What is an extra-adrenal paraganglioma?
Paragangliomas, also known as extra-adrenal pheochromocytomas, are neuroendocrine tumors that arise from chromaffin cells of the adrenal medulla and from neuroendocrine cells of the extra-adrenal autonomic paraganglia, with the majority of the tumours being benign in nature [1].
What is sympathetic paraganglioma?
Abstract. Paragangliomas are rare neuroendocrine tumors that arise in the sympathetic or parasympathetic nervous system. Sympathetic paragangliomas are mainly found in the adrenal medulla (designated pheochromocytomas) but may also have a thoracic, abdominal, or pelvic localization.
What triggers paraganglioma?
Most paragangliomas have no known cause, but some are caused by gene mutations that are passed from parents to children. Paraganglioma cells commonly secrete hormones known as catecholamines, including adrenaline, which is the fight-or-flight hormone.
Can you feel a paraganglioma on neck?
These tumors usually appears in the neck, but can extend into the space between the head and the neck. Some of most common symptoms are pain, hoarseness, fainting (syncope), difficulty swallowing (dysphagia), eye problems (Horner syndrome), and paralysis of the tongue.
Do paragangliomas come back?
People treated for a pheochromocytoma or paraganglioma need lifelong follow-up care. This is important because pheochromocytomas and paragangliomas can recur years after initial treatment. This care may include regular physical examinations, medical tests, or both.
Is a paraganglioma a brain tumor?
Paraganglioma is a type of neuroendocrine tumor that forms near certain blood vessels and nerves outside of the adrenal glands. The adrenal glands are important for making hormones that control many functions in the body and are located on top of the kidneys.
What kind of doctor treats paraganglioma?
A surgical oncologist is a doctor who specializes in cancer surgery. When it is possible, completely removing the entire tumor is the standard first treatment. For people with a head and neck paraganglioma that does not produce catecholamines, “watchful waiting” may be recommended instead.
How serious is a paraganglioma?
Paraganglioma are found in 2 out of every million people each year and is the cause of high blood pressure in less than 0.2% of people with high blood pressure. However, because paraganglioma release adrenaline in uncontrolled bursts, they can cause serious health problems like stroke, heart attacks, and even death.
Are paragangliomas hereditary?
Hereditary paraganglioma-pheochromocytoma is inherited in an autosomal dominant pattern , which means one copy of the altered gene in each cell is sufficient to increase the risk of developing tumors. An additional mutation that deletes the normal copy of the gene is needed to cause the condition.
How are paragangliomas diagnosed?
How is paraganglioma diagnosed? Lab Tests: If you have symptoms of paraganglioma, your doctor will order lab tests of your urine and blood to check your hormone levels. Imaging: Your doctor will use imaging scans such as MRI, CT, and PET to look at where the tumor is and how big it is.
What is the survival rate of paraganglioma?
Patients with a small paraganglioma that has not spread to other parts of the body have a five-year survival rate of about 95%. Patients with paraganglioma that has grown back (recurred) or spread to other parts of the body have a five-year survival rate between 34% and 60%.