Juvenile nasopharyngeal angiofibroma – Etiology, Pathology, Clinical Features and Management

• Juvenile nasopharyngeal angiofibroma is the most common benign tumour of the nasopharynx
• It is usually seen in males in the second decade of life and usually presents with recurrent profuse epistaxis

Etiology:

• Since it occurs in the second decade of life, it is proposed that nasopharyngeal angiofibroma is a testosterone dependent tumour
• A nidus of hamartomatous vascular tissue in the nasopharynx undergoes proliferation in response to hormonal stimulation

Site of occurence:

• The site of occurrence was initially thought to be the posterior wall and roof of nasopharynx
• But recent studies indicate that the site of origin is in fact the posterior part of nasal cavity, near sphenopalatine foramen

Pathology:

• Angiofibroma as the name suggests is composed of both fibrous and vascular elements
• The vascular elements are composed of endothelium lined blood vessels without muscle coat
• Hence there is increased risk of bleeding which is not responsive to application of adrenaline

Pathways of spread:

• Nasal cavity
• Paranasal sinuses
• Pterygomaxillary fossa
• Orbit – through superior or inferior orbital fissure
• Infratemporal fossa
• Cranial cavity
  • Usually the middle cranial fossa is involved. Spread can be through:
    • In front of foramen lacerum – lateral to carotid artery
    • Through wall of sphenoid sinus to region of sella turcica – medial to carotid artery
  • Anterior cranial fossa spread can occur through
    • Roof of ethmoid sinus
    • Cribriform plate

Clinical features:

• The usual presentation is one of recurrent, profuse epistaxis which is not necessarily associated with any provocative factor
• Nasal blockage and denasal speech
• Blockage to the eustachian tube opening results in conductive hearing loss and serous otitis media
• Extension to the orbit results in proptosis and involvement of II, III, IV and VI cranial nerves
• Swelling of cheek and broadened nasal bridge

Investigations:

• X-ray lateral view nasopharynx reveals the presence of a mass
• X-ray paranasal sinuses
• CT scan of head with contrast enhancement shows the extend of involvement and bony destruction – it is the investigation so choice
  • Anterior bowing of posterior wall of maxillary sinus – Holman Miller sign – is pathognomonic of angiofibroma
• MRI helps to better understand the soft tissue involvement
• Carotid angiography helps to ascertain the level of vascularity and has to be done before attempting embolization

Treatment:

• Surgical resection
  • There are different approaches available
    • Transpalatine
    • Transmaxillary
    • Intracranial – Extracranial
    • Extended lateral rhinotomy
    • Extended Denker’s approach
    • Infratemporal fossa
    • Endoscopic
  • Endoscopic resection is the preferred method
  • Blood loss during surgery is a major problem
  • Blood loss can be minimised by decreasing the vascularity of the tumor by any of the following methods
    • Hormonal therapy with diethyl stilbestrol
    • Cryotherapy
    • Embolization
• Hormonal therapy
  • Since nasopharyngeal angiofibroma is a androgen dependent tumour, diethyl stilbestrol or flutamide can be tried
• Radiotherapy
• Chemotherapy
  • For treatment of recurrence or residual lesions after surgery