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
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 this type of tumor affects only one adrenal gland. It 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.
Pheochromocytomas cause the adrenal glands to make too many hormones called catecholamines (which include adrenaline). The extra catecholamines cause common pheochromocytoma symptoms. These symptoms can include:
(Click Pheochromocytoma Symptoms for more information.)
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 making a diagnosis, 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.
(Click Diagnosing Pheochromocytoma for more information.)
Once a doctor has made a 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 the best treatment.
Pheochromocytoma stages include:
- Localized benign pheochromocytoma
- Regional pheochromocytoma
- Metastatic pheochromocytoma
- Recurrent pheochromocytoma.
(Click Pheochromocytoma Stages for more information.)
Treatment for pheochromocytoma varies based on:
- The pheochromocytoma stage
- The patient's age and general health.
In general, treatment can include:
- Surgery
- Radiation therapy
- Chemotherapy.
The doctor can describe the different treatment options 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 treatment is a decision that ideally involves the patient, family, and healthcare team.
Prognosis for Pheochromocytoma
The prognosis for this type of tumor depends on:
- The stage of pheochromocytoma
- The patient's age and general health.
(Click Pheochromocytoma Stages for more information.)
