This document provides information on juvenile nasopharyngeal angiofibroma (JNA), a benign but locally invasive vascular tumor that most commonly occurs in adolescent boys. It discusses the presentation, diagnostic approach, pathology, theories of pathogenesis, surgical techniques for resection including endoscopic and open approaches, preoperative embolization, and hormonal therapies for JNA. Imaging studies such as CT and MRI are useful for diagnosis by identifying the vascular tumor's site of origin and patterns of growth. Complete surgical resection is the main treatment, with the approach depending on tumor size, location and prior embolization.

1. Dr. PARTH MAKWANA
ASSISTANT PROFESSOR-ENT
DEPARTMENT
DR. M.K. SHAH MEDICAL COLLEGE
AND RESEARCH CENTRE,AHMEDABAD.
JUVENILE
NASOPHARYNGEAL
ANGIOFIBROMA

2. Introduction:
• Nasopharynx is an embryologic confluence of end of nasal
structures and beginning of pharynx
• Symptoms generally arise late because of capacity for tumor
growth and expansion
• Diagnostic inaccessibility of nasopharyngeal (NP) area,
tumors can be difficult to detect

3. DIAGNOSTIC APPROACH FOR
NASOPHARYNGEAL MASSES
• Presentation of an NP tumor is variable and ranges from ear,
nose, and throat symptoms to neck masses and cranial nerve
palsies
• Age and gender are important in D/D
• Usually due to adenoidal hypertrophy in pediatric but JNA
should be strongly considered in teenage boys
• In adults, NP malignancy should be default diagnosis
• In several Asian regions it is common and usually of epstein-
barr virus (EBV)–related, undifferentiated carcinoma

4. • Imaging studies before biopsy of mass.
• Histology of the NP mass dictates the management

5. • THORNWALDT CYST OR BURSA :
Next to adenoidal hypertrophy, it is m/c
epithelial growth in NP area
Result of a cleavage line between nasal
and pharyngeal embryologic processes
(Rathke pouch)
Usually asymptomatic
D/D should include
oMeningocele or meningoencephalocele
oGenerally do not need to be removed,
nor is biopsy necessary if diagnosis is
apparent

6. JUVENILE
NASOPHARYNGEAL
ANGIOFIBROMA

7. •Introduction :
Usually occurs in adolescent boys, thus commonly called
juvenile nasal angiofibroma (JNA)
<1% of all head and neck tumors
Benign but Locally infiltrative
Sarcomatous, malignant transformation is extremely rare
and attributable to prior radiotherapy
Slow-growing vascular tumor, arises in area of
Sphenopalatine foramen at root of pterygoid process on
lateral nasal wall
Maxillary sinus is m/c site for extra-NP angiofibroma, occur

8. Main blood supply : Internal maxillary artery
Others : Ascending pharyngeal artery, vidian artery,
inferomedial trunk or inferior hypophyseal artery (br of ICA)
rarely vertebral artery

9. •Spread:
• Tumor migrate beneath mucus membrane of NP, displacing it
downward in the process
• Grows in adjacent anatomical sites that offer less resistance
and invade cancellous bone of basisphenoid
• Medially  nasopharynx, nasal fossa, and eventually towards
contralateral side
• Laterally  infratemporal fossae, via an enlarged pterygo-
maxillary fissure  typical anterior displacement of posterior
maxillary wall  contact with masticatory muscles and cheek
• Posteriorly  ICA via vidian canal, cavernous sinus via foramen
rotundum and orbital apex via inferior orbital fissure

13. • White dotted line : spread
into cancellous bone of
basisphenoid along vidian
canal
• White arrowheads : spread
deep into pterygomaxillary
fossa toward masticatory
muscles, with ant.
Displacement of post.
Maxillary wall
• Black arrows : right vidian
nerve

14. • Bone involvement occurs via two main mechanisms:
1. Resorption by direct pressure of preexisting bony structures
with osteoclastic activation
2. Direct spread along perforating arteries into cancellous root
of pterygoid process
• In advanced cases
Extends posteriorly towards upper-middle of clivus
Laterally within greater wing of sphenoid
Erosion of the inner table of middle cranial fossa
Infiltration of dura is very rare

15. •Pathologic features:
Gross pathology usually shows a sessile, lobulated, rubbery,
dark red to tan gray mass that can be large
Color varies from pink to white
On section  reticulated, whorled or spongy appearance,
lacks true capsule but sharply demarcated edges
Microscopically, Composed of an admixture of vascular
tissue and fibrous stroma
Vessel walls lack elastic fibers and have incomplete or
absent smooth muscle -- tendency to bleed

