Paraganglioma: Symptoms, Causes, and Treatment — A Clear Guide for Patients and Families
Updated: 2 days ago
Medically reviewed by Dr. Baraa Alnahhal, MD · Last reviewed: September 2026
TL;DR
A paraganglioma is a rare tumor of nerve cells called chromaffin cells. It most often forms in the head, neck, abdomen, or pelvis, and is usually benign (not cancer). These tumors can make stress hormones called catecholamines, which cause high blood pressure, a pounding heart, sweating, and headaches. Diagnosis relies on blood and urine hormone tests plus imaging such as MRI, CT, MIBG, or PET scans. Treatment is usually surgery, often after medicines bring hormone levels under control. Genetic testing is often recommended because some paragangliomas run in families.
Quick Answer: What You Need to Know About Paraganglioma
A paraganglioma is a rare tumor that grows from chromaffin cells, a type of nerve cell found throughout the body. [1]
It most often starts in the head, neck, abdomen, or pelvis, and is most often benign (not cancerous). [1]
Some paragangliomas make catecholamine stress hormones, causing high blood pressure, a pounding heart, sweating, and headaches that come and go. [1]
Most are found in adults between 20 and 50, but they can form at any age. [1]
Diagnosis starts with blood and urine hormone tests, followed by imaging (MRI, CT, MIBG, or PET scans) and sometimes genetic testing. [2]
Treatment is most often surgery; hormone-blocking medicines are usually given first to make treatment safer. [2]
Watchful waiting with regular checkups is a valid option for slow-growing tumors that cause no symptoms. [2]
What Is a Paraganglioma?
A paraganglioma is a growth of cells — called a tumor — that can form in different places in the body. It develops from an important type of nerve cell called a chromaffin cell, which is found throughout the body and plays key roles, including controlling blood pressure. [1]
These tumors most often start in the head, neck, stomach area (abdomen), or pelvis. They are rare, and most often they are not cancer — a non-cancerous tumor is called benign. Sometimes, however, a paraganglioma is cancerous, meaning it can spread to other parts of the body. [1]
Many paragangliomas have no clear cause. Some are caused by DNA changes that pass from parents to children. The tumors can form at any age, but healthcare professionals most often find them in adults between the ages of 20 and 50. [1]
Treatment typically involves surgery to remove the tumor. If the paraganglioma is cancerous and has spread, additional treatments may be needed. [1]
Paraganglioma Symptoms: What to Look For
Paraganglioma symptoms depend on where the tumor starts. There are two distinct symptom patterns, and knowing the difference helps make sense of this condition. [1]
Symptoms of a head and neck paraganglioma
Paragangliomas in the head or neck often cause symptoms only as the tumor grows larger and presses on nearby structures. Possible symptoms include: [1]
A rhythmic pulsing or whooshing sound in the ears, called pulsatile tinnitus
Trouble swallowing
Hoarse voice
Hearing loss
Blurred vision
Dizziness
Symptoms of a hormone-making paraganglioma
When paragangliomas form elsewhere in the body, symptoms are usually caused by the hormones the tumor makes. These hormones are called catecholamines, and they help control the body's response to stress. They include adrenaline — the fight-or-flight hormone. [1]
Symptoms of hormone-making paragangliomas include: [1]
High blood pressure
A fast-beating, fluttering, or pounding heart
Sudden loss of color in the face
Sweating
Headache
Uncontrolled shaking in the hands or arms
General weakness
These symptoms may come and go, which is one reason they are easy to overlook or mistake for everyday stress.
When a paraganglioma causes no symptoms
Some people with paragangliomas have no symptoms at all. They may learn they have a tumor when imaging tests done for other reasons happen to spot it. [1]
When to see a doctor
Make an appointment with a healthcare professional if you are concerned you may have paraganglioma symptoms — especially if you notice multiple symptoms at the same time. [1]
It is also worth discussing your risk if you have high blood pressure that is hard to control — including blood pressure that needs more than one medicine to manage — or if your blood pressure spikes from time to time when it is measured. [1]
What Causes a Paraganglioma?
Paragangliomas often have no clear cause. Sometimes they run in families, caused by DNA changes passed from parents to children. Many people with paragangliomas have no family history, and the cause is not known. [1]
The cell-level mechanism
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, DNA gives instructions to grow and multiply at a set rate, and to die at a set time. In paraganglioma cells, the DNA changes give different instructions: they tell the cells to multiply quickly and to keep living when healthy cells would die. This causes the cell growth known as a tumor. [1]
Can it spread?
Most paragangliomas stay where they started and do not spread. But sometimes cells break away from the tumor and spread — this is called metastatic paraganglioma. When it spreads, it most often goes to nearby lymph nodes, and it can also spread to the lungs, liver, and bones. [1]
How is it related to pheochromocytoma?
A paraganglioma is closely related to another rare tumor called a pheochromocytoma — a tumor that starts in chromaffin cells inside the adrenal glands, the two glands that sit on top of the kidneys. [1]
Risk Factors: Who Is More Likely to Develop One?
