Juvenile Nasopharyngeal Angiofibroma

1. DEPARTMENT OF ENT AND HEAD AND NECK SURGERY
Topic : Juvenile Nasopharyngeal Angiofibroma
Presented by : Dr. Sarita

2. Introduction :
• Juvenile nasopharyngeal angiofibroma (JNA), it is also known as
juvenile angiofibroma (JAF), or fibromatous or angiofibromatous
hamartoma of the nasal cavity.
• It is a rare benign tumour affecting young adolescent males .
• It is a histologically benign but locally aggressive vascular tumor
affecting the nasal cavity.
• It is the commonest of all rare benign tumors of nasopharynx.
• It is characterised by loss of vascular muscle tonicity causing it to not
contract in an event of trauma and bleeding.

3. Aetiology :
• The exact cause is unknown.
• As the tumor is predominant in adolescent males, it is thought to be
testosterone dependent.
• Angiofibroma seems to be influenced by vascular endothelial growth
factor(VEGF), transforming growth factor-b1 and IGFI.
• Vascular endothelial growth factor is secreted by JNA and contributes
to strong vascularization of angiofibroma.

4. Factors that play a role in growth :
• Chromosomal abnormality : gain at chromosome number 4,6,8 and X
and losses on chromosome number 17, 22, and Y are most frequent.
• Increases prevalence of JA (25 times) in patient of FAP with mutation of
APC gene.
• This gene regulates beta catenin pathway, mutation of beta catenin
pathway is seen in sporadic and recurrent JA.
• Angiogenic growth factor VEGF has been found localised on both
endothelial and stromal cells.
• Overexpression of IGFI and
• hormonal

5. Feeder vessels of JNA :
• Internal maxillary artery
• Ascending pharyngeal artery, vidian artery, inferior hypophyseal
artery branch of internal carotid artery.
• Rarely vertebral artery.

6. Vascular anatomy :

7. Site of origin :
• JA has a epicenter of origin at the level of pterygopallatine fossa
which subsequently grows and spread through foramen and fissures
of skull base.
• Exact origin may be the area of sphenopalatine foramen, base of
pterygoid process or superior portion of choana.

8. Spread :
• Tumor migrates beneath mucus membrane of NP displacing it
downward in the process
• Grows in adjacent anatomical sites that offer less resistance and
invades cancellous bone of basi-sphenoid
• Medially : nasopharynx, nasal fossa eventually toward contralateral
side
• Laterally : Infratemporal fossa via an enlarged pterygomaxillary
fissure  anterior displacement of posterior maxillary wall 
contact with masticatory muscle and cheek.
• Posteriorly : ICA via vidian, cavernous sinus via foramen rotundum
and orbital apex via inferior orbital fissures.

10. Bony anatomy :

11. • Bone involvement occurs via two main mechanisms :
1. Resorption by direct pressure of prexisting bony structures with
osteoclastic activation.
2. Direct spread along perforating arteries into cancellous root of
pterygoid process.

12. Extensions of Nasopharyngeal fibroma :
JA may grow medially into the nasopharynx
(NaP) through the sphenopalatine foramen
(black dotted line and black arrowheads),
which is enlarged by the disease, superiorly,
into the sphenoid sinus (SpS) through the
sphenoid floor, and laterally into the
pterygopalatine fossa (PPF) through the
pterygopalatine fissure.
The cavernous sinus (CS) and middle
cranial fossa may be involved through the
superior orbital fissure, or from the
infratemporal fossa (ITF) through the
foramen rotundum and foramen
ovale. The vidian canal (VC) is also
involved.

13. The lesion grows into the infratemporal fossa (ITF) and pushes
anteriorly the posterior wall of the maxillary sinus (white dotted
line); posteriorly, it grows into the left vidian canal (VC) to the
medial genu of the carotid.

14. Permeative intraosseous growth inside the
cancellous
bone of the greater wing (GW) of the sphenoid.

15. Theories associated with its aetiopathogenesis :
• Ringertz theory : JNA arise from the periosteum of NP vault.
• Som & Neffson : Inequality in the growth of bones forming skull base
resulted in hypertrophy of underlying periosteum in response to
hormonal influence.
• Bensch & Ewings : Tumor probably arose from embryonic
fibrocartilage between basi-occiput and basi-sphenoid .
• Brunner : Suggested origin from conjoined pharyngobasillar and
buccopharyngeal fascia.

16. Theories associated with its aetiopathogenesis
• Sternberg : Proposed that JNAA could be a type of hemangioma like a
cutaneous hemangioma seen in children which regresses with age.
• Osborn : It could be due to either hamartoma or residual fetal erectile
tissue which were subjected to hormonal influence.
• Girgis & Fahmy : Observed cell nests of undifferentiated epitheloid
cell or “zellballen” at the growing edge of angiofibroma and so
considered it as a paraganglioma.

17. Theories associated with its aetiopathogenesis:
• Branchial arch artery theory :
• Schick et al postulated that it might develop from incomplete
regression of a brachial artery, which arises in embryogenesis
between days 22 and 24 and forms a temporary connection between
ventral and dorsal aorta.
• This artery usually regresses and forms a vascular plexus that either
involutes or may leave remnants, potentially leading to the
development of JA.
• Findings are supported by the fact that vessels in JA express laminin
alpha2, which is a marker of early angiogenesis.

