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].
Is paraganglioma a type of cancer?
Paragangliomas are usually noncancerous (benign). But some paragangliomas can become cancerous (malignant) and spread (metastasize) to other parts of the body. Paragangliomas are rare tumors.
Where are pheochromocytomas located?
These cells produce hormones needed for the body and are found in the adrenal glands. The adrenal glands are small organs located in the upper region of the abdomen on top of the kidneys. About 80-85% of pheochromocytomas grow in the inner layer of the adrenal gland, called the adrenal medulla.
Where is a paraganglioma located?
Paragangliomas are usually found in the head, neck, or torso. However, a type of paraganglioma known as pheochromocytoma develops in the adrenal glands . Adrenal glands are located on top of each kidney and produce hormones in response to stress. Most people with paraganglioma develop only one tumor in their lifetime.
What are the symptoms of paraganglioma?
Signs and symptoms of pheochromocytoma and paraganglioma include high blood pressure and headache.
- High blood pressure.
- Headache.
- Heavy sweating for no known reason.
- A strong, fast, or irregular heartbeat.
- Being shaky.
- Being extremely pale.
How long can you live with 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%.
Are pheochromocytomas always benign?
A pheochromocytoma is a rare, catecholamine-secreting tumor that may precipitate life-threatening hypertension.
What is the treatment for paraganglioma?
Treatment of localized benign pheochromocytoma or paraganglioma is usually surgery to completely remove the tumor. If the tumor is in the adrenal gland, the entire adrenal gland is removed. Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients.
What kind of tumor is an extra adrenal paraganglioma?
Extra-adrenal paragangliomas, nowadays often referred to as only paragangliomas, are classified as sympathetic or parasympathetic depending on the type of paraganglia in which they have their origin.
Where are the paraganglia located in a paraganglioma?
Sympathetic paragangliomas arise from chromaffin cells of paraganglia along the sympathetic chains and are usually located in the chest, abdomen, or pelvis (Figure 1).
How are paraganglia part of the neuroendocrine system?
Paraganglia consist of prominent neuroendocrine cells containing neurosecretory vesicles. Those that have been adequately investigated have been shown to contain active amine or peptide hormones and are therefore regarded as part of the neuroendocrine system.
How long does it take for a paraganglioma to grow?
Carotid body tumors and paragangliomas of the head and neck are typically painless, slow growing tumors that are often present for years prior to the patient seeking medical attention. They may attain large size and infiltrative growth and local recurrence may lead to death.