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What is a pheochromocytoma?

A pheochromocytoma is an uncommon and usually benign tumor that develops on your adrenal gland. The adrenal glands are above each kidney, and produce multiple hormones. This tumor, or abnormal growth of cells, can produce excess hormones that may lead to high blood pressure, anxiety, headaches, heart palpitations, and other symptoms.

Can a pheochromocytoma become cancerous?

Most pheochromocytomas are benign, meaning they are not cancerous and cannot spread to other parts of the body. However, 10-15% of pheochromocytomas will become cancerous.

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Causes

The cause of pheochromocytoma is unknown. However, about a third of cases occur in people who have family members with the disease.

Pheochromocytoma tumors are more likely to occur in people who have inherited conditions or disorders from their parents. The most common disorders that can increase the risk of developing pheochromocytoma tumors are: multiple endocrine neoplasia type II, Von Hippel-Lindau disease, neurofibromatosis 1, and hereditary paraganglioma syndrome. These potentially inherited cases are more commonly cancerous.

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Symptoms

What are pheochromocytoma symptoms?

‍Pheochromocytoma is most often diagnosed in individuals between the ages of 20-50. They usually have one or more of the following signs or symptoms:

High blood pressure
Headaches
Excessive sweating
Rapid or irregular heartbeat
Shortness of breath
Tremors
Pallor, or loss of color in the face
Panic attacks or increased anxiety symptoms

What makes pheochromocytoma symptoms worse?

Symptoms may be constant or can be triggered and/or made worse by the following:

Physical or emotional stress including physical exertion, surgery, or labor and delivery
Eating foods that are high in tyramine content, such as foods that are fermented, rancid, cured, or preserved. These include some cheeses, beers or wines, chocolate, and dried or smoked meats.
Drugs or medications:

Stimulants, like amphetamines or cocaine
Antidepressants like Amitriptyline, Doxepin, or Imipramine
Anesthesia
Beta-blockers

Diagnosis

How is a pheochromocytoma diagnosed?

‍It can be hard to diagnose pheochromocytoma because symptoms are nonspecific and are common to several other diseases. Genetic testing can help with diagnosis in individuals with a family history of pheochromocytoma. If your doctor suspects you have a pheochromocytoma, you may undergo a blood or urine tests to look for elevated stress-hormone levels. Your doctor may order an MRI or CT scan to check for a tumor.

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Treatment

How is a pheochromocytoma treated?

It’s typically treated with surgery to remove the tumor. Based on the extent of the tumor, your doctor will determine how much tissue is removed. If the pheochromocytoma is on both adrenal glands, just the tumors may be removed. If the pheochromocytoma exists on just one adrenal gland, the doctor may remove the tumor and the gland together. Your other adrenal gland will work to compensate for the loss. If the pheochromocytoma is cancerous, chemotherapy or radiation may also be necessary.

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References

**MacMillan, A. (n.d.). Pheochromocytoma: Symptoms, Causes, Treatment, and Prognosis. WebMD. https://www.webmd.com/cancer/what_is_pheochromocytoma**

**Mayo Foundation for Medical Education and Research. (2020, March 3). Pheochromocytoma. Mayo Clinic. https://www.mayoclinic.org/diseases-conditions/pheochromocytoma/symptoms-causes/syc-20355367**.

https://medlineplus.gov/genetics/condition/autoimmune-addison-disease/#inheritance.

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Information

Medically reviewed by:‍

Stuart Seigel, MD

Dr. Seigel is a board certified Endocrinologist. He completed his residency in internal medicine and fellowship in endocrinology, diabetes and metabolism at the University Of Pittsburgh Medical Center in 2011 and 2013.

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