Juvenile Nasopharyngeal Angiofibroma | Diagnosis & Treatments

How do we diagnose JNA?

There are several diagnostic procedures that may be used to determine whether your child has JNA. In addition to a complete medical history and physical examination, these may include:

  • Endoscopic evaluation: Physicians will use an endoscopic (a flexible or rigid telescope like device, often attached to a camera, inserted into the nose) to determine the presence of the JNA and its degree of nasal airway obstruction. This is typically performed in the Otolaryngology clinic.
  • Magnetic Resonance Imaging (MRI): a diagnostic imaging exam involving a large magnet, radio waves, and a computer to produce two- and three-dimensional images of predominantly the body's soft tissue organs.
  • Computerized tomography scan (also called CT or CAT scan): This imaging technique uses a combination of x-rays and computer technology to produce cross-sectional images (often called slices), both horizontally and vertically. CT scans provide greater detail of the body’s bony structures.
  • Pathology: Usually the combination of endoscopic evaluation, CT scan, and MRI is sufficient to make the diagnosis of JNA. Rarely, your child’s doctors may also need to obtain a biopsy to confirm the diagnosis.

After the necessary tests are completed, our experts meet to review and discuss what they have learned about your child's condition. They then meet with you to discuss the results and outline the best treatment options for your child.

How we treat juvenile nasopharyngeal angiofibroma

First-line therapy is usually surgery, with the goal of removing as much of the tumor as possible. Ideally the entire tumor is removed. In some situations, depending on where the tumor has spread, complete removal may not be possible.

The initial treatment of JNA has two components:

  • Angiographic embolization by interventional radiologists. This is used immediately before surgery to close off the blood vessels that supply the tumor in order to limit blood loss during surgery.
  • Surgery to remove the tumor. This surgery is often done endoscopically — minimally invasive surgery performed through the nose guided by a special telescope (endoscope) and your child’s pre-operative imaging studies. If the tumor is localized (not spread far beyond the nasal cavity), this can be accomplished in one surgery. However, if the tumor has expanded significantly, multiple surgeries may be required. If it has impacted the brain, neurosurgeons will also be involved. Surgery for JNA is very complex due to where the tumor is located, and it should be done by a surgeon with specific expertise in this type of surgery.

Sometimes surgeons will not be able to remove the entire tumor, such as when the tumor has expanded into the cranial cavity and is impacting the brain or carotid artery. In such instances, remnants (pieces) of tumor tissue will remain. Because it’s possible for these tumor remnants to regrow, your treatment team will discuss additional therapies to treat this residual disease with you and your child.

This additional therapy may include angiogenesis inhibitors, medications that inhibit the growth of new blood vessels. Sometimes, these medications can shrink the JNA tumor sufficiently to allow surgeons to further resect (surgically remove) the tumor. Other times, the patient will need to continue to take the medications until the disease resolves in early adulthood.

Radiation therapy is very rarely considered, as radiation, particularly so close to the brain, can have long term side effects. Medications can often control the disease with fewer side effects than radiation.

Follow-up treatment

Even if the patient’s tumor is fully removed during surgery, there is the possibility a new tumor may reoccur. For this reason, patients with JNA will need to have annual follow-up appointments, during which doctors will perform endoscopic examinations and take new imaging scans to monitor for possible recurrence.

If the tumor cannot be fully resected and adjuvant therapy is required, more frequent visits with a hematologist/oncologist will be necessary to monitor blood work, assess for side effects, and adjust medication dosages as needed. These visits will be in addition to the endoscopic examinations and periodic imaging studies mentioned above.

If your child is not able to return to Boston for follow-up care, our doctors can partner with physicians in your local area for such monitoring.

Long term outlook

Although JNA is a complex condition, the long term outcome is usually very positive. There could be side effects from surgical or medical treatment, but these are minimized by an experienced multidisciplinary team. JNA should not ever be fatal.