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JUVENILE NASOPHARYNGEAL ANGIOFIBROMA

Introduction
JNA: A rare, benign vascular tumor Only in adolescent males. Highly vascular, aggressive and locally invasive. Has the potential to cause severe morbidity by
Severe epistaxis, Intracranial spread High rate of recurrence.

Surgical Anatomy
Pterygopalatine fossa

Pterygoid plates

Infratemporal fossa

Surgical Anatomy

Incidence
0.5% of all head & neck cancers

Its incidence higher in India and Egypt


Reported incidence is 1: 5000-1:60,000

However, it is the commonest nasopharyngeal


benign tumour

Affects young adolescent males.


Age 9-18 yrs with median 14 yrs There are reports of occurrence in females, but if true the chromosomal studies or tissue diagnosis is to be reviewed

Natural History
The age of actual occurrence is not known. Age ranges from 9-18 yrs But not seen in young adults, Does spontaneous regression occur at end of puberty?.

Etiology
Large endothelial lined spaces found near the sphenopalatine foramen & base of the pterygoid plates in both male and female fetuses. Tumour occurs in males at puberty a hormonal response by the tumor is presumed.

However, no estrogen or progesterone receptors found in tumor specimens.


Circulating levels of gonadotropins in involved males have all been normal.

Histopathology
Gross Appearance: Irregular, smooth reddish purple mass. It has a firm, nodular texture with a sessile base. Nonencapsulated Develops multiple attachments with feeders

Histopathology
Microscopic Appearance : Vascular component in a fibrous stroma. Vascular component predominates. Vessel walls have a single endothelial lining. No muscular layer. Fibrous stroma has fine and coarse collagenous fibrils with characteristic stellate connective tissue cells interspersed.

Site of Origin & Location

The tumor appears to arise from In the posterolateral wall of posterior nares Near the roof of the nasal cavity at the sphenopalatine foramen The root of the pterygoid process of the sphenoid.

Site of origin

In the posterolateral wall of posterior nares

Sphenopalatine foramen

The root of the pterygoid process of the sphenoid.

Spread
Lesion simultaneously expands 1. Anteriorly into the nasal cavity 2. Posteriorly and superiorly in the nasopharynx. 3. Posteriorly into the sphenoid sinus.

4. Laterally it extends
a) Through the sphenopalatine foramen into the pterygopalatine fossa.

b) Further laterally into the infratemporal region


5. The lesion can extend intracranially by one of two routes. a) Through the expanded infra orbital fissures b) Through the sphenoid sinus through the sella turcica.

Spread
Anteriorly : Nasal Cavity

Superiorly: Sphenoid sinus

Posterioinferiorly: Oropharynx

Staging
Stage I : Tumor limited to nasopharynx and nasal cavity.
Stage II : Tumor invading the pterygopalatine fossa or the maxillary, ethmoid or sphenoid sinus. Stage III: Tumor invading the infratemporal fossa or orbital region or intracranial but extradural

Stage IV: Intracranial intradural tumor with infiltration of the


cavernous sinus, pituitary fossa or optic chiasm.

Signs and Symptoms


Presenting signs and symptoms dependon location, size, and extension of the tumor.
1. Progressive nasal obstruction with rhinorrhea. 90% 2. Recurrent epistaxis -seeks medical advice. 50% 3. Headache 25% 4. Facial deformity 10%

Signs and Symptoms On spread1. Conductive deafness. OME


2. Facial swelling, proptosis and sinusitis.

3. Further extension into the orbit and intracranial fossae may produce cranial nerve deficits.

Signs and Symptoms


O/E Mouth breathing May be anaemic. Nasal mass filling one nasal cavity Orbital mass Blunt probe cannot be passed medially Often seen hanging in the oropharynx Proptosis Decreased vision Usual initial diagnosis NASAL POLYP Saving grace CT Scan

Invs
Aim: To determine the
Location Spread Amount of enhancement (vascularity) Feeding vessels

Imaging
1. CT Scan 2. MRI 3. DSA NO FNAC/BIOPSY UNLESS IN OT JUST BEFORE SURGERY

CT SCAN
CT scan in axial and coronal planes. CT classical findings: 1) Anterior bowing of the posterior wall of the maxillary sinus 2) A dense homogeneous enhancement with contrast. 3) Disruption/ distortion of petrygoid plates

CT Scan

1. Anterior bowing of the posterior wall of the maxillary sinus 2. Distortion of pterygoid plates

Digital Subtraction Angiography


1. To determine the major feeding vessels 2. To embolise the major feeders, preoperatively 3. Blood loss earlier 2 litres now 600 ml

Treatment options 1. Surgery: Primary modality 2. Embolisation: Preop 48 hrs with gelfoam

3. Radiotherapy: Used for stage IV & recurrence


Low dose 3500 cGy over 3 weeks

4. Chemotherapy
5. Hormonal Therapy

Aims of surgery
1. Removal of disease 2. Protection of vital structures 3. Restoration of critical anatomic barriers 4. Functional and aesthetic reconstruction

Surgical Approaches

1. Transpalatal:

Stage I

2. Lateral Rhinotomy:
3. Maxillary Swing:

Stage II
Stage III

4. Craniofacial resection: Stage IV

WeberFerguson incision

Complications
Primary Hemorrhage Consumption coagulopathy Blindness due to embolisation Residual/ recurrent disease

Results
Extracranial growth cure rate: 100% Intracranial cure rate : 70% 90%

Recurrence/ residual growth:

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