Juvenile Nasopharyngeal Angiofibroma

Benign tumor but locally aggressive

Rare overall , But most common Benign tumor of Nasopharynx

More common in Adolescent males (Androgen dependent tumor) in 2nd decade of life .

The origin of JNA is from  Hamartomatous nidus of vascular tissue in region of sphenopalatine foramen , which is stimulated in puberty byAndrogen.

Associations :- 


Arises from Posterolateral wall of nasopharynx , precisely at trifurcation of sphenoidal process of palatine bone , Roof of pterygoid process & Horizontal process of vomer . From here tumor grows into 

Histopathology :- 

Angiogenesis and Vascular proliferation


Clinical presentation - 

Nasal cavity - Unilateral Nasal obstruction with septum deviation to contralateral side , Reccurent Spontaneous Epistaxis often Torrential is most common presentation, Nasal discharge/Rhinorrhea , Rhinolalia clausa / Hyponasal speech [ not Rhinolalia aperta / Hypernasal speech] 

Paranasal sinuses - Recurrent headaches due to chronic sinusitis

Orbits - Frog face deformity , Proptosis

Diplopia due to erosion of Superior orbital fissure

Unilateral conductive hearing loss and otitis media with effusion due to Obstruction of eustachian tube

Cranial nerve palsies (2,3,4,6) - anosmia

Facial dysmorphism (Broadening of nasal bridge , swelling of cheeks , proptosis , Frog face deformity)

On examination :-

 Nasal endoscopy - Sessile , lobulated , Firm , friable reddish / reddish purple mass within nasal cavity. But digital palpation must never be done until at time of operation as bleeding can occur



Diagnosis :-

Investigation of choice - CECT scan  (Contrast enchanced CT) (Extent of tumor , Bony destruction/displacements)  { Holman-Miller sign / Antral sign is Pathognomonic - Anterior bowing of Posterior wall of Maxillary sinus}

Others-  MRI (Soft tissue extensions) , Carotid angiography (Tumor extent , Vascularity and feeding vessels which mostly come from INTERNAL MAXILLARY ARTERY > ASCENDING PHARYNGEAL ARTERY of external carotid system , but can also come from internal carotid artery most commonly VIDIAN artery > Ophthalmic artery)

Biopsy is contraindicated as it is vascular tumor so massive bleeding can occur

*Radkowski staging system for JNA*  


Treatment :-

Treatment of choice - Surgical excision

Preoperative embolisation of feeding vessels to reduce intraoperative blood loss

Approaches - 

Endoscopic Transpalatine approach 

Sardana's approach = Transpalatine + sublabial 

Modified midfacial degloving approach

le fort 1 maxillary osteotomy approach


Stereotactic Radiotherapy (Gamma knife) for intracranial extensions or inoperable/unresectable tumors or recurrent cases

Hormonal therapy (Flutamide- Androgen receptor blocker)

HIGH RECURRENCE RATE


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