Pheochromocytoma

Pheochromocytoma is a rare, usually benign, tumor of the adrenal glands that produces excess catecholamines, such as epinephrine and norepinephrine.

Causes and Risk Factors

The exact cause of pheochromocytoma is not known, but it is believed to result from genetic mutations that affect the cells of the adrenal gland. Certain genetic syndromes, such as multiple endocrine neoplasia type 2 (MEN2), von Hippel-Lindau disease, and neurofibromatosis type 1, increase the risk of developing pheochromocytoma.

Symptoms

The symptoms of pheochromocytoma are caused by the excess production of catecholamines and can include:

• High blood pressure
• Headaches
• Panic attacks or anxiety
• Rapid heartbeat or palpitations
• Sweating or flushing
• Nausea and vomiting
• Weight loss
• Fatigue

Diagnosis

The diagnosis of pheochromocytoma is typically made through a combination of:

• Physical examination and medical history
• Imaging tests, such as computed tomography (CT) or magnetic resonance imaging (MRI), to visualize the tumor
• Blood and urine tests to measure the levels of catecholamines and their metabolites
• Genetic testing to identify any underlying genetic syndromes

Treatment

The primary treatment for pheochromocytoma is surgical removal of the tumor. Prior to surgery, medications such as alpha-blockers and beta-blockers may be used to control blood pressure and heart rate.

In some cases, chemotherapy or radiation therapy may be used to treat malignant pheochromocytomas that have spread to other parts of the body.

Complications

If left untreated, pheochromocytoma can lead to serious complications, including:

• Cardiovascular disease and heart failure
• Stroke or cerebral hemorrhage
• Kidney damage or kidney failure
• Malignant transformation of the tumor

Prognosis

The prognosis for pheochromocytoma is generally good if the tumor is diagnosed and treated early. The 5-year survival rate for patients with benign pheochromocytoma is approximately 95%. However, the prognosis is poorer for patients with malignant pheochromocytoma or those who experience complications.