Hereditary paraganglioma-pheochromocytoma syndromeis a condition in which tumors develop in structures called paraganglia. Paraganglia are bundles of cells of the peripheral nervous system (the nerves outside the brain and spinal cord). A tumor that develops in the paraganglia is called a paraganglioma.
There are two types of paragangliomas:
Sympathetic paragangliomas — These produce and release catecholamines into the bloodstream. Catecholamines are certain kinds of hormones, such as epinephrine, norepinephrine and dopamine. Hormones are chemical messengers that send important instructions to different parts of the body. Normally, catecholamines are released into the bloodstream by the adrenal glands. The adrenal glands, located on top of each kidney, produce catecholamines in response to stress.
Parasympathetic paragangliomas — These do not usually release catecholamines into the bloodstream.
Most paragangliomas are usually found in the head, neck or torso. A specific type of sympathetic paraganglioma, called a pheochromocytoma, develops in the adrenal glands.
One of the curious things about Neuroendocrine Cancer (NETs elsewhere in the text) is that it can very often exhibit one or more vague symptoms collectively known as a 'syndrome'. Syndrome is an apt word to describe these complications as the most general meaning in medical terms is a group of symptoms that together are characteristic of a specific disorder or disease". Having a syndrome can often be the difference between having a 'functional' condition or a non-functional' condition - see more below.This frequently makes Neuroendocrine Cancer very difficult to diagnose quickly. It's a very devious disease.It's NOT all about Carcinoid Syndrome!Most people think of Carcinoid Syndrome when they discuss NETs. Anyone suggesting that all NET patients get carcinoid syndrome or that all symptoms of NETs are caused by carcinoid…