16. •Factors that play a role in the growth :
• Chromosomal abnormalities: gains at chromosomes 4, 6, 8,
and X and losses on chromosomes 17, 22, and Y are most
frequent
• Increased prevalence of JA (25 times) in patients with
familial adenomatous polyposis (FAP), condition that is
associated with mutations of adenomatous polyposis coli
(APC) gene
This gene regulates beta-catenin pathway which
influences cell to cell adhesion. Mutations of beta-catenin
have been found in sporadic and recurrent JAs.

17. • Angiogenic growth factor, vascular endothelial growth
factor (VEGF) has been found localized on both endothelial
and stromal cells
• Overexpression of insulin-like growth factor II (IGFII)
• Hormonal factors: controversial

18. •Theories associated with its aetiopathogenesis:
Ringertz theory (1938) – JNA arose from the periosteum of
NP vault
Som & Neffson (1940) – inequalities in the growth of bones
forming skull base resulted in hypertrophy of the underlying
periosteum in response to hormonal influence.
Bensch & Ewing (1941) – tumour probably arose from
embryonic fibro cartilage between basi-occiput and basi-
sphenoid.
Brunner (1942) – suggested origin from conjoined
pharyngobasilar and buccopharyngeal fascia.

19. Sternberg (1954) – proposed that JNA could be a type of
hemangioma like a cutaneous hemangioma seen in children
which regresses with age
Osborn (1959) – it could be due to either a hamartoma or
residual fetal erectile tissue which were subjected to
hormonal influence
Girgis & fahmy (1973) – observed cell nests of
undifferentiated epitheloid cells or “zellballen” at the
growing edge of angiofibromas and so considered it as a
paraganglioma

20. ‘Branchial arch artery’ theory:
• Tumour origin is based on an incomplete regression of the first
branchial arch artery
• Able to explain different aspects of JNA:
1. During embryological development, it finally recedes close
to pterygoid base and sphenopalatine foramen regions
2. Vascular remnants of this artery are incorporated into
sphenopalatine and maxillary arteries themselves

21. Its connection to c4-segment of ICA accounts for vascular
supply from ICA, despite an anatomical distance between
this vessel and the JA
Elucidates common finding of residual tumour at pterygoid
base and clivus

22. •Clinical features:
Ages : 10 and 25 in males
Cardinal symptoms : nasal obstruction and intermittent
epistaxis
Others:
Chronic anaemia
Hyposmia or anosmia
Nasal intonation of voice
Deafness and otalgia
Headache

23. • In extensive lesions 
Nasal bones become splayed out
Swelling in temple and cheek
• Intraoral palpation between ascending ramus of mandible
and side of maxilla may reveal thickening of disease which
has crept around back of the antrum
• Trismus and bulging of parotid if Impaction of bulky mass in
infratemporal fossa
• Proptosis if orbital fissures are penetrated
• Frog face appearance if massive escape of diseases

24. • Diagnosis:
Endoscopic examination:
oRubbery vascular mass that
protrudes into anterior nasal
space
oMay have excessive bleeding on
contact
oSince epidemiologic and
endoscopic findings are typical,
biopsy is absolutely
contraindicated because of a
considerable and undue risk of
massive hemorrhage

25. Diagnosis on imaging is based on three factors
1. Site of origin
2. Hypervascularization after contrast enhancement
3. Patterns of growth
CT scan with contrast:
o Enhancing soft tissue mass arising from NP or lateral wall
of nose. Pterygopalatine fossa may be widened by tumor ,
bone erosion not present in general
MRI: Vascular tumor with flow voids within mass that
enhance on gadolinium imaging

26. Signs in CECT:
• HOLMAN MILLER SIGN: 80%
• Anterior bowing of
posterior wall of maxillary
antrum
• HONDOUSA SIGN:
• Widening of gap between
ramus of mandible &
maxillary body

27. • RAM HARAN SIGN:
• Quadrilateral appearance of
pterygoid wedge
• CHOP STICK SIGN:
• Post op appearance of
medial & lateral pterygoid
plates as two separate sticks
due to drilling & removal of
pterygoid wedge

