Juvenile Nasopharyngeal Angiofibroma

What Is a Juvenile Nasopharyngeal Angiofibroma?

A juvenile nasopharyngeal angiofibroma (JNA) is a growth in the area behind the nose. These growths are benign (not cancer), but can damage nerves and bones and block ear and sinus drainage. Almost all JNAs are in teenage boys.

What Are the Signs & Symptoms of a Juvenile Nasopharyngeal Angiofibroma?

Juvenile nasopharyngeal angiofibromas cause symptoms only when they're big enough to keep air from passing through the nose or press on parts of the nose and sinuses.

A child with a JNA may have:

• trouble breathing through the nose
• a lot of nosebleeds, often with blood coming from only one side

As the growth gets bigger, a child also may have:

• headaches
• pain in the upper areas of the face (sinus pain)
• swelling in the cheeks, mouth, or face
• trouble smelling odors
• trouble hearing
• vision changes

What Causes a Juvenile Nasopharyngeal Angiofibroma?

Doctors don't know for sure what causes a juvenile nasopharyngeal angiofibroma (nay-zoe-fuh-RIN-jee-uhl an-jee-oh-fye-BROH-muh). But many think the cells that form the growths are left over from early pregnancy.

How Is a Juvenile Nasopharyngeal Angiofibroma Diagnosed?

Doctors will ask a child about JNA symptoms, such as trouble breathing through the nose, and do an exam. Sometimes the doctor can see the growth when looking in the nose or mouth. But most are only visible when the doctor uses a tiny camera (endoscope) to check inside the nose.

The doctor will send the child to an ear, nose and throat (ENT, also called otolaryngology) specialist for diagnosis and treatment.

Many kinds of masses can grow behind the nose. So the ENT doctor will do the endoscopy and other tests such as:

• MRI (magnetic resonance imaging)
• CT or CAT scan (computerized tomography)
• X-rays that show blood vessels in and around the tumor (angiography)
• sometimes, a biopsy (taking a sample of the growth for testing)

How Is a Juvenile Nasopharyngeal Angiofibroma Treated?

A juvenile nasopharyngeal angiofibroma may be hard to treat if it grows into the bone separating the back of the nose from the brain and into the sinuses or eye sockets (orbits).

A team of specialists works together to treat the tumor. The team may include experts in:

• blood diseases and cancer (hematology/oncology)
• brain and nerve surgery (neurosurgery)
• ear, nose, and throat (ENT) surgery (otolaryngology)
• eye surgery (ophthalmology)
• image-guided treatment (interventional radiology)
• microscopic study of tissue samples (pathology)

Surgery is usually the first choice for treatment. Sometimes, image-guided treatment is recommended before surgery to shrink the growth and decrease bleeding during surgery. In most cases, surgery is done without any external incisions, using a camera on a thin tube passed into the nose (endoscopic surgery).

What Else Should I Know?

Surgeons might not be able to remove all of a juvenile nasopharyngeal angiofibroma that's grown into the sinuses or the bones in the back of the nose. If this happens, the JNA might grow back after surgery. Sometimes doctors use radiation therapy or medicine to slow this growth.

A JNA may grow well into adulthood, so patients need to have imaging studies (CT or MRI) repeated over time to look for regrowth. Patients will need follow-up with the care team specialists into early adulthood.

JNAs are almost always found in teenage boys, so scientists are looking for ways to treat the growths with hormones, hormone blockers, or other medicines.