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JUVENILE NASOPHARYNGEAL
ANGIOFIBROMA
R A A F I U L B A S H E E R Z A R G A R
INTRODUCTION
 It is also known as
nasopharyngeal
fibroma .
 It is a rare tumor
but commonest of
all benign tumor of
nasopharynx.
ETIOLOGY
 Idiopathic
 Mostly seen in adolescent males in 2nd decade of
life.
 Hormonal Theory
• Testosterone dependent
• Patients have a hamartomatous nidus of vascular
tissue and this is activated to form angiofibroma
when male sex hormone appears.
SITE
Posterior part of
nasal cavity close
to the superior
margin of
sphenopalatine
foramen
GROWTH
 Locally invasive benign
tumors.
 From site of origin to nasal
cavity, nasopharynx and
into the pterygopalatine
fossa, running behind the
posterior wall of maxillary
sinus which is pushed
forward as the tumor
grows. Laterally it extends
into pterygomaxillary
fossa then to
infratemporal fossa and
cheek.
PATHOLOGY
Severe bleeding as the vessels lose ability to contract
Made up of vascular and fibrous tissue mostly the vessels are just
endothelium lined spaces with no elastic or muscle coat
MICROSCOPIC
APPERANCE
ENDOTHELIUM
STROMA
EXTENSION
 Nasal cavity- Causing nasal obstruction,
epistaxis and nasal discharge
 Paranasal sinuses- Maxillary, sphenoid and
ethmoid sinuses can all be invaded
 Pterygomaxillary fossa, infratemporal fossa
and cheek
 Orbits-proptosis, frog face deformity
EXTENSION
 CRANIAL CAVITY
• ANTERIOR CRANIAL FOSSA
through roof of ethmoids or
cribriform plate
• MIDDLE CRANIAL FOSSA
through erosion of floor of middle
cranial fossa or indirectly by
invading the sphenoid sinus and
sella tunica.
SYMPTOMS
SEX-MOSTLY IN
MALES AGE-10-20YRS
PROFUSE, RECURRENT AND
PAINLESS EPISTAXIS LEAD TO
ANAEMIC DUE TO REPEATED
BLOOD LOSS.
SYMPTOMS
 Progressive nasal obstruction and denasal
speech
 Conductive hearing loss and otitis media with
effusion
 Mass in the nasopharynx
 Broadening of nasal bridge
 Proptosis
 Swelling of cheek
 Conductive hearing loss and serous otitis
media due to obstruction of Eustachian tube
SIGNS
 Anterior Rhinoscopy –
1. Pink or purplish nasopharyngeal mass
2. sessile, lobulated or smooth
3. obstructs one or both choanae
4. Consistency is firm but digital palpation is
never done because it can result in
profuse bleeding.
INVESTIGATIONS
 CT Scan
• Investigation of choice
• The extent of tumour,
bony destruction or
displacements can be
seen.
• Hollman-Miller Sign
Anterior bowing of maxilla
and Posterior bowing of
ptyerigoid.
Contrast CT scan juvenile
nasopharyngeal angiofibroma.
Note the pterygopalatine fossa
and infratemporal fossa
extension
INVESTIGATIONS
 MRI
 Carotid angiography
shows the extent of
tumors
STAGING
 Sessions’s classification, modified by Radkowski reflects
 IA Tumor limited to nose and nasopharyngeal vault
 IB Extension to paranasal sinuses
 IIA Minimal extension to pterygomaxillary fissure (PMF)
 IIB Full extension to PMF and/or erosion of orbital bones
 IIC Extension to infratemporal fossa and/or cheek or
posterior to pterygoid plates
 IIIA Erosion of skull base: minimal intracranial
 IIIB Extensive intracranial and/or cavernous sinus
extension
MEASURES TO REDUCE THE
VASCULARITY OF THE
TUMORREDUCE THE
VASCULARITY OF TUMOR
LARITY OF TUMOR
• .
• Embolization of the feeding vessels.
• Estrogen therapy: Stilboestrol 2.5 mg three
times a day for 3 weeks. Not preferred
currently.
• Preoperative radiation: Generally not
favored.
• Cryotherapy.
TREATMENT(OPEN
SURGICAL EXCISION)
 Surgical approaches
 Transpalatine: Tumours confined to nasopharynx, nasal cavity and
sphenoid sinus.
 Le Fort 1 osteotomy approach: For extension to paranasal sinuses,
pterygopalatine fossa and infratemporal fossa.
 Medial maxillectomy: It provides access to orbit, ethmoid and sphenoid
sinuses and anterior skull base.
 Sardana’s approach: Transpalatine + Sublabial.
 Extended lateral rhinotomy
 Extended Denker’s approach
 Intracranial-extracranial
 Infratemporal fossa
TREATMENT
TREATMENT
 RADIATION
THERAPY
• For advanced tumors
with intracranial
extension
COMPLICATIONS
 Secondary
malignancy
 Abnormal
craniofacial
development
 Cataracts
 Optic atrophy
 Osteoradionecrosis.
MANAGEMENT OF
RECURRENCE
 Radiotherapy
• 3000-3500 cGy of radiation given in 15 to
20 sittings.
 Chemotherapy
• Drugs like Vincristine, Doxorubicin ,
Dacarbazine
 The above methods can arrest the growth
and cause tumor regression but not total
tumor eradication
PROGNOSIS
Excellent prognosis on
complete surgical
removal.
