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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
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.
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.
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
Sphenopalatine foramen
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.
Spread
Anteriorly : Nasal Cavity
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
3. Further extension into the orbit and intracranial fossae may produce cranial nerve deficits.
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
Treatment options 1. Surgery: Primary modality 2. Embolisation: Preop 48 hrs with gelfoam
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
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%
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