19. Staging system :
• The main criterion guiding classification systems is the extent of the
lesion, with special reference to infratemporal extension, skull base
involvement, and intracranial invasion.
▪ Fisch staging system – most robust and practical.
defines clearly which surgical approach is required .
▪ Type 1 - Tumour limited to the nasopharyngeal cavity; bone
destruction negligible or limited to the sphenopalatine foramen
▪ Type 2 - Tumour invading the pterygopalatine fossa or the maxillary,
ethmoid or sphenoid sinus with bone destruction

20. Fisch staging contd.:
▪ Type 3a - Tumour invading the infratemporal fossa or orbital region:
without intracranial involvement
▪ Type 3b - Tumour invading the infratemporal fossa or orbital region:
with intracranial extradural (parasellar) involvement
▪ Type 4a - Intracranial intradural tumour: without infiltration of the
cavernous sinus, pituitary fossa or optic chiasma
▪ Type 4b - Intracranial intradural tumour: with infiltration of the
cavernous sinus, pituitary fossa or optic chiasma

22. Andrew etal staging :
▪ Type I – tumour limited to the nasopharyngeal cavity, bone
destruction negligible or limited to sphenopalatine foramen.
▪ Type II – Tumour invading the pterygopalatine fossa or the maxillary,
ethmoid or sphenoid sinus with bone destruction.
▪ Type III – Tumour invading the infratemporal fossa or orbital margin
(a) without intracranial involvement (b) with intracranial extradural
(parasellar) involvement
▪ Type IV – Intracranial intradural tumour (a) without infiltration of
cavernous sinus, pituitary fossa, optic chaisma (b) with infiltration of
cavernous sinus, pituitary fossa, optic chaisma Upto IVa, surgery is
advisable and for IVb, radiotherapy is recommended

23. Clinical features :
• Unilateral nasal obstruction and epistaxis (spontaneous and
recurrent). (most common)
• Advanced lesion show swelling of cheek, diplopia and headache.
• Denasal speech.
• Conductive hearing loss and otitis media wit effusion due to
obstruction of eustachian tube.
• In extensive lesions :
• Nasal bone becomes splayed out,
• swelling in temple and cheek
• Trismus and bulging of parotid
• Proptosis and frog face deformity.

24. Diagnosis :
• Endoscopy - The endoscopic finding of a smooth, hyper vascularized
lesion originating behind the middle turbinate, which is usually
laterally displaced against the lateral wall.

25. Diagnosis contd :
• On CT and MRI, the diagnosis of JA is based on the following three
features:
1. The area of origin.
2. its hypervascular appearance after contrast enhancement
3. and its pattern of growth.
• On MRI, the presence on both T1- and T2-weighted sequences of
several signal voids within the lesion, indicating major intralesional
vessels, further corroborates a diagnosis of JA.
• CT scan with contrast – enhancing soft tissue mass arising from NP,
lateral wall of nose. Pterygopalatine fossa may be widened by tumor.
• Biopsy is usually avoided.

26. CT showing destruction of bone
and extension into
pterygopalatine fossa
Juvenile angiofibroma on axial T2-weighted, spin-echo magnetic
resonance image. The lesion invades the left orbital apex (OA),
the lateral wall of the left sphenoid sinus (white arrows) is
completely destroyed, and the internal carotid artery (ICA) is
encased. On the right side, a bony barrier (arrowheads) still
separates the lesion from the internal carotid artery.

27. Hondusa sign : Widening of gap between ramus of mandible and
maxillary body.

28. Management :
• Pre- operative embolization :
• Intraoperative bleeding has always been considered one of the most
challenging issues in the management of JA.
• preoperative embolization, which is commonly performed 48 hours
decreasing intraoperative bleeding and therefore making assessment
of tumor borders at dissection more accurate.
• intra-arterial digital subtraction angiography, microcatheters, and
embolic agents such as polyvinyl alcohol particles makes super
selective embolization of feeding vessels even easier, Furthermore,
angiography provides a detailed map of feeding vessels.

29. Pre- embolization
After embolization

30. Management contd .:
• In small JAs of the maxillary artery, which can be clipped before
starting the dissection of the lesion.
• Surgery –
• Surgery is considered the mainstay in the management of JA.
Endoscopy Open approach

31. Endoscopic sinus surgery technique :
• Advantages :
• Magnified view of lesion and related anatomical structures from
multiple angles  better identification of interface between lesion
and soft tissue or adjacent bone structures
• allowed more accurate and complete dissection and better control of
bleeding.
• Indications :
• Fisch - type 1, type 2 and some type 3 (with limited medial invasion of
the infratemporal fossa) are done through this technique.
• Tumor involving nasal cavity, paranasal sinus and NP.
• Tumor with only medial infratemporal fossa involvement or extradural

