What Are Pheochromocytoma and Paraganglioma Syndromes?
Pheochromocytomas and paragangliomas are rare types of neuroendocrine tumors that are typically benign, or noncancerous. Pheochromocytomas and paragangliomas are caused by an abnormal growth in chromaffin cells. Chromaffin cells are a type of cell that makes neurohormones and releases the neurohormones into the blood. Neurohormones are chemicals that are made by nerve cells and used to send signals to other cells. While many pheochromocytomas and paragangliomas are benign, some can become cancerous (malignant). Early detection and treatment of the tumor is important for optimal results. The diseases can be diagnosed by blood and urine tests, imaging tests or genetic testing.
While pheochromocytoma and paraganglioma are often used interchangeably, paragangliomas that occur in the adrenal medulla are called pheochromocytomas, and those outside the adrenal gland are called paragangliomas.
How Pheochromocytomas and Paragangliomas Develop
Pheochromocytomas develop in the adrenal glands, which are located above each kidney in the back of the upper abdomen. When chromaffin cells form a tumor elsewhere in the body, they are called paragangliomas.
The adrenal glands create hormones called catecholamines which help control one's heart rate, blood pressure, blood sugar, and the way the body reacts to stress. Sometimes pheochromocytoma and paraganglioma syndromes release extra neurohormones, adrenaline (epinephrine) and noradrenaline (norepinephrine), into the blood and cause signs or symptoms of disease.
Symptoms of Pheochromocytomas and Paragangliomas
Symptoms of pheochromocytoma and paraganglioma syndromes include:
High blood pressure/hypertension (most common)
Heavy sweating for no reason (hyperhidrosis)
Strong, fast, or irregular heartbeat
Extreme paleness (pallor)
How We Treat Pheochromocytomas and Paragangliomas
Dana-Farber Brigham Cancer Center experts at our Neuroendocrine and Carcinoid Tumors Program, part of the Gastrointestinal Treatment Cancer Center, are studying pheochromocytoma and paraganglioma syndromes to better understand who is at high risk and determine the best treatment. The program's multidisciplinary approach to treatment often involves medical therapies, surgery and chemotherapy. Experts work together to create an individualized treatment plan for each patient.
Pheochromocytomas and paragangliomas are increasingly being recognized as part of a larger syndrome with a genetic basis. Patients and families also have access to our Center for Cancer Genetics and Prevention. Adrenal endocrinologists, adrenal geneticists, and genetic counselors provide patients with new genetic technologies that can identify their risk, or a family member's risk, for developing pheochromocytoma and paraganglioma syndromes.