that come back also have the potential to be metastatic. If you have pheo or para and had surgery to remove it, be sure to continue to check in with your doctor to monitor for any returning tumors.

Pheo and para can affect both men and women, and people of all ages.

Most people are in their 40s or 50s when they are diagnosed, but the disease can also affect children and the elderly.

How Rare are Pheo and Para?

Between 660–2,630 patients are diagnosed with pheo and para each year in the US. In total, as many as 130,000 people are living with pheo or para in the US. Information and support may seem difficult to find, but there is a strong community for patients and their families. Connect with other patients who have pheo and para.
Out of 1,000,000 people, 5,180 are diagnosed with cancer, 673 are diagnosed with rare cancer, and fewer than 8 are diagnosed with pheo or para each year.

Pheo are more common than para, but they are both rare. Fewer than 8 out of every 1,000,000 people are diagnosed with pheo or para in the US each year.

Seek Treatment at a Specialized Hospital

Because pheo and para are so rare, many doctors may have limited experience with these diseases. However, there are some doctors who specialize in treating this type of tumor.
Treatment from an experienced team at a Center of Excellence is critical.

Pheo and para should be managed by a team of doctors.
Treating pheo and para can be challenging, and many different treatments are considered so different types of medical experts are involved, including:

  • Oncologists, who specialize in treating cancer
  • Endocrinologists, who specialize in treating hormone-related diseases
  • Surgeons, who specialize in removing tumors with surgery
  • Genetic counselors, who help families identify and understand heritable genetic mutations that can cause disease
  • Radiologists, who use methods like CT or MRI to look for cancer in the body
  • Nuclear medicine physicians, nuclear radiologists, and radiation oncologists, who use a specialized molecule called MIBG (meta-iodobenzylguanidine) to look for and/or treat pheo and para

Certain hospitals have teams of doctors who deal specifically with pheo and para. This type of hospital may be known as a Center of Excellence, or COE, which means it has teams of different types of medical experts working together to treat patients. For those with metastatic pheo or para tumors, prompt treatment is essential because only 20-60% of people will survive 5 years after their diagnosis.