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

#Arrangoiz #HeadandNeckSurgeon #HeadandNeckTumors #CancerSurgeon #Teacher

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