28. • Pathognomonic sign : erosion of upper medial pterygoid
plate which is found in 98%
• Differential diagnosis includes other hypervascularized
lesions:
Hemangiopericytoma
Lobular capillary hemangioma
Paraganglioma

29. Rich vascularity of tumour gives rise to typical small dotted flow voids
(salt pepper appearance)

30. •Angiography:
b/l vascular supply is around 36% hence both carotid systems
require angiographic evaluation
1. Assess vascular supply of JA
2. Allow embolization of feeding vessels prior to surgery
More as a surgical treatment adjuvant via possibility of
embolization rather than diagnostic tool
MR angiography is least invasive form of vascular imaging
that will show size feeding vessels
Vascular blush seen in postnasal space and adjacent areas is
diagnostic

31. • However, final proof of diagnosis is histologic.

32. •Staging:
Several staging systems have been proposed –
Fisch
Chandlers
Andrews
Radkowski
• Fisch  most robust and practical. Defines clearly which
surgical approach is required
• Radkowski  appeals to those involved with management of
smaller tumours as there are more subdivisions

35. • Chandler’s staging of JNA:
A. Stage 1 : Tumor is confined to nasopharynx
B. Stage 2 : Extends into nasal cavity or sphenoid
C. Stage 3 : Tumor involves maxillary sinus, ethmoid sinus,
infratemporal fossa, orbit, cheek, and cavernous
sinus
D. Stage 4 : Tumor is intracranial

36. •General surgical principles:
• The surgical approach is dependent on
1. Tumour location and extent
2. Pattern of vascular supply
3. Effectiveness of embolisation
4. Facial skeletal maturity
5. Experience of the surgical team
• JNAs may be resected by endoscopic, open or combined
(endoscopic & open) techniques

37. •Endoscopic sinus surgery techniques
Mainstay of surgical resection in present era
• Advantages:
Magnified view of lesion and related anatomical structures
from multiple angles  better identification of interface
between lesion and soft tissues or adjacent bone strs
Allows more accurate and complete dissection and better
control of bleeding

38. •Indications:
Tumours involving nasal
cavity, paranasal sinuses,
and nasopharynx
Tumours with only medial
infratemporal fossa
involvement or extradural
parasellar involvement with
limited intracranial
extension
Facilitation of open
approaches
•Relative
contraindications:
Lateral infratemporal fossa
involvement,
Extensive parasellar
extension,
Encasementof the optic
nerve,
Intradural spread, or
cavernous sinus involvement

40. •Complications:
Pain
Bleeding
Infection
Hyposmia
Synechiae
Orbital Injury, loss of vision
Cerebrospinal fluid leak
Intracranial injury
Tumour recidivism if margins are not cleared

41. •Open approaches:
Tumours that extend to infra-temporal fossa
Tumours with intradural extension
In centres that lack endo-scopic expertise
In conjunction with endo-scopic resection e.g. Anterior
antrostomy (caldwell-luc) may be employed to gain access to,
and clip, internal maxillary artery

42. • Includes:
Medial maxillectomy
Le fort 1 osteotomy
Transpalatal
Maxillary swing
Infratemporal fossa
Facial translocation

43. •Intraoral transpalatal approach: (Wilson’s)
 Allows for access to NP (for small lesions)

47. • Medial maxillectomy approach:
For tumors extending to pterygopalatine fossa
Soft tissue elevation preferably with a midfacial degloving
incision. Advantages are:
Excellent exposure of lesion
Direct control of internal maxillary artery in pterygopalatine
fossa
Very satisfactory cosmetic outcome
• Commonest complication – Vestibular stenosis, Infraorbital
N. Injury
lateral rhinotomy only required when superior parts of
ethmoids are to be dissected

48. Midfacial degloving approach:

49. Lateral rhinotomy:

50. •Le fort type 1 osteotomy:
Will allow an inferior approach to maxillary and ethmoid
sinuses and to pterygopalatine canal
For extension outside NP into paranasal sinuses
Complication – Malocclusion, Necrosis of maxilla

51. • Facial translocation approach:
Affords a large window of access
For lesions that involve infratemporal fossa or large lesions
that involve majority of sinuses

52. Adjuvant Therapies:

53. • Preoperative embolization:
• Reduces intra-operative bleeding
• May shrink the tumor
• Enables better visualization of surgical field (endoscopic
setting), facilitates dissection
However, risk of recurrence increased
Tumour shrinkage makes borders ill-defined leads to
inadequate resection
Done 24–72 hours pre-operatively
Stroke, visual loss, facial paralysis, or carotid dissection may
occur if feeders from ICA embolized

