Juvenile nasopharyngeal angiofibroma
Juvenile Nasopharyngeal Angiofibroma (JNA) is a relatively rare tumor of the nasopharynx, comprising only 0.5% of all head and neck tumors. These tumors are benign, however they are locally invasive. In the general population, JNA occurs at an incidence of roughly 1:150,000, however JNA predominately appears in males age 14-25. Occasional cases of JNA in adolescent girls and men older than 25 have been reported, though these are rare. Some patients appear to develop JNA as a manifestation of Familial Adenomatous Polyposis syndrome. These tumors most commonly begin in the upper posterior part of the nasal cavity, but may invade surrounding structures as they grow.
These tumors rarely cause symptoms until they have invaded surrounding structures. Approximately 91% of patients with JNA present with nasal obstruction in one nostril. Recurrent epistaxis (bleeding from the nose) is also common, occurring in 63% of patients. Other signs and symptoms include: nasal discharge, pain, sinusitis, facial deformity, hearing impairment, otitis media (middle ear infections and inflammation), proptosis (bulging of the eyes out of the orbit), and diplopia (double vision). Often, the patient will have the symptoms for about 6 months to a year before being diagnosed with JNA.
CT and MRI scans are both important in evaluation and management of JNA. CT provides information about the bones of the sinus cavity surrounding the tumor, while MRI is useful for understanding the tumor itself and surrounding important structures such as arteries and nerves.
JNA may be followed by imaging the patient over time. In some cases, no intervention may be indicated, as these tumors may decrease in size on their own. In cases of continually growing tumors, tumors that are associated with significant symptoms, or tumors that invade critical structures, treatment may be indicated. Surgical resection is the treatment of choice for JNA. Surgery may be performed using 'open' techniques, which involve making incisions on the face or in the mouth to gain a wide route of access to the lesion. However, newer techniques rely on the use of endoscopic approaches. Endoscopic surgery involves using long cameras and instruments to operate through the notrils with no outer incisions.
Additional treatment tools include embolization, chemotherapy, and radiation. Embolization is a technique where a small catheter is guided inside the blood vessel to the blood vessel supplying the tumor. Liquid 'glue-like' substances are injected to block off the blood supply to the tumor. This can make surgery to remove it shorter and safer. Chemotherapy is useful as a secondary tool for the treatment of residual or recurrent disease. Radiation therapy may be successful in controlling JNA growth in up to 85-91% of cases. However, especially since these tumors are most typical in younger patients (in whom radiation should be avoided when possible), surgery remains the treatment of choice.
Julie Kretzer and Justin F. Fraser, MD
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