Juvenile nasopharyngeal angiofibroma is a rare, benign tumor that typically affects adolescent males. It originates in the nasopharynx near the sphenopalatine foramen. Surgery is the primary treatment, with a shift towards endoscopic techniques rather than open approaches. Endoscopic surgery has shown similar recurrence rates to open techniques for early-stage tumors. Precise surgical removal is the goal to prevent local destruction and recurrence of this uncommon fibrovascular growth.

1. Juvenile Nasopharyngeal
Angiofibroma
DEPCIT
approach
Angus Shao

2. Definition
• Rare, benign
• Locally destructive fibrovascular tumour
JNA Otolaryngol Clin N Am 44 (2011)
989–1004

3. Epidemiology
• Males
• Teenage, young adult - range from 9 to 29
years (mean age, 15 years)
• 0.05% ? of H&N tumours
• Extremely rare in female/ patient older than
25
JNA Otolaryngol Clin N Am 44 (2011)
989–1004

4. Aetiology
• Unknown
• Theories:
–Nonchromaffin paraganglionic cells
–Vascular hamartoma (Girgis 1973)
–JNA stroma cells (Coutinho-Camillo 2008)
• Vascular endothelial growth factor receptor-2
• Transforming growth factor beta 1
• Insulin-like growth factor 2
• Deletion of chr 17 (p53)

5. Aetiology
• Hormonal Receptors (Montag 2006)
– Androgen
– Estrogen
• Embryologic chondrocartilage of skull bones (Schiff 1959)
– Superior margin of sphenopalatine foramen
– Trifurcation
• Palatine bone
• Horizontal ala of vomer
• Root of pterygoid process

6. Origin
(Operative Techniques in Otolaryngology 1999; 10(2): 101-106.)
Controversial
• Posterolateral nasal wall at
sphenopalatine foramen
• Vidian canal

7. Pathology
• Macro
– well defined, mucosalised, red/purple lobulated
mass arising in the nasopharynx from the lateral
wall, posterior to MT

8. Pathology
• Micro
– non-encapsulated, fibrous pseudocapsule
– spindle/stellate cells in a rich collagen matrix
– with vascular spaces devoid of elastic fibers (elastic
lamina)
• Lack muscularis layer
– Partially androgen dependent
• Receptors for testosterone, DHT, Androgen
– not useful in Tx
– B-catenin mutation
• APC/B-catenin mutation in FAP
• JNA 25 times more likely in FAP - controversial
(Hauptman 2007)

9. Clinical
• Adolescent male
• Unilateral nasal
obstruction most common
• Recurrent epistaxis
• Nasal mass
– Smooth, lobulated
– Compressible
– Purplish or reddish hue
(Operative Techniques in Otolaryngology 2011; 22(4):281-284.)

10. Staging
• No universal staging system
• Most commonly accepted:
– Radkowski
(Radkowski 1996)

11. Investigation
• Bloods
• Biopsy??!!!!
• Imaging

12. Timing of surgery related to
embolisation
• Within 24 hr  negate the benefits of
embolization, insufficient devascularization and
tumor necrosis  greater operative blood loss
• thrombus formation and multinucleated giant cell
reaction within 7 days of embolization
• recanalization and partial revascularization can be
observed in 30% of embolized vessels after 7 days
• Maximal tumour softening observed at 8 days
J NeuroIntervent Surg doi:10.1136/neurintsurg-2012-010350

13. Treatment
• Surgical disease
• Open vs Endoscopic
• Rtx (unresectable) / Chemotherapy(rarely)
• Hormonal therapy
• Observation? !

14. Open Approach
• Transpalatal
• Lateral Rhinotomy
• Mandibular swing
• Midfacial degloving

15. Endoscopic
• Shift towards endoscopic approach in last 10
years
• Mostly for early disease
• Endoscopic appropriate up to stage IIIA
tumors (Wormald 2003)

16. Endoscopic Coblation Technique

17. Annals of Otology, Rhinology & Laryngology
122(6):353-357.

18. Summary
• Benign rare but locally destructive disease in
adolescent male
• Fibrovascular tumour originated at SPF/Vidian
canal
• Surgery is the treatment of choice (most)
• Shift to endoscopic approach with similar rate
of recurrence compared to open technique