FEBRUARY 22nd, 2023


Juvenile Nasopharyngeal Angiofibroma: Most common benign vascular tumor in the nasopharynx

  • Presenting Symptoms: Recurrent (mostly) unilateral epistaxis, rhinorrhea, nasal obstruction, decreased taste/smell, headache, facial swelling, proptosis

  • Epidemiology: Adolescent, M>>F

  • Etiology: Largely unknown, though there is a theorized relation to adolescent male hormonal changes

  • Pathophysiology: Benign, slow-growing vascular tumor originating in pterygopalatine fossa that may invade locally; possible intracranial spread 

  • Diagnosis: 

    • Imaging (CT/CTA or MRI/MRA) of paranasal sinuses; AVOID BIOPSY (risk of bleed)

    • Fisch’s Classification: 

      • I: limited to nasal cavity

      • II: extends into pterygomaxillary fossa or sinuses with bony destruction present

      • III: invades infratemporal fossa, orbit, or parasellar area

      • IV: extends into cavernous sinus, optic chiasma, or pituitary fossa

  • Treatment: 

    • Surgery: FESS preferred to open approach; consider pre-op embolization; high risk of recurrence if not completely resected

    • Radiation and chemotherapy: increased risk of complications; used in unresectable tumors or non-surgical candidates or failed surgery

    • Hormone therapy: estrogen, flutamide to reduce tumor size, not routinely used 


Endoscopy

Kirkham, E., Manning, S., Moe, K.S. (2018). Juvenile Nasopharyngeal Angiofibroma. In: Perkins, J., Balakrishnan, K. (eds) Evidence-Based Management of Head and Neck Vascular Anomalies. Springer, Cham.

 

Axial CT

Widened sphenopalatine foramen + bowing of posterior maxillary wall (Holman-Miller sign)

Anil K. Lalwani: Current Diagnosis & Treatment Otolaryngology—Head and Neck Surgery, Fourth Edition Copyright McGraw-Hill Education. All rights reserved.

 

Further Reading:
General
Staging
Radiation Therapy
Chemo/Hormone Therapy
Complications

 

Written by: Graham Pingree

EDUCATIONAL PEARL