Juvenile nasopharyngeal angiofibroma (JNA) is one of the most common benign nasal cavity tumors of adolescence. It often acts in a malignant manner by eroding into the surrounding sinuses, orbit, or cranial vault.
History of the Procedure
Although Hippocrates described lesions similar to JNAs, Chelius associated the tumor with adolescent males in 1847. A review by Martin et al in 1948 defined most of the associated features described below. The first successful surgical resection of a probable JNA is credited to Liston in 1841 at University College Hospital in London.
It is found almost exclusively in adolescent males (average age, 14-18 y). Young females given this diagnosis should undergo chromosomal studies or should have the diagnosis questioned. Incidence of JNA is 1 case per 5000-60,000 ear, nose, and throat patients and accounts for 0.5% of all head and neck tumors. The wide range of reported cases may stem from misdiagnosis and inclusion of other lesions. Incidence is reported to be higher in Egypt and India.
The cause has not been determined. The most accepted theory is that JNAs originate from sex steroid–stimulated hamartomatous tissue located in the turbinate cartilage. The proposed hormonal influence may explain why (rarely) some JNAs involute after puberty. Recent studies by Bretani demonstrate estrogen and progesterone receptors in JNA, but gonadotropin levels in all patients are normal. Another proposed theory includes tumor originating from embryonal chondrocartilage of the occipital plate.
The proposed origin of the JNA is located along the posterior-lateral wall in the roof of the nasopharynx, usually in the region of the superior margin of the sphenopalatine foramen and the posterior aspect of the middle turbinate. Fetal histology confirms large areas of endothelial tissue in this region. Rather than invading surrounding tissue, this tumor displaces and distorts, relying on pressure necrosis to destroy and push through its bony confines. Intracranial extension is noted in 10-20% of cases.
Signs and symptoms are present for an average of 6 months prior to the diagnosis, commonly with extension beyond the nasopharynx.
Frequent epistaxis or blood-tinged nasal discharge
Nasal obstruction and rhinorrhea
Conductive hearing loss from eustachian-tube obstruction
Diplopia, which occurs secondary to erosion into the cranial cavity and pressure on the optic chiasm
Rarely anosmia, recurrent otitis media, and eye pain
Visible firm grayish-red mass in the posterior nasal pharynx; nonencapsulated and often lobulated; can be sessile or pedunculated
Proptosis, a bulging palate, an intraoral buccal mucosa mass, cheek mass, or a swelling over the zygoma (common with local extension)
Symptoms of invasion to surrounding tissues, chronic nosebleeds, and nasal obstruction portend the need for prompt surgical intervention. Enlargement of a known JNA also may indicate the need for surgical intervention.
From its origin along the posterior middle turbinate, the JNA extends inferiorly, displacing the soft palate, eroding into the hard palate, and medially displacing the nasal septum. Filling the nasal cavity, it extends anteriorly through the posterior wall of the maxillary sinus, laterally through the pterygomaxillary fissure into the temporal and infratemporal fossa, and superiorly into the orbit and cranial cavity. JNAs most commonly are fed by the internal maxillary artery; however, with growth of the tumor, surrounding vessels may be parasitized.
Surgical contraindications include extension into or involvement of unresectable intracranial contents. Involvement of the lateral wall of the cavernous sinus is considered to be resectable in specific cases.