Juvenile Nasopharyngeal Angiofibroma (JNA)

Juvenile nasopharyngeal angiofibroma (JNA), while rare, is the most common benign tumor of the space at the back of the nasal cavity that connects the nose to the mouth. This space is called the nasopharynx.

These growths are noncancerous, but are still serious as they can bleed profusely, spread and damage nerves and bones and block ear and sinus drainage. Over time, the tumor can invade the eye socket or cranial cavity, causing blindness or other significant neurologic problems.

How juvenile nasopharyngeal angiofibromas develop is not exactly known. However, because these tumors occur almost exclusively in adolescent boys, hormones are thought to play a role.  The site of origin is theorized to be in the region of a tiny area along the side of the nasal cavity called the the sphenopalatine foramen. JNAs extend out from here into the space behind the maxillary sinus (the large cavity in the cheek) and into the nasopharynx, sphenoid sinus and other areas of the back of the nasal cavity. There is no evidence they are hereditary. JNA is extremely rare, with an incident rate of one occurrence in 150,000 individuals.

Initially, when the growth is small, a child with a JNA may have:

• trouble breathing through one side of the nose
• a lot of nosebleeds, usually with blood coming from only one nostril
• a runny nose on one side that doesn't go away after several days

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

• headaches or pain in the upper areas of the face (sinus pain)
• trouble smelling odors
• trouble hearing
• change in facial appearance (swelling in the cheeks, mouth or face)
• complete obstruction of both sides of the nose
• persistent discharge from both sides of the nose
• trouble seeing or visual disturbance
• droopy eyelids and bulging eye(s)
• sleep apnea
• facial numbness

A primary care pediatrician will ask about a child’s JNA symptoms — such as trouble breathing through the nose, number of nose bleeds and so forth — and do an exam. Sometimes the doctor can see the growth when looking in the nose or mouth, but most are only visible by using a tiny camera called an endoscope to check inside the nose.

If the primary care doctor suspects a JNA, they will send the child to an ear, nose and throat (ENT, also called otolaryngology) specialist for confirmation of the diagnosis and treatment.

To determine the exact location of the tumor and see how far it has spread, the otolaryngologist will do an endoscopy and order other tests such as:

• MRI (magnetic resonance imaging)
• CT or CAT (computerized tomography) scan
• X-rays that show blood vessels in and around the tumor (angiography)

Because JNAs involve blood vessels (vascular), it is not recommended to biopsy these lesions.  Their appearance on examination and imaging is usually enough to give the diagnosis.

Surgery to remove the growth is the primary treatment for JNA. At Children’s Hospital of Philadelphia (CHOP), patients are treated by our Head and Neck Disorders Program, which has highly skilled surgeons who have expertise in this delicate surgery.

These tumors have a large blood supply and many blood vessels, which could cause significant bleeding during surgery. To reduce the amount of blood loss at the time of surgery, the child may undergo a procedure called angiography with embolization the day before surgery. A thin tube (catheter) is inserted into the blood vessels near the growth and, either with a coil, small beads or medicine to induce a clot, the vessels are blocked to reduce the flow of blood to the JNA.

Most JNAs can be removed using endoscopic surgical techniques — entering the nasopharynx with small instruments through the nostrils. Using endoscopy reduces discomfort, shortens healing times and does not leave visible scars.

Additional approaches may be necessary for larger JNAs, including an incision under the lip to enter the maxillary sinus and provide access to the JNA in the space behind the maxillary sinus. For extensive JN’s, an open approach may be required, such as an incision on the face to allow removal of the cheek bone. That may need to be combined with an incision across the scalp to allow access to a portion of the JNA that may be extending up toward or pushing on the brain.

Because JNA tumors have the potential to recur after surgical removal, patients will need regular nasal endoscopic examination and imaging studies into adulthood to check if the tumor has regrown. If the tumor returns and is getting larger, it may need to be surgically removed.
Only in rare, very advanced or aggressive cases is radiation therapy considered for initial treatment or following surgical debulking. Radiation targets the tumor with high-energy beams that are meant to shrink the JNA.

In about half of children with JNA, the tumor will regrow after being removed. Regrowth usually occurs within two years following surgery, most often because surgeons were unable to remove a piece of the tumor because of its location. That is why regular follow-up imaging is important. The Head and Neck Disorders Program at CHOP will continue to follow children with JNA into young adulthood.