Where do pheochromocytoma and paraganglioma tumors come from?
The neuroendocrine system is made up of special cells throughout the body. These cells use chemicals called hormones to communicate. Pheochromocytoma and paraganglioma are neuroendocrine tumors, which means they develop from these hormone-producing cells.
What makes pheochromocytoma and paraganglioma tumors unique?
Pheochromocytoma and paraganglioma tumors produce and release increased amounts of hormones. Normally, these hormones are a part of the body’s “fight or flight” response. Uncontrolled levels of these hormones can result in a wide variety of symptoms, including:
- High blood pressure (hypertension)
- Rapid heartbeat
These symptoms can be very serious for some people. Managing symptoms is therefore an important part of treating pheochromocytoma and paraganglioma.
Hormones produced by pheochromocytoma and paraganglioma are known as "catecholamines," and can include epinephrine (adrenaline), norepinephrine (noradrenaline), and dopamine.
How are pheochromocytoma and paraganglioma treated?
Most pheochromocytoma and paraganglioma tumors can be removed with surgery. However, some people have tumors that spread or cannot be removed with surgery. These unresectable, locally advanced, or metastatic tumors are called "advanced pheochromocytoma and paraganglioma."
- Unresectable means that a tumor or set of tumors cannot be removed with surgery
- Locally advanced means that the disease has spread into nearby tissue
- Metastatic means that the disease has spread to other parts of the body
If you or someone you know is living with advanced pheochromocytoma or paraganglioma, it is important to understand the nature of the disease—and what treatments may help.
There are dual goals for treating pheochromocytoma and paraganglioma
If a tumor has not spread to other parts of the body, it can usually be removed with surgery. However, for advanced pheochromocytoma and paraganglioma there are two goals of therapy:
Pheochromocytoma and paraganglioma symptoms can be acute, severe, and even life-threatening if untreated.
Tumor growth is the leading cause of death for people with advanced pheochromocytoma and paraganglioma.