angiofibroma

1. Angiofibroma
Dr. Krishna Koirala
Nov 20, 2017

2. ● Benign, highly vascular and locally
aggressive tumour of nasopharynx which
occurs exclusively in prepubertal and
adolescent males
● Accounts for 0.05% of all head and neck
neoplasms
● Friedberg (1940) : “Angiofibroma”

3. • Synonyms
– Angiofibroma
– Juvenile Nasopharyngeal angiofibroma
– Nasopharyngeal fibroma
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4. • Age of onset - second decade (7-19 years )
• Mean age at diagnosis : 14 years
• May regress in late teens but may persist into
adulthood
• Rare after 25 years of age
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5. Site of origin
• Close proximity to the posterior attachment of
the middle turbinate near the superior border
of sphenopalatine foramen
• ? from nonchromaffin paraganglionic cells of
the terminal branches of maxillary artery
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8. Theories Of Origin
• Hormonal : occurrence in adolescent males
• Desmoplastic response of the nasopharyngeal
periosteum or embryonic fibrocartilage between the
basiocciput and the basisphenoid
• Hamartomas testosterone Angiofibroma
• Nest cells (undifferentiated Epitheloid)
• Vestiges of atrophied stapedial artery
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9. • Hamartoma
– Benign, focal malformation that resembles a
neoplasm in the tissue of its origin
– Not a malignant tumor, grows at the same rate as
the surrounding tissues
– Composed of tissue elements normally found at
that site which are growing in a disorganized mass
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10. Pathophysiology
• Starts adjacent to the sphenopalatine foramen
• Large tumors - bilobed or dumbbell shaped :
(one portion of the tumor filling the naso -
pharynx and other portion extending to the
pterygopalatine fossa)

11. Spread
• Anterior growth
–Nasal cavity (filled on one side , septum
deviates to the other side), maxillary sinus
• Superior growth
–Sphenoid sinus, cavernous sinus , pituitary
fossa, optic chaisma , middle cranial fossa
–Anterior skull base  Middle cranial fossa

12. • Lateral spread
– Pterygopalatine fossa  Pterygomaxillary fissure
 infratemporal fossa  Cheek
– Greater wing of the sphenoid  middle fossa dura
– Infraorbital fissures  Orbit (Proptosis ,optic
nerve atrophy )
• Posterior
– Nasopharynx
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14. Symptoms
• Nasal obstruction (80-90%)
– Most frequent symptom
• Epistaxis (45-60%)
– Mostly unilateral and recurrent : painless, profuse,
unprovoked
• Headache (25%)
– Blocked paranasal sinuses, Intracranial
• Facial swelling (10 - 18%)

15. • Other symptoms
– Unilateral rhinorrhea
– Anosmia/ hyposmia
– Rhinolalia clausa
– Deafness, otalgia
– Swelling of the palate
– Deformity of the cheek
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16. Signs
• Nasal /Nasopharyngeal mass (80%)
• Orbital mass (15%) , Proptosis (10 -15%)
• Cheek swelling and trismus (infratemporal fossa
involvement)
• Frog face deformity
• Serous otitis media (ET blockage)
• Cranial nerve involvement (II, III, IV, V VI)

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Characteristic Presentation : Teenage or young adult
male with recurrent epistaxis , nasal mass and nasal
obstruction

18. Investigations
• Plain x-ray of Nose and PNS
– Haziness of the sinuses, bone erosion
• CT scan of Nose and PNS (CECT)
– Extent / vascularity of tumor
– Holman Miller sign ( Anterior bowing of posterior
wall of maxillary antrum)
– Bone erosion / Widening of sphenopalatine
foramen

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21. • Magnetic resonance imaging (MRI)
– Delineate and define the soft tissue extent in
cases of intracranial involvement
• Angiography (DSA)
– Extent ,tumor blush, feeding arteries
• Tumor Biopsy : Contraindicated
– Vessels are thin walled, lack elastic fibers, absent
or incomplete smooth muscle (cause for excessive
bleeding)
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Hypertrophic maxillary artery is the main feeder

23. • Other Investigations
– CBC, Urine R/E, ESR, Bleeding and Clotting profile
– ECG
– X-ray chest
– Blood group and cross match
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24. Staging
Stage I: Tumor limited to Nasal cavity or
nasopharynx with no bony destruction
Stage II: Tumor invading pterygopalatine fossa
or PNS
Stage III: Tumor invading infratemporal fossa
/orbit or parasellar region
Stage IV: Tumor invading cavernous sinus/
optic chaisma /pituitary fossa
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25. Differential Diagnosis
• Other causes of nasal obstruction
– Antrochoanal polyp, teratoma, encephalocele,
dermoids, inverted papilloma, rhabdomyosarcoma,
squamous cell carcinoma
• Other causes of epistaxis
– Systemic or local
• Other causes of proptosis or orbital swellings
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26. Treatment Options
• Surgery
– Gold standard
• Radiotherapy
– Reserved for unresectable tumor, intracranial
extension , recurrent cases
– 3000 – 3500 cGy in 15 -18# over 3 - 3.5 wks
– Proton stereotactic radiotherapy

27. • Chemotherapy
–Recurrent tumors with previous surgery and
radiation
• Hormone therapy
–To reduce vascularity before surgery
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28. Surgical Approaches
• Intranasal Endoscopic
• Transpalatal
• Transmaxillary
– Extended Denker’s approach
– Midfacial degloving
– Extended lateral rhinotomy
• Infratemporal fossa
• Anterior Subcranial
• Image guided Surgery (Recent Advance)

29. Preop. reduction of tumor vascularity
• Embolization of feeding arteries
– 24 to 72 hours pre operative
– Gelfoam (resorbed in approximately 2 weeks)
– Polyvinyl alcohol foam (more permanent )
• Estrogen Therapy
– Diethylstilbestrol 2.5 mg PO TDS for 3-6 wks (Cellular
contraction, increase in collagen and fibroblasts 
decreases bleeding, reduces size)

30. • Testosterone Receptor blocker
–Flutamide
• Radiotherapy
–Proton stereotactic RT
• Cryotherapy
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31. Embolization

32. Selection of Surgical Approach

33. • Intranasal endoscopic
Approach
– Small tumor in nose,
PNS , nasopharynx,
pterygopalatine fossa
and even for large
tumors
– Newer technique
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34. • Transpalatal Approach (Wilson)
– Small tumor in Nasopharynx
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35. • Transpalatal + Sublabial Approach( Sardana)
– Large tumor of Nose/PNS/ Nasopharynx
• Transmaxillary Approach
– For tumors extending to pterygopalatine fossa
– Extended Lateral rhinotomy, mid facial degloving ,
Denker’s
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Midfacial degloving

37. Infratemporal fossa approach with or
without craniotomy
• For tumors extending to
infratemporal fossa/
intracranial extension

38. • Anterior subcranial approach
–Intracranial extension
• Image guided surgery
–Small/ medium size tumors
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