Pheochromocytoma

A pheochromocytoma is a tumor that most often develops in special cells of the adrenal gland called chromaffin cells. Pheochromocytomas produce too many hormones called catecholamines, which include adrenaline. Most pheochromocytomas start in the inside part of the adrenal gland called the adrenal medulla. Symptoms of a pheochromocytoma can include severe high blood pressure, headaches, and sweating. Treatment for a pheochromocytoma can include surgery, radiation therapy, and chemotherapy.

 

What Is a Pheochromocytoma?

A pheochromocytoma is a tumor that most commonly occurs within special cells of the adrenal gland called chromaffin cells. This type of tumor produces too many hormones called catecholamines, which include adrenaline. The high levels of these hormones can cause potential serious complications, including severe high blood pressure.
 

Understanding the Adrenal Glands and Pheochromocytoma

Most pheochromocytomas start within the inside part of the adrenal gland called the adrenal medulla where most chromaffin cells are located. There are two adrenal glands, one above each kidney in the back of the upper abdomen. Cells in the adrenal glands make important hormones that help the body work properly, called catecholamines. Catecholamines produced by the adrenal gland include epinephrine (adrenaline), norepinephrine, and (rarely) dopamine.
 
A pheochromocytoma makes large amounts of catecholamines, which can result in serious complications. Usually pheochromocytoma affects only one adrenal gland. Pheochromocytoma may also start in other parts of the body, such as the area around the heart or bladder.
 
Most tumors that start in the chromaffin cells do not spread to other parts of the body, meaning they are not cancer. These are called benign tumors. If a tumor is found, the doctor will need to determine whether it is cancerous or benign.
 
A pheochromocytoma is sometimes part of a condition called multiple endocrine neoplasia syndrome (MEN). People with MEN often have other cancers (such as thyroid cancer) and other hormonal problems.
 

Symptoms of a Pheochromocytoma

Pheochromocytomas cause the adrenal glands to make too many hormones called catecholamines (which include adrenaline). The extra catecholamines cause common pheochromocytoma symptoms. These symptoms of pheochromocytoma can include:
 

Diagnosing a Pheochromocytoma

If a person has possible pheochromocytoma symptoms, the doctor may do a physical exam and ask about the patient's personal and family medical history. In addition, when diagnosing pheochromocytoma, the doctor will also likely recommend additional tests and procedures, including:
 
  • Blood and urine tests to see if there are extra hormones in the body
  • CT scan
  • Nuclear medicine scans (metaiodobenzylguanidine scans, or MIBG scans for short)
  • MRIs.
     

Pheochromocytoma Staging

Once a doctor has made a pheochromocytoma diagnosis, he or she will order more tests to find out if cancer cells have spread to other parts of the body. This is called pheochromocytoma staging. It is important to know the stage in order to plan pheochromocytoma treatment.
 
 
  • Localized benign pheochromocytoma
  • Regional pheochromocytoma
  • Metastatic pheochromocytoma
  • Recurrent pheochromocytoma.
     

Treating a Pheochromocytoma

Treatment for pheochromocytoma varies based on:
 
In general, treatment for pheochromocytoma can include:
 
  • Surgery
  • Radiation therapy
  • Chemotherapy.
     
The doctor can describe the treatment options for pheochromocytoma and the expected results of each. The patient and the doctor can work together to develop a treatment plan that meets the patient's medical needs and personal values. Choosing the most appropriate pheochromocytoma treatment is a decision that ideally involves the patient, family, and healthcare team.
 

Pheochromocytoma: Prognosis

 
  • The stage of pheochromocytoma
  • The patient's age and general health.
     
Written by/reviewed by: Arthur Schoenstadt, MD
Last reviewed by: Arthur Schoenstadt, MD