Juvenile Nasopharyngeal Angiofibroma

Updated: Feb 10, 2021
  • Author: Ted L Tewfik, MD; Chief Editor: Arlen D Meyers, MD, MBA  more...
  • Print

Practice Essentials

Juvenile nasopharyngeal angiofibroma (JNA) is a benign tumor that tends to bleed and occurs in the nasopharynx of prepubertal and adolescent males. [1, 2]

Workup in juvenile nasopharyngeal angiofibroma

Plain radiography views of the sinuses may demonstrate nasopharyngeal polyp. Bowing of the posterior wall of the maxillary sinus and maxillary sinus opacification is very suggestive of juvenile nasopharyngeal angiofibroma (JNA). Newer radiographic modalities have surpassed plain films in usefulness.

Magnetic resonance imaging (MRI) is indicated to delineate and define the extent of the tumor, especially in cases of intracranial involvement.

Bilateral vascular supply may be an underappreciated factor in JNA, and thorough radiographic investigation via angiography of bilateral carotid systems should be routinely performed preoperatively. [3]

Different staging systems exist for nasopharyngeal angiofibroma. The two most commonly used are those of Sessions and Fisch.

Surgical management of juvenile nasopharyngeal angiofibroma

A lateral rhinotomy, transpalatal, transmaxillary, or sphenoethmoidal route is used for small tumors (Fisch stage I or II), while the infratemporal fossa approach is used when the tumor has a large lateral extension.

The midfacial degloving approach, with or without a Le Fort osteotomy, improves posterior access to the tumor.

The facial translocation approach is combined with Weber-Ferguson incision and coronal extension for a frontotemporal craniotomy with midface osteotomies for access.

An extended anterior subcranial approach facilitates en bloc tumor removal, optic nerve decompression, and exposure of the cavernous sinus.

Some authors advocate the use of intranasal endoscopic surgery for lesions with limited extension to the infratemporal fossa.


History of the Procedure

Hippocrates described the tumor in the 5th century BC, but Friedberg first used the term angiofibroma in 1940. Other titles (eg, nasopharyngeal fibroma, bleeding fibroma of adolescence, fibroangioma) have also been used.

The image below depicts a coronal CT scan.

Coronal CT scan of the lesion filling the left nas Coronal CT scan of the lesion filling the left nasal cavity and ethmoid sinuses, blocking the maxillary sinus and deviating the nasal septum to the right side.



Juvenile nasopharyngeal angiofibroma (JNA) accounts for 0.05% of all head and neck tumors. A frequency of 1:5,000-1:60,000 in otolaryngology patients has been reported.


Juvenile nasopharyngeal angiofibroma (JNA) occurs exclusively in males. Females with juvenile nasopharyngeal angiofibroma (JNA) should undergo genetic testing.


Onset is most commonly in the second decade; the range is 7-19 years. Juvenile nasopharyngeal angiofibroma (JNA) is rare in patients older than 25 years.



The lesion originates in close proximity to the posterior attachment of the middle turbinate, near the superior border of the sphenopalatine foramen.

A hormonal theory has been suggested because of the lesion's occurrence in adolescent males. Moreover, a study by Liu et al found that JNAs express higher levels of hormone receptors and vascular endothelial growth factor (VEGF) than does normal nasal mucosa, a possible indication that interaction between the receptors and VEGF is associated with JNA initiation and growth. [4]

Other theories include a desmoplastic response of the nasopharyngeal periosteum or the embryonic fibrocartilage between the basiocciput and the basisphenoid.

Etiology from nonchromaffin paraganglionic cells of the terminal branches of the maxillary artery has also been suggested. Comparative genomic hybridization analysis of these tumors revealed deletions of chromosome 17, including regions for the tumor suppressor gene p53 as well as the Her-2/neu oncogene.



The tumor starts adjacent to the sphenopalatine foramen. Large tumors are frequently bilobed or dumbbell-shaped, with one portion of the tumor filling the nasopharynx and the other portion extending to the pterygopalatine fossa. [5]

Anterior growth occurs under the nasopharyngeal mucous membrane, displacing it anteriorly and inferiorly toward the postnasal space. Eventually, the nasal cavity is filled on one side, and the septum deviates to the other side. Superior growth is directed toward the sphenoid sinus, which may also be eroded. The cavernous sinus may become invaded if the tumor advances further.

Lateral spread is directed toward the pterygopalatine fossa, bowing the posterior wall of the maxillary sinus. Later, the infratemporal fossa is invaded. Occasionally, the greater wing of the sphenoid may be eroded, exposing the middle fossa dura. Proptosis and optic nerve atrophy result if orbital fissures are encroached upon by the tumor. Extranasopharyngeal angiofibroma is extremely rare and tends to occur in older patients, predominately in females, but the tumor is less vascular and less aggressive than juvenile nasopharyngeal angiofibroma (JNA).




See the list below:

  • Nasal obstruction (80-90%) - Most frequent symptom, especially in initial stages

  • Epistaxis (45-60%) - Mostly unilateral and recurrent; usually severe epistaxis that necessitates medical attention; diagnosis of angiofibroma in adolescent males to be ruled out

  • Headache (25%) - Especially if paranasal sinuses are blocked

  • Facial swelling (10-18%)

  • Other symptoms - Unilateral rhinorrhea, anosmia, hyposmia, rhinolalia, deafness, otalgia, swelling of the palate, deformity of the cheek


See the list below:

  • Nasal mass (80%)

  • Orbital mass (15%)

  • Proptosis (10-15%)

  • Other signs include serous otitis due to eustachian tube blockage, zygomatic swelling, and trismus that denote spread of the tumor to the infratemporal fossa, decreasing vision due to optic nerve tenting (rare)


See the list below: