Nasopharyngeal Angiofibroma

• Angiofibroma of the Nasopharynx:
  • Juvenile nasopharyngeal angiofibroma (JNA) occurs:
    • In male teenagers
    • Is a benign but locally aggressive tumor.
  • The tumor contains:
    • Both vascular and fibrous elements:
      • Intermingling together

• Epidemiology:
  • Juvenile nasopharyngeal angiofibromas occur:
    • Almost exclusively in males:
      • Usually in adolescence:
        • Approximately 15 years
  • They account for only 0.5% of all head and neck tumors:
    • But are the most common of the benign nasopharyngeal neoplasms

• The patient presents with:
  • Nasal obstruction and
  • Epistaxis:
    • One symptom usually predominates:
      • This depends on the proportion of either of the two elements present:
        • Recurrent epistaxis:
          • Which can be severe:
            • Is usually the presenting symptom
• The origin of JNA:
  • Is the posterolateral aspect of the roof of the nasal cavity:
    • In the region of the sphenopalatine foramen
  • When the tumor increases in size:
    • It may extend into:
      • The pterygopalatine fossa and then posteromedially into the nasopharynx or it may enlarge laterally into the infratemporal fossa
    • Other routes of expansion include:
      • Superiorly:
        • Eroding the sphenoid sinus
      • Anteriorly:
        • Into the maxillary sinus.
        • Growth into the orbit through the inferior orbital fissure:
          • Will lead to proptosis
          • In some cases extension superiorly into the middle cranial fossa
• On macroscopic examination:
  • The angiofibroma is:
    • Lobulated in appearance
  • Its consistency ranges from:
    • Spongy to a varying degree of firmness:
      • Depending on the proportion of:
        • Vascular tissue and fibrous component that forms the tumor
• Microscopically:
  • Tumor is uncapsulated
  • Formed by numerous blood vessels of varying calibre coursing through a fibrous tissue stroma
  • The thickness of the muscular coat of these vessels varies and:
    • In general elastic fibres in these vessels are lacking:
      • Thus the ability to retract is reduced:
        • This lack of contractile tissue is the pathological reason:
          • For frequent episodes of epistaxis once a minor vessel starts to bleed
• These clinical features of repeated epistaxis and nasal obstruction in a male adolescent:
  • Together with the finding of a vascular mass in the nasopharynx:
    • Clinches the diagnosis
• Imaging studies are required:
  • To confirm the diagnosis and to assess the extent of the tumor
  • Plain radiographs:
    • No longer play a role in the workup of a suspected juvenile nasopharyngeal angiofibroma:
      • However:
        • They may still be obtained in some instances during the assessment of nasal obstruction, or symptoms of sinus obstructions
    • Findings include:
      • Visualization of a nasopharyngeal mass
      • Opacification of the sphenoid sinus
      • Anterior bowing of the posterior wall of the maxillary antrum:
        • Holman-Miller sign:
          • Pathognomonic
      • Widening of the pterygomaxillary fissure and pterygopalatine fossa
      • Erosion of the base medial pterygoid plate
• Angiography reveals:
  • Typical vascular tumor blush with multiple sources of blood supply and for large tumors bilateral supply is not uncommon:
    • The internal maxillary artery and its branches are usually the principal feeder

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