Juvenile Angiofibroma

Dr. Roshani Aryal

II Year
LMCTH
Pterygopalatine fossa
• A space between the pterygoid
plates and the posterior wall of
maxillary sinus.
• Shaped like an inverted pyramid
Boundaries
• Medially : Perpendicular plate of
palatine bone
• Laterally: Narrowing to
pterygomaxillary fissure
• Anteriorly :Post wall of maxillary
sinus
• Posteriorly: Med & Lat pterygoid
plates
• Superiorly/Roof- Undersurface of
body of sphenoid.
• Floor-Pyramidal process of palatine
bone
• Inferiorly- None
Contains:-
1)Pterygopalatine ganglion
2)Terminal third of the maxillary
artery
3)Maxillary Nerve –CN V2
4) Greater & deep petrosal nerve.
Pterygopalatine fossa Communications
• Med - with nasal cavity via sphenopalatine F.
• Lat - infratemporal fossa via the pterygomaxillary fissure.
• Ant and sup- with orbit via the inferior orbital fissure
• Post & sup- with Meckel cave & cavernous sinus (of MCF) via the
Foramen rotundum
• Post & inf- with MCF via the vidian(Pterygoid) canal, which transmits
the Vidian nerve
• Foramen Rotundum
Infratemporal fossa
Boundaries :-
1) Lat. -Ramus and condylar process of
the mandible.
2)Med.-Medial pterygoid muscle.
3)Ant. – ends in inferior orbital fissure
behind maxilla.
4)Post. -Carotid sheath(behind) and
styloid apparatus
5)Sup. - Greater wing of the sphenoid
bone.
6)Inf. -No anatomical. Continued down
with neck
• The contents of the fossa are :-
1. Deep lobe of parotid
2. Lateral and Medial pterygoid muscles
3. The maxillary artery and its branches
4. The pterygoid venous plexus and maxillary veins
5. Branches of the mandibular nerve
6. otic ganglion
7. chorda tympani
Introduction
• It is an uncommon, histologically
benign and extremely vascular
tumor that usually arises from the
lateral wall of posterior nasal
cavity close to sphenopalatine
foramen and pterygoid base.
Synonyms :
• Nasopharyngeal angiofibroma
• Bleeding fibroma
• Fibroangioma
• Exclusively disease of Adolescent male
• Mean age at presentations: 14yrs
• Range-> 7-19 yrs ( second decade )
• Rarely present after age of 25yrs
Aetiopathogenesis of JNA
• Tumour origin : Incomplete regression of the first branchial arch artery
(Schick and colleagues).
• It explains the following aspects of JNA :
First branchial arch artery finally recedes close to the pterygoid base
and sphenopalatine foramen regions.
Vascular remnants of this artery are incorporated into the
sphenopalatine and maxillary arteries themselves.
• This embryological vessel is connected to the C4-segment of the ICA :
Persistent vascular remnants of the first branchial arch artery
contacting the ICA are able to account for the observation of a
vascular supply from the ICA, despite an anatomical distance between
this vessel and the JA.
Other proposed theories:

• Ringertz(1938): suggested tumour arose from periosteum of

nasopharyngeal vault.
• Som and Neffson (1940) : - Unequal growth of skull base bones -
hypertrophy of periosteum in response to hormonal influence
• Bensch and Ewing (1941) : tumor probably arise from embroynic
fibrocartilage between basiocciput & basisphenoid
• Brunner: Suggested an origin from the conjoined pharyngobasilar
and buccopharyngeal fascia.
• Marten et al (1948) : Hormonal theory suggesting that these tumor
results from deficiency of androgens or overactivity of estrogens
• Osborn (1959) : they explain that swelling could be d/t either
hamartomas or residual fetal erectile tissue which were subject to
hormonal influence
• Girgis and Fahmy (1973) : Cell nests of Undifferentiated epitheloid
cells at the growing edge of angiofibroma. Appearance was similar to
Paragangliomas
Pathology
Gross-
• Firm, slightly spongy lobulated
swellings
• Nodularity of which increases
with age
• Color: pink to white(part present
in nasopharynx covered by
mucosa so pink and in adjacent
extrapharyngeal area white or
gray)
Cut section
• Reticulated , whorled or spongy
appearance
• Lacks true capsule
• Edge sharply demarcated and
easily distinguishable from
surrounding tissue
Microscopically