54. Gelfoam
Polyvinyl alcohol foam
Coils
Micro-particles or
Liquid glue
Ethylene–vinyl alcohol co-polymer (Onyx®):
1. shows deep penetration into tumor
2. more extensive tumor necrosis
3. embolization of large portions of tumor via fewer
catheterizations
4. safe withdrawal of catheter

56. •Limiting factors for successful embolization:
Vessel tortuosity
Vasospasm
Prior sacrifice of internal maxillary artery or external carotid
artery
Most serious complication : loss of vision secondary to
occlusion of central retinal artery
direct intra-tumoral embolization under, radiographic
control if feeder not accessible

57. •Hormonal Therapy:
Tumor enlargement with administration of testosterone and
shrinkage with estrogen therapy
Flutamide (2-methyl-n-[4-nitro-3{trifluoromethyl}phenyl]
propanamide), orally active non-steroidal androgen
antagonist
Gates et al.  tumor reduction of 44% in 4 of 5 cases
receiving 6 week treatment course
Labra et al.  tumor reduction of only 11.1% in 6 cases
receiving 3 week treatment course
Current recommendation  6 week course of flutamide as
adjuvant therapy in post-pubertal patient

58. •Radiation:
For tumors that recur after surgery and those with intracranial
extension, where clearance of margins may be difficult
3000 – 5500 cGy in 15 -18# over 3 - 3.5 wks
Tumour regression is very slow (over 2-3 year) and is by
radiation vasculitis and occlusion of vessels by perivascular
fibrosis
• Intensity-modulated radiotherapy:
Creates conformal dose distribution to minimize dose to
adjacent tissues
Has been used to deliver 3,400–4,500 cGy to patients with
extensive disease involving cavernous sinus and skull base

59. • Proton RT 
Creates even tighter dose distribution
Further reduce risk of late effects
• Stereotactic radiosurgery 
For minimal, well-defined residual tumor following
incomplete resection
• Disadvantages :
Risk of marginal miss because of necessarily tight dose
distribution
Higher risk of late complications

60. •Complications of radiotherapy:
Up to 33%
Growth retardation
Panhypopituitarism
Temporal lobe necrosis
Cataracts
Radiation keratopathy
Thyroid and nasopharyngeal malignancies

61. •Hypotensive Anesthesia:
Provide a relative bloodless field  tumor removal is easier,
quicker and satisfactory

62. Complications:
• Recurrence 
Most common complication (25 %)
More likely in patients with advanced disease and in those
treated by inexperienced surgeons
Younger patient
Usu. Develop as a consequence of invasion of basisphenoid
Disease-free status five years after primary surgery probably
represents cure

63. • Infraorbital nerve sensory deficits and nasal vestibular
stenosis  complication of mid-facial degloving,.
• Prolonged nasal crusting  may develop into ozaena
(regular nasal douching with saline and the use of glucose in
glycerine drops can alleviate)
• Ocular problems with more extensive resections
1. Displacement of globe caused by loss of bony support,
2. Ophthalmoplegia
3. Visual loss

64. • Trigemino-cardiac reflex:
Characterized by –
1. Bradycardia / Asystole
2. Hypotension
3. Apnea
4. Gastric Hypermotility
Incidence – 4 %
Cause – Manipulation of PPF, ITF, NP Mucosa
To prevent – 4% Xylocaine pack in PPF , ITF
If occurs – Stop all manipulation, IV Crystalloids, wait for 10-
15 min

65. • Management of ICA injury:
Don’t panic Don’t pack
Use 2 suctions
1 – 2 cm3 muscle harvested from thigh or abdomen
Crushed & placed over bleeding point for at least 3-5 min →
activates platelet fibrin plug
Reinforce with surgicel
If still not controlled → Endovascular intervention by
angiography team

66. References:
1. Scott browns ORL & HNS 6th, 7th and 8th edition.
2. Cummings ORL & HNS 6TH edition.
3. López, Fernando, et al. "Nasal juvenile angiofibroma: Current
perspectives with emphasis on management." 2017
4. Nicolai P, Schreiber A, Bolzoni Villaret A. “Juvenile
angiofibroma: evolution of management.” 2011
5. Open atlas. JUVENILE NASOPHARYNGEAL ANGIOFIBROMA
SURGERY.