I would like any information you can provide for a condition called juvenile angiofibroma of the sinuses. My son has been diagnosed for a second time with this condition. As you may well understand, we are quite concerned. Is there anything that contributes to this condition? Are there any avenues we should be exploring to prevent another occurrence? He is now 18 years old and getting ready for college. Should we consider keeping him close by for the next four years or can he go to a school 10 hours away?

I.M.

Juvenile nasopharyngeal angiofibroma (JNA) is a benign tumor (that is, not a cancer) which, nevertheless, can cause big problems. That's because JNAs bleed readily and occur in an area where surgical access is difficult. Also, JNAs frequently grow into adjacent areas: they may extend into the sinuses, the orbit (the bony chamber that surrounds the eye) and the cranium (the bony chamber that surrounds the brain). Complete surgical removal of a JNA that has grown in this fashion can be very challenging. Radiation therapy is an option for patients with such extensive tumors.
Just a word about terminology: Juvenile refers to the fact that these tumors are often diagnosed in boys before puberty. (JNAs occur exclusively in boys; a diagnosis of JNA in a girl would be highly suspect.) Nasopharyngeal refers to the site of origin of these tumors. The nasopharynx is the "top of the throat," essentially the junction between the nasal cavity and the pharynx (throat). It is located above and behind the soft palate. Finally, angiofibroma refers to the appearance of these tumors under a microscope: they are composed of fibrous tissue and vascular tissue. ("Angio" means "vessel.")

Boys with JNA develop constant nasal obstruction which may, initially, be one-sided. Frequent, severe nosebleeds are also common. As the tumor grows, the child's voice will change due to the loss of resonance from the nasal cavity. (The same effect is seen in children with large adenoids.) Very large tumors may cause deformity of the palate, cheek or eye. An MRI scan is extremely helpful for establishing the correct diagnosis.

As far as I know, there are no predisposing factors for JNA other than gender and nationality. JNAs are most commonly reported in India and Egypt. In the United States, JNAs appear to be more common in Hispanic than in non-Hispanic populations.

In answer to your second question, I think you need to distinguish between "recurrent" and "residual" tumor. If a tumor is completely excised by previous surgery but returns months or years later, this is a "recurrence." On the other hand, with JNA, it is not uncommon for residual tumor to be left behind after surgery. This is usually not the surgeon's fault, by the way; JNAs get their fingers into everything, and complete surgical excision can, at times, be impractical. Indeed, radical removal of JNA would necessitate quite a bit of injury to the patient. Let's say, for example, that the tumor had invaded the orbit and was firmly entrenched in the tissues surrounding the eye. If JNA were a cancer, radical removal (in this example) would require removal of the eye. But JNA is not a cancer. Most surgeons would not remove a healthy eye to treat a benign tumor.

Do you need to keep your son close to home? The answer to this question depends a great deal on the severity of his problem and his access to medical care at the college of his choice. In my opinion, JNAs are best treated at tertiary referral centers -- in other words, major medical centers that receive "tough cases" from their region. (The Mayo Clinic is arguably the best-known example of a tertiary referral center.) If your son is going to a university with an excellent medical center, he may be better off there than at home. This decision is probably best made in consultation with your son's doctor, who will be most familiar with the seriousness of your son's condition and with the quality of medical care at or near the college your son is considering attending.