Paraganglioma – Symptoms and Causes
Overview
A paraganglioma is a rare growth that develops from specific nerve cells found throughout the body. These tumors most commonly appear in the head, neck, abdomen, or pelvis.
Most paragangliomas are benign (non-cancerous), though some can be malignant (cancerous) and spread to other parts of the body.
The cause of paragangliomas isn’t always clear. Some cases are linked to inherited genetic changes that pass from parents to children.
These tumors can develop at any age, but healthcare providers typically diagnose them in adults between 20 and 50 years old.
Signs and Symptoms
Paraganglioma symptoms vary depending on the tumor location. These tumors typically develop in the head, neck, abdomen, or pelvic region.
When paragangliomas form in the head or neck, you might experience:
- A pulsing or whooshing sound in your ears
- Difficulty swallowing
- Voice changes or hoarseness
- Reduced hearing
- Vision problems
- Feelings of lightheadedness
These symptoms often develop as the tumor grows and presses against nearby tissues and structures.
Paragangliomas in other body areas frequently cause symptoms related to hormone production. These tumors can release hormones called catecholamines, which include adrenaline and help manage the body’s stress response.
Hormone-producing paragangliomas may cause:
- Elevated blood pressure
- Heart palpitations (feeling like your heart is racing or pounding)
- Sudden paleness
- Excessive sweating
- Headaches
- Tremors in the hands or arms
- Weakness throughout the body
These symptoms typically come and go rather than remaining constant.
Some people with paragangliomas experience no symptoms at all. Their tumors may be discovered accidentally during imaging tests performed for unrelated reasons.
When Medical Care Is Needed
Schedule an appointment with a healthcare provider if you notice potential paraganglioma symptoms, especially if you experience multiple symptoms simultaneously.
You should also discuss your paraganglioma risk with your doctor if you have difficult-to-control high blood pressure requiring multiple medications.
Additionally, seek medical advice if you notice your blood pressure readings spike occasionally when measured at home or during medical appointments.
Causes
Paragangliomas develop when special nerve cells called chromaffin cells experience DNA changes. These cells normally help control important body functions like blood pressure.
The exact cause of paragangliomas remains unclear in many cases. Some key factors include:
Genetic Factors
- Inherited DNA mutations passed from parents to children
- Some patients have a family history of these tumors
- Many cases occur without any family history
Cell Growth Problems
When chromaffin cells develop DNA mutations, they receive faulty instructions. Instead of following normal growth patterns, these cells:
- Multiply rapidly without control
- Fail to die when they should
- Form masses of abnormal cells (tumors)
Most paragangliomas remain confined to their original location. However, some can become metastatic, meaning cells break away and spread to other body areas. Common sites for spread include:
- Nearby lymph nodes
- Lungs
- Liver
- Bones
Paragangliomas are closely related to pheochromocytomas. The main difference is location—pheochromocytomas form specifically in the adrenal glands (located above the kidneys), while paragangliomas develop in nerve tissues elsewhere in the body.
Both tumor types arise from the same cell type and share similar behaviors, though their specific symptoms may differ based on where they grow and what hormones they might produce.
Risk Factors
If paragangliomas run in your family, it may indicate the presence of certain inherited DNA changes.
Several inherited health conditions can increase the risk of developing paragangliomas:
Multiple Endocrine Neoplasia, Type 2 (MEN 2): This condition causes hormone-producing gland tumors, particularly in the thyroid and parathyroid glands. Both types (2A and 2B) increase paraganglioma risk.
Von Hippel-Lindau Disease: This disorder leads to tumor and cyst formation in multiple body areas, including the brain, spinal cord, and kidneys.
Neurofibromatosis 1: This condition causes skin tumors called neurofibromas and can also affect the optic nerve (the nerve connecting the eye to the brain).
Hereditary Paraganglioma Syndromes: These genetic conditions specifically cause pheochromocytomas or paragangliomas. Affected individuals often develop multiple paragangliomas.
Carney-Stratakis Dyad: This rare inherited condition causes both digestive tract tumors and paragangliomas.
If you have any of these conditions or a family history of paragangliomas, talk with your doctor about your personal risk and appropriate monitoring options.
