Paragangliomas are tumors that develop from neuroendocrine cells that are not from adrenal gland. Pheochromocytoma are like paragangliomas except they that they develop from neuroendocrine cells from within the adrenal gland. Neuroendocrine cells are cells that are a combination between a nerve cell and a hormone-producing endocrine cell. These cells can be from the sympathetic nervous system or the parasympathetic nervous system.
Types of Paraganglioma:
Sympathetic paragangliomas are tumors that have developed from neuroendocrine cells from the sympathetic nervous system. These cells produce excess amount of catecholamines which causes an increase heart rate, blood pressure, breathing and sweating. Sympathetic paragangliomas are often found in the abdomen and thorax. As they produce excess of catecholamines they are sometimes call a secreting paraganglioma (sPGL).Parasympathetic paragangliomas arise from neuroendocrine cells from the parasympathetic nervous system. These tumors are generally located in the carotid body and the head and neck area. They are often painless and slow growing, and are less likely to produce hormones so usually do not have symptoms of increased heart rate etc. Instead signs and symptoms are often linked to their size and when large enough can cause compression of tissue and nerves close to the tumour.
Treatment:
Treatment options depends on the type, location and stage of the paraganglioma. Medication can be taken to normalize blood pressure and heart rate if this is an issue. Surgery is often the treatment of choice and chemotherapy may be suitable in some cases.
Drugs used to treat Paraganglioma
| Name | Drug Class |
|---|---|
| Iobenguane i-131 | Therapeutic radiopharmaceuticals |
| Azedra | Therapeutic radiopharmaceuticals |
