Paraganglioma & Pheochromocytoma

About Paraganglioma & Pheochromocytoma

Paragangliomas and Pheochromocytomas and are rare tumors associated with ganglia of the sympathetic nervous system. Paragangliomas grow along the nerve pathways of the peripheral nervous system. There are two types of paragangliomas, sympathetic and parasympathetic. Sympathetic paragangliomas grow along nerve pathways found in the head, neck, chest cavity, abdomen, pelvis and bladder, outside of the adrenal gland. Pheochromocytomas are a type of sympathetic paraganglioma that develop in the adrenal medulla of the adrenal gland. Sympathetic paragangliomas produce hormones called catecholamines, such as epinephrine (adrenaline) or norepinephrine control the “fight or flight” response in response to stress that can cause rapid heartrate, high blood pressure, breathing, headaches and sweating. These hormone surges can lead to life-threatening health problems, such as a stroke, heart attack, hemorrhage, or sudden death.

The majority of parasympathetic paragangliomas are located in the head, neck and skull base along the glossopharyngeal and vagus nerves usually do not produce hormones. The parasympathetic nervous system controls involuntary body functions such as saliva production and digestion.

Paragangliomas are typically slow growing, but approximately 40-50% can become cancerous and spread distantly to other parts of the body. These tumors can affect people of any age but most often show up between the ages of 25 and 50, it is estimated that up to 25% of paragangliomas are inherited. Other names for this condition include: familial paraganglioma syndrome, familial paraganglioma syndrome, FPGL FPGL/PHEO, hereditary paraganglioma-pheochromocytoma syndromes, glomus tumor, extra-adrenal pheochromocytomas, chromaffin and non-chromaffin paragangliomas, primary adrenal gland tumor, adrenal gland chromaffin paraganglioma, adrenal gland chromaffinoma, adrenal gland paraganglioma, intraadrenal paraganglioma.