Paraganglioma

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.

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.