THANK
YOU

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Juvenile nasopharyngeal angiofibroma

  • 1. JUVENILE NASOPHARYNGEAL ANGIOFIBROMA R A A F I U L B A S H E E R Z A R G A R
  • 2. INTRODUCTION  It is also known as nasopharyngeal fibroma .  It is a rare tumor but commonest of all benign tumor of nasopharynx.
  • 3. ETIOLOGY  Idiopathic  Mostly seen in adolescent males in 2nd decade of life.  Hormonal Theory • Testosterone dependent • Patients have a hamartomatous nidus of vascular tissue and this is activated to form angiofibroma when male sex hormone appears.
  • 4. SITE Posterior part of nasal cavity close to the superior margin of sphenopalatine foramen
  • 5. GROWTH  Locally invasive benign tumors.  From site of origin to nasal cavity, nasopharynx and into the pterygopalatine fossa, running behind the posterior wall of maxillary sinus which is pushed forward as the tumor grows. Laterally it extends into pterygomaxillary fossa then to infratemporal fossa and cheek.
  • 6. PATHOLOGY Severe bleeding as the vessels lose ability to contract Made up of vascular and fibrous tissue mostly the vessels are just endothelium lined spaces with no elastic or muscle coat
  • 8. EXTENSION  Nasal cavity- Causing nasal obstruction, epistaxis and nasal discharge  Paranasal sinuses- Maxillary, sphenoid and ethmoid sinuses can all be invaded  Pterygomaxillary fossa, infratemporal fossa and cheek  Orbits-proptosis, frog face deformity
  • 9. EXTENSION  CRANIAL CAVITY • ANTERIOR CRANIAL FOSSA through roof of ethmoids or cribriform plate • MIDDLE CRANIAL FOSSA through erosion of floor of middle cranial fossa or indirectly by invading the sphenoid sinus and sella tunica.
  • 10. SYMPTOMS SEX-MOSTLY IN MALES AGE-10-20YRS PROFUSE, RECURRENT AND PAINLESS EPISTAXIS LEAD TO ANAEMIC DUE TO REPEATED BLOOD LOSS.
  • 11. SYMPTOMS  Progressive nasal obstruction and denasal speech  Conductive hearing loss and otitis media with effusion  Mass in the nasopharynx  Broadening of nasal bridge  Proptosis  Swelling of cheek  Conductive hearing loss and serous otitis media due to obstruction of Eustachian tube
  • 12. SIGNS  Anterior Rhinoscopy – 1. Pink or purplish nasopharyngeal mass 2. sessile, lobulated or smooth 3. obstructs one or both choanae 4. Consistency is firm but digital palpation is never done because it can result in profuse bleeding.
  • 13. INVESTIGATIONS  CT Scan • Investigation of choice • The extent of tumour, bony destruction or displacements can be seen. • Hollman-Miller Sign Anterior bowing of maxilla and Posterior bowing of ptyerigoid. Contrast CT scan juvenile nasopharyngeal angiofibroma. Note the pterygopalatine fossa and infratemporal fossa extension
  • 14. INVESTIGATIONS  MRI  Carotid angiography shows the extent of tumors
  • 15. STAGING  Sessions’s classification, modified by Radkowski reflects  IA Tumor limited to nose and nasopharyngeal vault  IB Extension to paranasal sinuses  IIA Minimal extension to pterygomaxillary fissure (PMF)  IIB Full extension to PMF and/or erosion of orbital bones  IIC Extension to infratemporal fossa and/or cheek or posterior to pterygoid plates  IIIA Erosion of skull base: minimal intracranial  IIIB Extensive intracranial and/or cavernous sinus extension
  • 16. MEASURES TO REDUCE THE VASCULARITY OF THE TUMORREDUCE THE VASCULARITY OF TUMOR LARITY OF TUMOR • . • Embolization of the feeding vessels. • Estrogen therapy: Stilboestrol 2.5 mg three times a day for 3 weeks. Not preferred currently. • Preoperative radiation: Generally not favored. • Cryotherapy.
  • 17. TREATMENT(OPEN SURGICAL EXCISION)  Surgical approaches  Transpalatine: Tumours confined to nasopharynx, nasal cavity and sphenoid sinus.  Le Fort 1 osteotomy approach: For extension to paranasal sinuses, pterygopalatine fossa and infratemporal fossa.  Medial maxillectomy: It provides access to orbit, ethmoid and sphenoid sinuses and anterior skull base.  Sardana’s approach: Transpalatine + Sublabial.  Extended lateral rhinotomy  Extended Denker’s approach  Intracranial-extracranial  Infratemporal fossa
  • 19. TREATMENT  RADIATION THERAPY • For advanced tumors with intracranial extension
  • 20. COMPLICATIONS  Secondary malignancy  Abnormal craniofacial development  Cataracts  Optic atrophy  Osteoradionecrosis.
  • 21. MANAGEMENT OF RECURRENCE  Radiotherapy • 3000-3500 cGy of radiation given in 15 to 20 sittings.  Chemotherapy • Drugs like Vincristine, Doxorubicin , Dacarbazine  The above methods can arrest the growth and cause tumor regression but not total tumor eradication