32. Endoscopic sinus surgery technique :
• Complications :
• Pain
• Bleeding
• Infection
• Synechiae
• Orbital injury
• Loss of vision
• Intracranial injury

33. Management Contd. :
Open Surgical approaches used to remove angiofibroma, depending
on its origin and extensions, are listed below :
1. Transpalatal approach (Wilson’s) – allows access to NP (for small
lesions)
2. Transpalatine + Sublabial (Sardana’s approach)
3. Lateral rhinotomy with medial maxillectomy
(a) Via facial incision
(b) Via degloving approach
4. Transmaxillary (Le Fort I) approach
5. Maxillary swing approach or facial translocation approach, or Wei’s
operation

34. Management contd. :
• Transpalatal approach
• Wilson in 1951 described this
approach.
• exposure to nasopharynx as well as
extensions into the sphenoid sinus
and choana.
• It gives no visible scar and post op
healing is good.
• Indications:

35. ▪ A forward curved incision is made
just in front of the junction of hard and
soft palate.
▪ Mucoperiosteum is separated either
way. Posterior spine of the hard palate
is removed.
▪ Incision is extended laterally and
downwards on either side along the
pterygomandibular raphe.
▪ The mucosa of the lateral pharyngeal
wall is not divided and care is taken not
to damage the greater palatine vessels.
▪ Thus with blunt dissection the
periosteum is elevated, growth is
separated and finally avulsed in one
piece.

36. Transpalatal + Sublabial approach (Sardana’s)
▪ For Tumor extending to -cheek -ITF/PPF -Nasopharynx
▪ Incision : Pyriform aperture to max. tuberosity.
▪ Adv :
No bone removed : No facial deformity
No facial scar
▪ Disadv :
Extensive Tumor manipulation by sublabial route may cause
ankylosis of TMJ.

37. Lateral rhinotomy :
▪ Stage IIb– lateral rhinotomy combined with resection of
medial antral wall (medial maxillectomy
▪ Indication 1. Tumours limited to
nasopharynx,ethmoid,sphenoid,maxillary sinus
▪ Tumours extending to orbit,pterygopalatine
fossa,infratemporal fossa(medial end)
▪ Structure removed : Frontal process of maxilla

38. Lateral rhinotomy :

39. Maxillary swing :
▪ MAXILLARY SWING Described by Wei in 1991
▪ Indication Nasopharyngeal tumours with limited extension to
anterior infratemporal region.
▪ Contraindication-- Tumours extending into posterior infratemporal
region or para sellar region’
▪ Incision: Weber-Fergusson Longmire
▪ Advantages • Exposes nasopharynx and surrounding areas from
antero-lateral aspect • Entire maxilla separated from its bony
foundations • Sufficient exposure for dissection between both carotid
arteries

40. Mid facial degloving :
The mid facial
degloving approach avoids a facial scar
and provides good
access for limited lateral extensions, but
access to the superior and posterior
tumour surfaces can be limiting, and it
may therefore be inappropriate in cases
with significant sphenoid
Involvement or dural exposure.

42. Post operative work-up and recurrence :
• Postoperative surveillance is based on periodic endoscopic and imaging
examination, which should be performed for a minimum of 3 years
because most residual lesions tend to grow submucosally, contrast-
enhanced CT or preferably MRI plays a key role in their early detection.
• Imaging studies performed in the immediate postoperative period would
identify residual JA tissue more easily, because of the absence of any
inflammatory changes.

43. Post operative work-up and recurrence contd.:
▪ Recurrence :
▪ Younger age of onset
▪ Increased vascularity on HPE
▪ Piece-meal removal at surgery
▪ If periosteum was not elevated and removed
▪ Tumor left behind in ethmoid/max sinus /PPF/MCF, behind pterygoid
plates

44. Minimising residual/recurrence :
adequate surgical exposure, control of vascular feeders, and systematic
tumour removal would serve to minimize residual disease
▪ drilling out the basisphenoid ensures that no residual tumour remains
in the pterygoid canal or cancellous bone of the sphenoid.
▪ Prolonged clinical and radiological monitoring is necessary for all
these patients in view of the very high incidence of recurrent disease.
▪ Disease-free status five years after primary surgery probably
represents cure.

45. Management contd. :
• Radiotherapy :
• Radiotherapy is used for intracranial extension of disease when
tumour derives its blood supply from the internal carotid system.
• Recurrent angiofibromas have also been treated by radiotherapy.
Intensity modulated radiotherapy—a newer mode of treatment—may
be employed.
• A dose of 3000 to 3500 cGy in 15–18 fractions is delivered in 3–3.5
weeks.
• Tumour regresses slowly in about a year, sometimes even up to 3
years.

46. Management Contd. :
• Hormonal therapy :
• Hormonal therapy as the primary or adjunctive treatment has been
used with no significant regression in practice.
• Diethylstilbestrol and flutamide (an androgen blocker) have been
used in young male patients in puberty.
• Chemotherapy :
• Very aggressive recurrent tumours and residual lesions have been
treated by chemotherapy. Doxorubicin, vincristine and dacarbazine
have been used in combination.

47. Bibliography :
• Scott & Brown
• Cummings
• P Dhingra