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A paraganglioma is a growth of cells that can happen in different places in the body. The growth, called a tumor, forms from an important type of nerve cell that's found throughout the body. Paragangliomas most often start in the head, neck, stomach area or pelvis.
A paraganglioma is rare. And most often, it's not cancer. When a tumor isn't cancer, it's called benign. Sometimes a paraganglioma is cancerous. A cancerous tumor can spread to other parts of the body.
Paragangliomas often don't have a clear cause. Some paragangliomas are caused by DNA changes that pass from parents to children.
Paragangliomas can form at any age. Healthcare professionals most often find them in adults between the ages of 20 and 50.
Paraganglioma treatment typically involves surgery to remove the tumor. If the paraganglioma is cancerous and spreads to other areas of the body, more treatments may be needed.
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الأعراض
Paraganglioma symptoms may depend on where the tumor starts. Paragangliomas most often start in the head, neck, stomach area or pelvis.
Symptoms of a paraganglioma in the head or neck can include:
- A rhythmic pulsing or whooshing sound in the ears, called pulsatile tinnitus.
- Trouble swallowing.
- Hoarse voice.
- Hearing loss.
- Blurred vision.
- Dizziness.
Symptoms of a paraganglioma in the head and neck area might happen as the tumor grows larger. The tumor can press on nearby structures.
When paragangliomas form in other places in the body, the symptoms are more likely to be caused by hormones that the paraganglioma makes. The hormones, called catecholamines, play a role in how the body response to stress. They include adrenaline, also known as the fight-or-flight hormone.
Symptoms of paragangliomas that make hormones include:
- High blood pressure.
- Feelings of having a fast-beating, fluttering or pounding heart.
- The sudden loss of color in the face.
- Sweating.
- Headache.
- Uncontrolled shaking in the hands or arms.
- General weakness.
These symptoms may come and go.
Some people with paragangliomas don't have any symptoms. They may learn they have these tumors when imaging tests done for other reasons happen to spot the tumors.
When to see a doctor
Make an appointment with a doctor or other healthcare professional if you're concerned you may have paraganglioma symptoms. This is important if you have multiple paraganglioma symptoms at the same time.
Talk with your healthcare professional about your risk of paraganglioma if you have high blood pressure that's hard to control. This includes high blood pressure that needs treatment with more than one medicine. Also talk with your healthcare professional if your blood pressure spikes from time to time when you or a healthcare professional measures it.
الأسباب
Paragangliomas often don't have a clear cause. Sometimes these tumors run in families. They can be caused by DNA changes that are passed from parents to children. But many people with paragangliomas don't have a family history of these tumors and the cause is not known.
A paraganglioma is a growth of cells. It forms from a type of nerve cell called a chromaffin cell. Chromaffin cells perform key roles in the body, including controlling blood pressure.
A paraganglioma starts when chromaffin cells develop changes in their DNA. A cell's DNA holds the instructions that tell the cell what to do. In healthy cells, the DNA gives instructions to grow and multiply at a set rate. The instructions also tell the cells to die at a set time. In paraganglioma cells, the DNA changes give different instructions. The changes tell the paraganglioma cells to make many more cells quickly. The cells keep living when healthy cells would die. This causes a growth of cells called a tumor.
Most paragangliomas stay where they started. They don't spread to other parts of the body. But sometimes cells can break away from a paraganglioma and spread. When this happens, it's called metastatic paraganglioma. When a paraganglioma spreads, it most often spreads to nearby lymph nodes. It also can spread to the lungs, liver and bones.
A paraganglioma is closely related to another rare tumor called a pheochromocytoma. A pheochromocytoma is a tumor that starts in chromaffin cells in the adrenal glands. The adrenal glands are two glands that sit on top of the kidneys.
عوامل الخطورة
The risk of paraganglioma is higher in people who have a family history of this tumor. Some paragangliomas are caused by DNA changes that pass from parents to children. Having a family history of paraganglioma might be a sign that certain DNA changes run in your family.
Some other health conditions caused by DNA changes that pass from parents to children raise the risk of paragangliomas. These conditions include:
- Multiple endocrine neoplasia, type 2. Multiple endocrine neoplasia, type 2, also called MEN 2, can cause tumors in one or more of the glands that make hormones, including the thyroid and the parathyroid glands. There are two types of MEN 2 — type 2A and type 2B. Both raise the risk of paragangliomas.
- Von Hippel-Lindau disease. Von Hippel-Lindau disease can cause tumors and cysts to form in many parts of the body. Possible sites include the brain, spinal cord and kidneys.
- Neurofibromatosis 1. Neurofibromatosis 1 causes tumors called neurofibromas to form in the skin. The condition also can cause tumors of the optic nerve. The optic nerve is the nerve at the back of the eye that connects to the brain.
- Hereditary paraganglioma syndromes. Hereditary paraganglioma syndromes can cause pheochromocytomas or paragangliomas. People with these syndromes often have more than one paraganglioma.
- Carney-Stratakis dyad. Carney-Stratakis dyad causes tumors of the digestive tract and paragangliomas.