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Definition

• Common, benign, vascular neoplasm


• Accounts for 0.5% of all head and neck tumors.

Incidence :
 Commonest benign tumor of the nasopharynx
 Sex : Only in males
 Age : Adolescents ( below 18 y )
 Distribution : Higher in INDIA and EGYPT
Definition

Most common head &


neck tumour is
JNA
Clinical Approach

Etiology :
I. Hormonal theory (testosterone-
dependant tumor )

II. Beta-catenin gene mutations

III. Hamartomatous ectopic part of vascular


tissue (most common theory )
Clinical Approach

Site Of Origin :
Postero-superior part of lateral wall
At the site of sphenopalatine foramen
Behavior :
Anteriorly : to the nasal cavity
Laterally : to pterygopalatine fossa
Forwards and laterally : to the orbit
Upwards : intra-cranial cavity (Sella turcica)
Clinical Picture

Symptoms :
Nasal obstruction (80-90%) - commonest symptom
Epistaxis (45-60%) - unilateral and recurrent
Headache (25%)
oFacial swelling (10-18%)
Others :

Unilateral rhinorrhea, anosmia, hyposmia,


deafness, otalgia
Clinical Picture

Signs :
oNasal mass (80%)
Orbital mass (15%)
(Proptosis ( 10-15%
Others :
serous otitis : eustachian tube blockage
zygomatic swelling and trismus

decreasing vision :(optic nerve tenting (rare


Clinical Picture

Most common
symptom is
Nasal
Obstruction
Investigation

Imaging Studies:

Plain X-ray
CT scanning & MRI :
Show extension of the tumour
Angiography :

ECA branches is the 1ry feeders


Biopsy :
• Cause severe bleeding
• Exclude malignancy
Investigation

Staging :
Stage I :Tumors limited to nasal cavity,
nasopharynx with no bony destruction

Stage II : Tumors invading pterygomaxillary


fossa, paranasal sinuses with bony destruction

Stage III : Tumors invading infratemporal fossa,


orbit and/or parasellar region remaining lateral
to cavernous sinus

Stage IV :Tumors invading cavernous sinus,


optic chiasmal region, and/or pituitary fossa
Investigation

Differential Diagnosis :

 Antrochoanal polyp
Investigation

Differential Diagnosis :

 Inverting papilloma
Investigation

Differential Diagnosis :

 Rhabdomyosarcoma
Investigation

Differential Diagnosis :

 Squamous cell carcinoma


Treatment

Medical therapy:
•Hormonal therapy :
The testosterone receptor blocker flutamide

Inhibition of Growth factors :


Therapy of inoperable JNA.

Radiotherapy

80% cure rates with radiation therapy


Stereotactic radiotherapy (Gamma knife)
Three-dimensional conformal radiotherapy
Treatment

Surgical therapy:

Lateral rhinotomy: small tumors (stage I or II).


Infratemporal fossa approach : large lateral
extension
The midfacial degloving approach :
With or without LeFort osteotomy improves
posterior access to the tumour
Intranasal endoscopic surgery :
tumors limited to the nasal cavity and
paranasal sinuses
Treatment

Main treatment of
JNA is
Surgical
Complication

• Excessive bleeding
• Malignant transformation
• Transient blindness
• Osteoradionecrosis
• Fistula of the palate
• Recurrence :
Complication

Risk factors for recurrence :

1. Tumor in the pterygoid fossa


2. Tumor in basisphenoid
3. Erosion of the clivus
4. Intracranial extension
5. Feeders from the internal carotid artery
6. Young age
7. Residual tumour
Mostafa El-Sayed
Marwa Wageeh

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