Juvenile Nasopharyngeal Angiofibroma
DEPCIT approach
Angus Shao
Definition
• Rare, benign • Locally destructive fibrovascular tumour
JNA Otolaryngol Clin N Am 44 (2011) 989–1004
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
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)
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
Origin
(Operative Techniques in Otolaryngology 1999; 10(2): 101-106.)
Controversial • Posterolateral nasal wall at
sphenopalatine foramen• Vidian canal
Pathology
• Macro– well defined, mucosalised, red/purple lobulated
mass arising in the nasopharynx from the lateral wall, posterior to MT
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)
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.)
Staging
• No universal staging system• Most commonly accepted:
– Radkowski(Radkowski 1996)
Investigation
• Bloods• Biopsy??!!!!• Imaging
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
Treatment
• Surgical disease• Open vs Endoscopic
• Rtx (unresectable) / Chemotherapy(rarely)• Hormonal therapy• Observation? !
Open Approach
• Transpalatal• Lateral Rhinotomy• Mandibular swing• Midfacial degloving
Endoscopic
• Shift towards endoscopic approach in last 10 years
• Mostly for early disease • Endoscopic appropriate up to stage IIIA
tumors (Wormald 2003)
Endoscopic Coblation Technique
Annals of Otology, Rhinology & Laryngology 122(6):353-357.
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