jna reg presentation

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