The risk of paraganglioma is higher in people with a family history of this tumor. Some paragangliomas are caused by inherited DNA changes, so a family history may signal that certain DNA changes run in your family. [1]
Certain other inherited conditions — themselves caused by DNA changes passed from parent to child — also raise the risk: [1]
| Inherited condition | What it is | |---|---| | Multiple endocrine neoplasia type 2 (MEN 2) | Causes tumors in one or more hormone-making glands, including the thyroid and parathyroid glands. Two types (2A and 2B) both raise paraganglioma risk. | | Von Hippel-Lindau disease | Causes tumors and cysts in many parts of the body, including the brain, spinal cord, and kidneys. | | Neurofibromatosis 1 | Causes tumors called neurofibromas in the skin and can cause tumors of the optic nerve (the nerve at the back of the eye that connects to the brain). | | Hereditary paraganglioma syndromes | Can cause pheochromocytomas or paragangliomas; people with these syndromes often have more than one paraganglioma. | | Carney-Stratakis dyad | Causes tumors of the digestive tract along with paragangliomas. |
How Is Paraganglioma Diagnosed?
Diagnosis often starts with blood and urine tests. These tests look for signs that a tumor is making extra hormones — they may detect extra catecholamines or other clues, such as a protein called chromogranin A. [2]
Imaging tests
If symptoms, family history, or blood and urine tests suggest a paraganglioma, imaging tests can show the tumor's location and size and help guide treatment options. [2]
| Imaging test | How it works | |---|---| | MRI (magnetic resonance imaging) | Uses radio waves and a magnetic field to make detailed images. | | CT scan (computerized tomography) | Combines a series of X-ray images taken from various angles around the body. | | MIBG scan (metaiodobenzylguanidine) | Detects an injected radioactive tracer that is absorbed by paragangliomas. | | PET scan (positron emission tomography) | Detects a radioactive tracer absorbed by the tumor. |
Genetic testing
Some inherited DNA changes make paragangliomas more likely to form. If you have a paraganglioma, your healthcare team may recommend genetic testing to look for these DNA changes. Results can help predict the chances of the tumor coming back after treatment. [2]
Parents, children, or siblings can also be checked for DNA changes that raise paraganglioma risk. A genetic counselor — a professional trained in genetics — can help you and your loved ones decide whether to get tested. [2]
Paraganglioma Treatment Options
Most often, treatment involves surgery to remove the tumor. If the tumor makes hormones, healthcare professionals usually use medicines to block the hormones first. If surgery cannot remove the tumor or if it has spread, other treatments may be needed. [2]
Your options depend on where the tumor is located, whether it is cancerous and has spread, and whether it makes extra hormones that cause symptoms. [2]
Step 1: Controlling the hormones first
If the paraganglioma makes extra catecholamines, you will likely need treatments to block their effects or lower their levels — this brings high blood pressure down and controls other symptoms. This step matters: blood pressure and symptoms must be under control before other treatment starts, because treatment can cause the tumor to release very large amounts of catecholamines, leading to serious problems. [2]
Medicines used include some blood pressure medicines — alpha blockers, beta blockers, and calcium channel blockers. Other measures can include eating a diet high in sodium and drinking plenty of fluids. [2]
Step 2: Surgery
Surgery removes the tumor. Even when full removal is not possible, surgery may remove as much of the tumor as possible. The type of surgery — and the surgeon who performs it — depends on the tumor's location: [2]
| Tumor location | Specialist who may operate | |---|---| | Head and neck area | Head and neck surgeons | | Brain, spine, and nerves | Neurosurgeons | | Hormone-making glands | Endocrine surgeons | | Blood vessels | Vascular surgeons |
Sometimes surgeons from different specialties work together during the operation. [2]
If surgery is not enough
When the tumor cannot be fully removed or has spread, additional treatments may be used: [2]
| Treatment | How it works | |---|---| | Radiation therapy | Powerful energy beams (X-rays, protons, or other sources) treat the tumor when surgery cannot remove it completely; can also ease pain from tumors that have spread. | | Stereotactic radiosurgery | A special radiation used for head and neck paragangliomas; many weak beams converge on the tumor, delivering a large dose exactly where they meet. | | Ablation therapy | Heat or cold kills tumor cells and controls growth; options include radiofrequency ablation (electrical heat) and cryoablation (freezing with cold gas). | | Chemotherapy | Strong medicines that shrink tumors that have spread; hormone-control medicines are given first when the tumor makes extra hormones. | | Targeted therapy | Medicines attack specific chemicals in tumor cells, causing them to die; used when surgery is not an option or the tumor has spread. | | PRRT (peptide receptor radionuclide therapy) | Medicine delivers radiation directly to tumor cells: one part finds tumor cells, the other carries radiation. Given through a vein, it sticks to paraganglioma cells and delivers radiation over days to weeks. Lutetium Lu 177 dotatate (Lutathera) is one such medicine. |
Watchful waiting
