faq’s

pheochromocytoma & paraganglioma

What is pheochromocytoma (pheo)?

What is pheochromocytoma (pheo)?

Pheochromocytoma is a neuroendocrine tumor. The tumor originates from chromaffin cells in the medulla (inside portion) of the adrenal gland. Pheo can secrete excessive amounts of catecholamines (hormones that make up adrenaline). Pathologists that suspect a tumor of chromaffin cells stain the tissue with chromic salts. If the tumor is pheo positive, the tissue will turn a dark dusky color. Hence the name, Pheochrome- (staining dark and dusky with chromic salts) -cytoma (a general term for a cellular tumor).

What is paraganglioma (para)?

What is paraganglioma (para)?

Paragangliomas are closely related to pheochromocytoma. However, paras originate from the ganglia (group of nerve cells) of the nervous system, allowing them to form anywhere along the sympathetic and parasympathetic nervous systems. There are two types of paragangliomas – chromaffin paragangliomas and nonchromaffin paragangliomas.

Chromaffin paragangliomas arise from chromaffin cells, and are sometimes called extra-adrenal pheochromocytoma, which means the tumor is found outside the adrenal gland. These tumors usually secrete hormones similar pheo. Chromaffin paras are rarer than pheo, but have a higher chance of malignancy (cancerous).

Nonchromaffin paragangliomas are usually named based on the primary anatomical site of origin. For example, a Carotid Body Tumor (CBT) is another name for a neck paraganglioma. Nonchromaffin paras arise from glomus cells instead of chromaffin cells. They are commonly noncancerous, and generally present in the head and neck areas. Nonchromaffin paras rarely secrete hormones, and can occur within familial lines.

Is it cancer?

Is it cancer?

Pheo and Para can be non-metastatic and metastatic. Non-metastatic means that the tumor(s) have not spread to other parts of the body.  Metastatic means that the tumor has spread to other parts of the body.  All pheos and paras have the potential to become metastatic.  It is important to have routine monitoring to catch tumors early before they become metastatic.

Which of the following are symptoms of pheochromocytoma and paraganglioma?

Which of the following are symptoms of pheochromocytoma and paraganglioma?

Functioning pheo and para (meaning pheos and paras that secrete the excessive catecholamines) can produce multiple symptoms. Some patients experience all symptoms on the list, while others may only experience one or two. Those symptoms include (but are not limited to):

  • High blood pressure
  • Rapid heart rate / forceful heartbeat
  • Profound Sweating
  • Flushing
  • Abdominal pain
  • Sudden-onset of headaches (usually severe)
  • Anxiety
  • Emotional mood swings
  • Joint pains
  • Pale Skin
  • Weight loss

Due to the fact that many symptoms are common in many other ailments, pheo has been nicknamed, “The Great Mimic,” and while many pheo patients experience high blood pressure, pheo only counts for about 1% of high blood pressure cases. That means most people with high blood pressure will not have pheo.

Non-Functioning pheos and paras may not yield the same symptoms as functioning pheos and paras. They often go undetected, are found incidentally, or start causing symptoms like pain or swelling of surrounding organs when tumors grow too large. For instance, Carotid Body Tumors (neck paras) are often found by seeing or feeling a lump in the neck before other symptoms like pain or difficulty swallowing occurs.

What are the dangers to pheo and para?

What are the dangers to pheo and para?

Functioning pheo and para create excessive amounts of catecholamines in a body, elevating the risk of heart attack, stroke, and even kidney failure. For patients with functioning pheos and para, there is also a danger to outpatient procedures that involve anesthesia; this includes dental procedures that involve certain numbing agents. A reaction to the anesthetics causes the body to release a large amount of catecholamines that can send the patient into a catecholamine crisis and cause stroke or heart attack.

Even non-functioning tumors pose a threat by growing too large and/or causing nerve and organ damage, and because they are made of the same cells as functioning tumors, they can “switch on” at any time causing all the dangers that a functioning pheo and para patient experience.

How common is pheochromocytoma and paraganglioma?

How common is pheochromocytoma and paraganglioma?

Recent research has indicated that as many as 1 in 3,000 will be diagnosed with pheo para in their lifetime, but many go years without a diagnosis.  Although it is considered a rare disease, it is not as rare as previously thought.  Still, many doctors will go their entire career without ever seeing a pheo or para patient.

Is it Genetic? (Can I pass this down to my children?)

Is it Genetic? (Can I pass this down to my children?)

Approximately 30-40% of pheochromocytomas and paragangliomas are hereditary.

There is a 50% chance you can pass it down to your children if you carry a genetic mutation.  If you are diagnosed with pheo para, discuss genetic testing with your doctor.  Read more information on our genetics page.

Is there a cure for paraganglioma or pheochromocytoma?

Is there a cure for paraganglioma or pheochromocytoma?

The only cure to para and pheo at this time is surgical removal of the tumor. There are treatment options available to patients who have inoperable tumors and for those who have metastatic pheo or para. To find out more about treatment options, visit the pheo education page or the para education page.

If it is not pheo or para, what could it be?

If is is not pheo or para, what could it be?

Given its rarity and the fact that pheo is called, “The Great Mimic” there are many ailments and diseases that have similar symptoms of pheo and para. Here is a list of differential diagnoses.