What is a paraganglioma?
A paraganglioma—also called a “para,” or a PG—is a type of neuroendocrine tumor that forms near clusters of nerve cells called ganglia. Although they’re frequently noncancerous, they can still cause problems. They’re found more frequently in people with genetic mutations usually passed on by parents.
At Penn Medicine, our expert endocrinology and neurosurgery specialists have years of experience with these rare neuroendocrine tumors. We can find answers, protect your health, and get you the individualized care you need. While many people have successful paraganglioma treatment with surgery, we offer a range of additional options for those who need more.
Understanding paragangliomas and related tumors
Many paragangliomas involve nerve cells called chromaffin cells—the same cells that can give rise to a related tumor called a pheochromocytoma. There are differences between the two types of chromaffin-related tumors:
- Paragangliomas can occur in any area where there are ganglia or nerve endings for the sympathetic or parasympathetic (more common) nervous systems. They’re usually found in the abdomen, pelvis, or head and neck (head and neck PGLs). Parasympathetic paras are much less likely to secrete (release) hormones than sympathetic tumors.
- Pheochromocytomas are found in the adrenal gland. Pheochromocytomas arise from sympathetic ganglia and usually release hormones related to the fight or flight response.
Other paragangliomas form in related cells called glomus cells, part of the parasympathetic nervous system. These tumors grow in the chest, neck, and at the bottom of the skull.
Most paragangliomas are slow-growing, benign (noncancerous), and confined to one spot when they’re found. However, even benign paragangliomas can pose a risk. If the tumors release hormones, for example, effects may include high blood pressure and other heart health problems.
Up to 25 percent of paragangliomas can be cancerous and eventually spread (metastasize), typically to the lymph nodes, lungs, liver, and bones. Metastatic paraganglioma may run in families and is often less aggressive than other metastatic cancers. Treatments are available to manage the tumor and control symptoms. People can live for decades with metastatic paraganglioma with a good quality of life.
Paraganglioma symptoms
Paragangliomas often get called the “great mimics” or “great masqueraders” because they can share symptoms with many other conditions.
Paragangliomas in the head and neck can push on nerves and other structures, causing a wide variety of potential symptoms. Symptoms depend on the location and may include:
- Hearing loss
- Pain
- Swallowing issues
- Ringing in the ears
Elsewhere in the body, symptoms vary. Sometimes, they’re subtle. But many of these tumors cause a noticeable, characteristic set of signs that may come and go. Episodes typically last less than an hour, but may continue for several days. The experience may happen a couple of times a week or just a few times a month.
Classic signs are:
- Anxiety, nervousness, or panic attacks
- Headaches
- Heart palpitations, marked by the heart pounding, fluttering, or beating rapidly
- Hypertension (high blood pressure), though levels may rise and fall and sometimes even drop too low
- Sweating
These symptoms overlap with other conditions, but when they occur together, they can indicate paraganglioma or pheochromocytoma. Paragangliomas cause these symptoms by boosting the production and release of hormones called catecholamines. Catecholamines, which include epinephrine (adrenaline), norepinephrine (noradrenaline), and dopamine, are neurotransmitters. They bring instructions from nerves to other parts of the body.
Because some paragangliomas produce hormones or press on nearby structures, they can cause a range of less common symptoms, including:
- Dizziness
- Dyspnea, or difficulty breathing
- Fatigue
- Flushing of the skin
- Hyperglycemia (high blood sugar)
- Nausea
- Pain in the back or belly
- Pale appearance
- Vomiting
These symptoms can vary widely and may come and go. If you notice persistent or unexplained symptoms, it’s important to talk with your care team.
Diagnosing paraganglioma
Sometimes paraganglioma is found during a scan done for another reason. In other cases, people come to us because of symptoms or known genetic variants in their families, or to confirm a previous diagnosis. Most people are diagnosed between ages 20 and 50.
If our team suspects paraganglioma, we recommend genetic testing to identify any related variants and help guide next steps. We also review your family history for related tumors or inherited conditions, use blood or urine tests to look for hormone-related markers, and perform imaging scans to locate tumors and understand how they are behaving.
Expert care from many angles
With a range of experts thoroughly reviewing cases together, our team ensures you receive the treatment recommendations most likely to help. We consider not just the immediate problem, but the months and years ahead. We always prioritize your preferences and quality of life, balancing treatment benefits with potential side effects.
For paragangliomas that release adrenaline or noradrenaline, we use medications to block the catecholamine hormones before any medical procedure. That step prevents blood pressure surges and instability during procedures, making the treatment safer.