Sometimes healthcare professionals recommend against starting treatment right away, choosing instead to watch the condition closely with regular checkups. This watchful waiting approach may be an option when a paraganglioma grows slowly and causes no symptoms. [2]
Clinical trials
Clinical trials study new treatments and new ways of using older ones. If you are interested in trials for paraganglioma, talk with your healthcare professional about your options and weigh the benefits and risks together. [2]
Preparing for Your Appointment
Start by making an appointment if you have symptoms that worry you. If a paraganglioma is suspected, you may be referred to a specialist who treats hormone-related conditions — an endocrinologist. [2]
Before the appointment, ask if any preparation is needed (for example, stopping eating a certain number of hours before a test). Bring a list of your symptoms, key personal information (stresses, recent life changes, family medical history), and all medicines, vitamins, and supplements you take with their doses. If you can, bring a family member or friend who can help remember the information you receive. [2]
Helpful questions to ask include what is likely causing your symptoms and whether other causes are possible, what tests you need, whether the condition is likely short term or ongoing, what treatment is recommended and what alternatives exist, how to manage other health conditions together, whether any restrictions apply, and whether you should see a specialist. [2]
Expect your healthcare professional to ask when symptoms started, whether they are constant or come and go, how severe they are, what makes them better or worse, and whether you have a family history of paragangliomas or genetic conditions. [2]
Complications to Be Aware Of
While most paragangliomas are benign, serious problems can arise. Metastatic paraganglioma can spread to lymph nodes, lungs, liver, and bones. Untreated hormone-making tumors cause repeated blood pressure spikes and heart strain, and treatment itself can trigger dangerous catecholamine release if hormone levels are not controlled first. [1] [2] The importance of hormone control before treatment, along with the value of imaging follow-up and genetic counseling for families, is why ongoing care matters even for slow-growing tumors.
Conclusion and Next Steps
Paragangliomas are rare, and most are benign — but their hormone effects and location-related symptoms deserve real attention. If you have hard-to-control blood pressure, blood pressure that spikes, or clusters of symptoms such as a pounding heart, sweating, and headaches, talk with a healthcare professional. Diagnostic testing is straightforward, hormone control makes treatment safer, and surgery often resolves the tumor. And if a paraganglioma is found, genetic testing can protect you and your family. Do not wait out unexplained blood pressure spikes — an evaluation is the safest next step.
Frequently Asked Questions
What is a paraganglioma?
A paraganglioma is a rare tumor that grows from chromaffin cells, a type of nerve cell found throughout the body. It most often forms in the head, neck, abdomen, or pelvis and is usually benign (not cancer). [1]
Is a paraganglioma cancer?
Most often, no. Paragangliomas are usually benign, but sometimes they are cancerous and can spread — most often to nearby lymph nodes, and sometimes to the lungs, liver, and bones. [1]
Is a paraganglioma the same as a pheochromocytoma?
They are closely related. A pheochromocytoma is a tumor that starts in chromaffin cells in the adrenal glands (on top of the kidneys), while a paraganglioma forms in chromaffin cells elsewhere in the body. [1]
What symptoms should I watch for?
Head and neck tumors can cause a pulsing or whooshing sound in the ears, trouble swallowing, a hoarse voice, hearing loss, blurred vision, and dizziness. Tumors elsewhere often cause hormone symptoms: high blood pressure, a pounding heart, sweating, headaches, shaking, and sudden facial paleness. [1]
Why does my blood pressure spike sometimes?
Intermittent blood pressure spikes can be a sign of a hormone-making paraganglioma. If your blood pressure is hard to control or spikes when measured, talk with a healthcare professional about checking hormone levels. [1]
How is a paraganglioma diagnosed?
Diagnosis usually starts with blood and urine tests that measure catecholamine hormones or the protein chromogranin A, followed by imaging (MRI, CT, MIBG, or PET scans). Genetic testing is often recommended. [2]
Is paraganglioma inherited?
Sometimes. Some paragangliomas are caused by DNA changes passed from parents to children, and conditions such as MEN 2, Von Hippel-Lindau disease, neurofibromatosis 1, hereditary paraganglioma syndromes, and Carney-Stratakis dyad raise the risk. [1]
What treatments are available?
Treatment is most often surgery, usually after hormone-blocking medicines (alpha blockers, beta blockers, calcium channel blockers) bring symptoms under control. Radiation, stereotactic radiosurgery, ablation, chemotherapy, targeted therapy, and PRRT are options when surgery is not enough or the tumor has spread. Watchful waiting may suit slow-growing, symptom-free tumors. [2]
References
Paraganglioma — Symptoms & causes. https://www.mayoclinic.org/diseases-conditions/paraganglioma/symptoms-causes/syc-20575682
Paraganglioma — Diagnosis & treatment. https://www.mayoclinic.org/diseases-conditions/paraganglioma/diagnosis-treatment/drc-20575710
This article is for informational purposes only and is not a substitute for professional medical advice.
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