Pheochromocytomas & Paragangliomas

Pheochromocytomas are adrenal tumors that produce excess catecholamines (epinephrine and norepinephrine). Normally, these hormones help the body respond to emotional and physical stress. In excess, however, these hormones can lead to several serious health conditions, including severe high blood pressure and strokes. Most pheochromocytomas begin in the adrenal medulla, located within the adrenal glands. Paragangliomas are catecholamine-producing nodules that are found in the abdomen, pelvis, chest, and neck.

An estimated 800 new cases of pheochromocytoma are diagnosed each year in the United States. Most pheochromocytomas are benign, but up to 10 percent are malignant and can spread (metastasize) beyond the adrenal gland.

Multiple endocrine neoplasia (MEN) and von Hippel Lindau disease (VHL) are two genetic conditions that may increase the risk of developing a pheochromocytoma and other tumors. Patients with signs of MEN or VHL are referred to our genetic counseling service for further evaluation.

Symptoms

Symptoms caused by excess catecholamines include:

  • sweating
  • headaches
  • high blood pressure
  • rapid heart rate
  • heart palpitations
  • anxiety
  • fever
  • visual changes
  • elevated blood sugar

In some cases, pheochromocytomas can cause sudden cardiac arrest or stroke. People with pheochromocytomas who experience a severe injury or undergo surgery are at risk of a life-threatening surge in adrenaline. This situation can be avoided if a pheochromocytoma is diagnosed and treated by a team of specialists.

Diagnosis

An endocrinologist, surgeon, or other specialist can diagnose a pheochromocytoma by reviewing the patient’s medical history, performing a physical examination, and measuring levels of catecholamines and their byproducts in the blood and urine with laboratory tests. If laboratory tests indicate the presence of a tumor, imaging tests such as CT and MRI are used to identify its exact location.

A nuclear imaging technique called MIBG ScintiScan may help locate tumors that are too small to be detected by CT or MRI, or have spread. In this procedure, the patient receives an injection of MIBG, a protein similar to adrenaline, combined with a radioactive substance. Radiation-sensitive imaging tests reveal tumors that have absorbed the MIBG.

Treatment

Surgery is the treatment of choice for pheochromocytomas. However, surgery is recommended only after the patient has been treated with medical therapy to stabilize blood pressure.

Patients with a malignant pheochromocytoma that has spread (metastasized) beyond the adrenal glands may require one or a combination of treatments, including medical therapy to control blood pressure, surgery, radiolabeled MIBG therapy, external radiation therapy, and chemotherapy. Medical management to control blood pressure, combined with surgery, also may be recommended for patients with a benign paraganglioma.