Juvenile Nasopharyngeal Angiofibroma (JNA)

What is juvenile nasopharyngeal angiofibroma (JNA)?

A juvenile nasopharyngeal angiofibroma (JNA) is a rare, benign (noncancerous) tumor that grows behind your nose. It mainly consists of blood vessels. Though these tumors aren’t cancerous, they’re still serious and very aggressive. A JNA tumor can extend from your nasal cavity to your sinuses, eye socket, skull and brain.

Another name for this condition is juvenile angiofibroma (JA).

What age does juvenile nasopharyngeal angiofibroma occur?

The vast majority of JNA tumors grow in adolescent or prepubescent boys and people assigned male at birth (AMAB) between the ages of 10 and 20. Very rarely, it can appear in older men or boys as young as 7 years old. Nasopharyngeal angiofibromas almost never affect women or people assigned female at birth (AFAB).

How rare is a JNA tumor?

Nasopharyngeal angiofibroma is very rare. In the United States, there are only about 50 new cases each year.

What are the symptoms of a juvenile nasopharyngeal angiofibroma (JNA)?

In the early stages, symptoms include:

• Frequent or severe nosebleeds.
• Nasal congestion.
• Runny nose.

Symptoms get worse as the tumor grows. Advanced symptoms can include:

• Facial swelling, especially in your cheeks.
• Pain.
• Watery eyes.
• Droopy eyelids and bulging eyes.
• Headaches.
• Vision loss.
• Double vision.
• Hearing loss.
• Speech issues.
• Sleep apnea.
• Facial numbness.

What causes juvenile nasopharyngeal angiofibroma (JNA)?

Experts don’t fully understand what causes JNA. Because these tumors occur almost exclusively in people assigned male at birth, hormones likely play a role. Experts don’t believe JNA tumors are hereditary.

Juvenile nasopharyngeal angiofibroma risk factors

There aren’t a lot of clear-cut risk factors for JNA. Even though the condition isn’t hereditary, you’re more likely to develop JNA if any of your biological family members have familial adenomatous polyposis (FAP) — a rare disease where precancerous polyps form in your large intestine. Experts continue to explore this relationship.

What are the complications of juvenile nasopharyngeal angiofibroma?

Left untreated, nasopharyngeal angiofibroma can lead to:

• Difficulty breathing.
• Vision, hearing and speech issues.
• Facial abnormalities such as droopy eyelids or bulging eyes.
• Loss of sense of smell (anosmia).
• Severe nosebleeds and massive blood loss.
• Mental status change.

How is juvenile nasopharyngeal angiofibroma diagnosed?

A healthcare provider can diagnose JNA during an examination. After looking at your nose, they’ll ask about your symptoms. They may also refer you to an otolaryngologist (ENT) for further examination.

What tests can help diagnose juvenile nasopharyngeal angiofibroma?

If your healthcare provider suspects JNA, they’ll want to take imaging tests. These may include:

• CT (computed tomography) scans.
• MRI (magnetic resonance imaging).
• PET (positron emission tomography) scans.

They may also perform nasal endoscopy to look at the tumor. An endoscope is a long, flexible tube with a light and camera on the end. A provider will carefully insert the endoscope into your nose to see inside your nasal cavity. They’ll use a numbing spray, so you’ll feel pressure, but you shouldn’t feel pain.

How is juvenile nasopharyngeal angiofibroma treated?

Surgery is the preferred juvenile nasopharyngeal angiofibroma treatment. Some people also need radiation therapy.

Treatment depends on the size and location of the JNA tumor.

Juvenile nasopharyngeal angiofibroma surgery

The goal of surgery is to remove the entire JNA tumor. Depending on the tumor’s size and location, a surgeon may be able to remove it through your nose (endoscopic surgery). An otolaryngologist will perform this surgery in a hospital setting.

Radiation therapy for JNA

In some cases, surgeons can’t remove the entire tumor due to its location. If it’s not possible to remove the tumor — or if it grows back after surgery — your healthcare provider may recommend repeat surgery or radiation therapy. This treatment uses high-energy X-ray beams to shrink the tumor.

Can I prevent juvenile nasopharyngeal angiofibroma?

There’s no known way to prevent JNA tumors.

What can I expect if I or someone I love has JNA?

If you or someone you know has JNA, prompt treatment is key. Your healthcare provider will determine an appropriate treatment plan based on your unique situation.

What is the prognosis for JNA?

The outlook for people with juvenile nasopharyngeal angiofibroma depends on the size and location of the tumor. In most cases, a healthcare provider can remove the tumor or shrink it with radiation therapy. After treatment, your provider will schedule regular imaging tests to check for signs of recurrence (regrowth).

In up to 50% of cases, JNA tumors regrow after surgical removal. Regrowth usually occurs within two years after surgery, usually because a piece of the tumor still remains. If the tumor starts to grow again, your provider may schedule another surgery or recommend radiation treatments to shrink it.

When should I call my healthcare provider?

You should schedule an appointment with your healthcare provider if you develop:

• Frequent nosebleeds.
• Difficulty breathing.
• Persistent nasal congestion.
• Runny nose.

What questions should I ask my healthcare provider?

If you or your child received a JNA diagnosis, here are some questions you may want to ask at your next visit:

• What’s the exact location of the tumor?
• How big is it?
• Do you think you can remove it with surgery?
• Will I need radiation therapy?
• What are the chances that the tumor will come back?
• How often should I have imaging tests?

A note from Cleveland Clinic

A JNA diagnosis can feel scary and overwhelming. Your healthcare provider is here to help. Even though JNA tumors aren’t cancerous, they can spread to surrounding areas like your sinuses, skull and brain, resulting in a wide range of complications. Surgery is the go-to treatment for JNA tumors, but some people need radiation treatment, too. Talk to your healthcare provider about additional resources, including local or online support groups that can help you during this challenging time.