Pheochromocytoma and paraganglioma are rare tumors that can be benign (not cancer) or malignant. Pheochromocytomas form in the adrenal glands, and paragangliomas usually along nerve pathways in the head, neck, and spine. Explore the links on this page to learn more about these tumors, their treatment, research, and clinical trials.

Pheochromocytoma and Paraganglioma Treatment

  • Pheochromocytoma and paraganglioma are rare tumors that come from the same type of tissue.
  • Pheochromocytoma is a rare tumor that forms in the adrenal medulla (the center of the adrenal gland).
  • Paragangliomas form outside the adrenal gland.
  • Certain inherited disorders and changes in certain genes increase the risk of pheochromocytoma or paraganglioma.
  • Signs and symptoms of pheochromocytoma and paraganglioma include high blood pressure and headache.
  • Signs and symptoms of pheochromocytoma and paraganglioma may occur at any time or be brought on by certain events.
  • Tests that examine the blood and urine are used to detect (find) and diagnose pheochromocytoma and paraganglioma.
  • Genetic counseling is part of the treatment plan for patients with pheochromocytoma or paraganglioma.
  • Certain factors affect prognosis (chance of recovery) and treatment options.

Pheochromocytoma is a rare tumor that forms in the adrenal medulla (the center of the adrenal gland).

Pheochromocytoma forms in the adrenal glands. There are two adrenal glands, one on top of each kidney in the back of the upper abdomen. Each adrenal gland has two parts. The outer layer of the adrenal gland is the adrenal cortex. The center of the adrenal gland is the adrenal medulla.

Pheochromocytoma is a rare tumor of the adrenal medulla. Usually, pheochromocytoma affects one adrenal gland, but it may affect both adrenal glands. Sometimes there is more than one tumor in one adrenal gland.

The adrenal glands make important hormones called catecholamines. Adrenaline (epinephrine) and noradrenaline (norepinephrine) are two types of catecholamines that help control heart rate, blood pressure, blood sugar, and the way the body reacts to stress. Sometimes a pheochromocytoma will release extra adrenaline and noradrenaline into the blood and cause signs or symptoms of disease.

Paragangliomas form outside the adrenal gland.

Paragangliomas are rare tumors that form near the carotid artery, along nerve pathways in the head and neck, and in other parts of the body. Some paragangliomas make extra catecholamines called adrenaline and noradrenaline. The release of these extra catecholamines into the blood may cause signs or symptoms of disease.

Signs and symptoms of pheochromocytoma and paraganglioma include high blood pressure and headache.

High blood pressure.
Headache.
Heavy sweating for no known reason.
A strong, fast, or irregular heartbeat.
Being shaky.
Being extremely pale.

The most common sign is high blood pressure. It may be hard to control. Very high blood pressure can cause serious health problems such as irregular heartbeat, heart attack, stroke, or death.

Stages of Pheochromocytoma and Paraganglioma

  • After pheochromocytoma and paraganglioma have been diagnosed, tests are done to find out if the tumor has spread to other parts of the body.
  • There are three ways that cancer spreads in the body.
  • Cancer may spread from where it began to other parts of the body.
  • There is no standard staging system for pheochromocytoma and paraganglioma.
  • Pheochromocytoma and paraganglioma are described as localized, regional, or metastatic.
    • Localized pheochromocytoma and paraganglioma
    • Regional pheochromocytoma and paraganglioma
    • Metastatic pheochromocytoma and paraganglioma

Childhood Pheochromocytoma and Paraganglioma Treatment

  • Pheochromocytoma forms in the adrenal gland.
  • Paraganglioma forms in nerve tissue near the carotid artery, along nerve pathways in the head and neck, and in other parts of the body.
  • Pheochromocytoma and paraganglioma may be benign (not cancer) or malignant (cancer).
  • Inheriting certain gene mutations (changes) increases the risk of pheochromocytoma and paraganglioma.
  • Signs and symptoms of pheochromocytoma and paraganglioma occur if too much adrenaline or noradrenaline is released into the blood.
  • Tests and procedures used to diagnose and stage pheochromocytoma and paraganglioma depend on the patient’s signs and symptoms and family history.
  • Certain factors affect prognosis (chance of recovery) and treatment options.

What is Cancer?

  • Cancer can start almost anywhere in the human body, which is made up of trillions of cells. Normally, human cells grow and divide to form new cells as the body needs them. When cells grow old or become damaged, they die, and new cells take their place.

  • Cancer prevention is action taken to lower the risk of getting cancer. This can include maintaining a healthy lifestyle, avoiding exposure to known cancer-causing substances, and taking medicines or vaccines that can prevent cancer from developing.

  • Cancer can cause many different symptoms. Most often these symptoms are not caused by cancer, but by benign tumors or other problems. If you have symptoms that last for a couple of weeks, your doctor will do a physical exam and order tests or other procedures to find out what is causing your symptoms.