Our team has deep experience with these medications, having successfully used them on more than 400 people — one of the nation’s highest volumes. We tailor the medication choice and the dose for each case. While most people only need the blockers short-term, those with metastatic disease or more symptoms may need them longer.
We always consider surgery as a first step in paraganglioma treatment. Outside the head and neck, we can often remove these tumors, relieving symptoms and potentially providing a cure. Even when paragangliomas spread or return, surgery can still be effective.
Our expert surgeons often take a minimally invasive approach to removing paragangliomas, using only small incisions. Larger tumors may need an open operation. We may also turn to radiation therapy after surgery to destroy any remaining cancer cells.
Paragangliomas in the head and neck can be more challenging, as they often sit close to sensitive nerves or blood vessels. Depending on the size and location of the tumor, we may recommend limited surgery followed by radiation therapy, or radiation alone. Radiation can stop the tumor from growing and eventually shrink it.
If we do perform head and neck surgery, Penn therapists help with any needed care to restore hearing, speaking, or swallowing.
When surgery is not enough or not possible, we may recommend pausing treatment, at least temporarily.
Because paraganglioma is a slow-growing disease, even metastatic tumors may not grow much (or at all) in a few months or even a year. A “watchful waiting” approach allows us to save treatment options for a later date and avoid side effects. We still monitor with scans, blood tests, and urine tests and manage any hormone-related symptoms.
For widespread disease or tumors causing more symptoms, the choice for further treatment depends on how widespread it has become:
Ablation and radiation therapy: We can use these focused therapies to treat metastatic growths. Ablation can destroy cancerous tissue with heat or cold, while radiation therapy can stop tumor growth.
Systemic therapies: Treatments that target cancer cells throughout the body can be effective against paraganglioma. Our team uses the most current data and our extensive experience when recommending systemic therapies. We have multiple options, so if a treatment stops working, we can switch to another therapy. We may also have clinical trials available. Systemic therapies include:
- Drug therapies: Injectable drugs that mimic hormones can relieve symptoms. Chemotherapy can also keep cancer in check. We offer newer, pill-based regimens that can be easier to take. Targeted therapy, a newer approach under development, aims at the ways cancer grows.
- Nuclear medicine: We pair controlled radioactive particles with substances that certain paragangliomas take into their cells. Our program led the national trial for Azedra, the only approved nuclear medicine therapy for paraganglioma. Other options are under study.
People with paragangliomas require lifelong checkups with scans and blood or urine tests, even after successful surgery. Our team creates a personalized plan based on your circumstances.
The type of monitoring that’s right for you is based on factors such as whether you have an inherited genetic variant (and which one). Many people with variants need a full-body, rapid MRI, a method we developed in our program.
Recognized expertise in rare neuroendocrine tumors
Paragangliomas and pheochromocytomas can be challenging, so you need a team of experts with experience diagnosing and treating these tumors. We run a focused program within Penn’s Neuroendocrine Tumor Program, caring for more than 200 people with these rare tumors each year.
When you come to our program, you’ll find:
- Coordination: Our clinicians represent a range of specialties working closely together. Many serve on a weekly neuroendocrine tumor board. For paragangliomas in the head and neck, they’re joined by specialized oncologists, ENT surgeons, and neurosurgeons.
- Expertise: All of our specialists have deep experience with paragangliomas. Many of our providers publish leading papers and give national talks, including for genetics, nuclear medicine, and symptom control.
- Innovation: We offer clinical trials to improve care and develop new treatments. We also run a research effort to secure and analyze tumor samples to answer questions about paragangliomas and find new treatments. Learn more about neuroendocrine tumor research and clinical trials.
- Center of Excellence: We hold a Center of Excellence designation from the Pheo Para Alliance, the leading advocacy and research organization for paragangliomas and pheochromocytomas. It recognizes our personalized care, range of specialists, and research projects. We’re one of only nine such centers in the world, seven in the U.S., and the only program east of the Appalachians.
- Support: We try to make your experience as easy and stress-free as possible. A nurse coordinator oversees records, scheduling, and many other needs. Throughout your treatment, we offer a range of additional support for neuroendocrine tumors.
Rated “exceptional” by The National Cancer Institute
Penn Medicine’s Abramson Cancer Center is a world leader in cancer research, patient care, and education. Our status as a national leader in cancer care is reflected in our continuous designation as a Comprehensive Cancer Center by the National Cancer Institute (NCI) since 1973, one of 7 such centers in the United States. The ACC is also a member of the National Comprehensive Cancer Network, one of a select few cancer centers in the U.S., that are working to promote equitable access to high-quality, advanced cancer care.