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Angiofibroma
Dr. Krishna Koirala
2019-02-11
● 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”
• Synonyms
– Angiofibroma
– Juvenile Nasopharyngeal angiofibroma
– Nasopharyngeal fibroma
Tuesday, June 30, 2020 3
• 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
Tuesday, June 30, 2020 4
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
5
Tuesday, June 30, 2020 6
Tuesday, June 30, 2020 7
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
8
• 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
Tuesday, June 30, 2020 9
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)
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
• 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
12
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%)
• Other symptoms
– Unilateral rhinorrhea
– Anosmia/ hyposmia
– Rhinolalia clausa
– Deafness, otalgia
– Swelling of the palate
– Deformity of the cheek
Tuesday, June 30, 2020 15
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)
Tuesday, June 30, 2020 17
Characteristic Presentation : Teenage or young adult
male with recurrent epistaxis , nasal mass and nasal
obstruction
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
Tuesday, June 30, 2020 19
• 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)
Tuesday, June 30, 2020 21
Tuesday, June 30, 2020 22
Hypertrophic maxillary artery is the main feeder
• Other Investigations
– CBC, Urine R/E, ESR, Bleeding and Clotting profile
– ECG
– X-ray chest
– Blood group and cross match
Tuesday, June 30, 2020 23
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
24
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
Tuesday, June 30, 2020 25
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
• Chemotherapy
–Recurrent tumors with previous surgery and
radiation
• Hormone therapy
–To reduce vascularity before surgery
Tuesday, June 30, 2020 27
Surgical Approaches
• Intranasal Endoscopic
• Transpalatal
• Transmaxillary
– Extended Denker’s approach
– Midfacial degloving
– Extended lateral rhinotomy
• Infratemporal fossa
• Anterior Subcranial
• Image guided Surgery (Recent Advance)
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)
• Testosterone Receptor blocker
–Flutamide
• Radiotherapy
–Proton stereotactic RT
• Cryotherapy
Tuesday, June 30, 2020 30
Embolization
Selection of Surgical Approach
• Intranasal endoscopic
Approach
– Small tumor in nose,
PNS , nasopharynx,
pterygopalatine fossa
and even for large
tumors
– Newer technique
33
• Transpalatal Approach (Wilson)
– Small tumor in Nasopharynx
34
• 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
Tuesday, June 30, 2020 35
Tuesday, June 30, 2020 38
Midfacial degloving
Infratemporal fossa approach with or
without craniotomy
• For tumors extending to
infratemporal fossa/
intracranial extension
• Anterior subcranial approach
–Intracranial extension
• Image guided surgery
–Small/ medium size tumors
Tuesday, June 30, 2020 40

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Angiofibroma

  • 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 Tuesday, June 30, 2020 3
  • 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 Tuesday, June 30, 2020 4
  • 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 5
  • 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 8
  • 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 Tuesday, June 30, 2020 9
  • 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 12
  • 13.
  • 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 Tuesday, June 30, 2020 15
  • 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)
  • 17. Tuesday, June 30, 2020 17 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
  • 19. Tuesday, June 30, 2020 19
  • 20.
  • 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) Tuesday, June 30, 2020 21
  • 22. Tuesday, June 30, 2020 22 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 Tuesday, June 30, 2020 23
  • 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 24
  • 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 Tuesday, June 30, 2020 25
  • 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 Tuesday, June 30, 2020 27
  • 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 Tuesday, June 30, 2020 30
  • 33. • Intranasal endoscopic Approach – Small tumor in nose, PNS , nasopharynx, pterygopalatine fossa and even for large tumors – Newer technique 33
  • 34. • Transpalatal Approach (Wilson) – Small tumor in Nasopharynx 34
  • 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 Tuesday, June 30, 2020 35
  • 36.
  • 37.
  • 38. Tuesday, June 30, 2020 38 Midfacial degloving
  • 39. Infratemporal fossa approach with or without craniotomy • For tumors extending to infratemporal fossa/ intracranial extension
  • 40. • Anterior subcranial approach –Intracranial extension • Image guided surgery –Small/ medium size tumors Tuesday, June 30, 2020 40

Editor's Notes

  1. Teenage or young adult male Recurrent epistaxis Nasal obstruction