• Vascular Component:
endothelium lined spaces
without any muscular or elastic
fibers or the muscular coat is
incomplete.
• Stromal Cells: Stellate or
spindle shaped in appearance and
lay down the collagen tissue.
Blood supply:
• Early stages: sphenopalatine
and maxillary arteries.
• Advanced stages:
 Further branches of the external
carotid artery (ECA) i.e
ascending pharyngeal artery
 Internal carotid arteries (ICAs),
 Vertebral arteries : large JAs
Site of origin

• Earlier: Arise from the roof of

nasopharynx or the anterior wall
of sphenoid bone.
• Now : Arises from the lateral
wall of the posterior nasal cavity
close to the sphenopalatine
foramen and the pterygoid base
Spread:

• Anterior: Nasal cavity +

paranasal sinus
• Posterior: Nasopharynx
• Laterally:
goes to Pterygopalatine fossa.
1. Infratemporal fossa: Cheek
2. Inferior orbital fissure: orbit
Superior: 1. Sphenoid sinus

 Middle cranial fossa

 Cavernous sinus

 Optic chiasma

 Pituitary fossa

2. Skull base

 Middle cranial fossa

Symptoms

1. Nasal obstruction (80-90%) with denasal speech (rhinolalia clausa)

2. Epistaxis (50-60%): Persistent, Painless , Profuse, Paroxysmal,

Unprovoked

3. Headache (25%)

4. Facial swelling (20%): cheek & palatal swelling

• Hearing loss secondary to Eustachian tube obstruction,
• Anosmia and

Anterior Rhinoscopy: abundant mucopurulent secretion together with

bowing of nasal septum towards uninvolved site.

Posterior Rhinoscopy: Pink or red mass in the nasopharynx.

Intraoral palpation in the interval between ascending ramus of
mandible and site of maxilla: Fullness
Signs
• Nasal or nasopharyngeal
mass(80%)
• Frog face
deformity(Proptosis+nasal
bridge broadening)
• Orbital mass(15%)
• Proptosis (10-15%)
• Decreased vision
• Otitis media with effusion due to
ET blockage
• Trismus :involvement of
pterygoid muscle involvement
• Involvement of 2, 3 4 and 6 CN
Investigations:
• Plain lateral skull radiograph :
 Opacity in the sinuses.
 Mass in the nasopharynx
Anterior bowing of the posterior
wall of the maxillary sinus
(Holman-Miller sign/antral sign).
CT scan PNS with contrast

• Extent of tumor

• Bone destruction or displacements

• Anterior bowing of posterior

maxillary wall (Miller Holman’s
antral sign)

• Tumor enhancement
Other Investigations

• M.R.I.: for intra-cranial involvement

• Digital Subtraction Angiography (D.S.A.):

a. Extent of tumor

b. Its vascularity

c. Feeding arteries for embolization

 Biopsy: contraindicated (profuse bleeding)

Fisch Staging System( based on CT imaging
and spread of tumor)
• Type 1 –Tumor limited to nasopharyngeal cavity : bone destruction
negligible or limited to sphenopalatine foramen
• Type 2- Tumor invading pterygopalatine fossa or maxillary, ethmoid
or sphenoid sinus with bone destruction
• Type 3- Tumor invading Infratemporal fossa or orbital region:
a. Without intracranial involvement
b. With intracranial extradural (parasellar) involvement
• Type 4- intracranial intradural tumor
a. Without infiltration of cavernous sinus, pituitary fossa or optic
chiasma
b. With infiltration of cavernous sinus, pituitary fossa or optic
chiasma
Radkowski classification
• Ia- Limited to nose and nasopharyngeal area
• Ib- Extension into one or more sinuses
• IIa- Minimal extension into Pterygopalatine fossa
• IIb- Occupation of pterygopalatine fossa without orbital erosion
• IIc- Infratemporal fossa extension without cheek or pterygoid plate
involvement
• IIIa- Erosion of skull base(middle cranial fossa or pterygoids).

• IIIb- Erosion of skull base with intracranial extension with or without

cavernous sinus involvement
Janakiram Staging system
Differential diagnosis
• Infected Antrochoanal polyp
• Teratoma
• Olfactory Neuroblastoma
• Lobular capillary haemangioma
• Rhabdomyosarcoma
• Squamous cell carcinoma
Treatment
• Surgery
• Radiation therapy
• Chemotherapy
• Hormone therapy

Surgery is the gold standard of treatment

Hormone therapy
• It has been proposed due to the androgen receptors associated with
JNAs in an attempt to decrease tumor size and vascularity.
• Androgen receptor blocker- Flutamide(250mg tds for 6 weeks)

• Estrogen : decrease size and vascularity of the tumor, but has

feminizing side effects.(5-15mg/kg daily for 4-6 weeks)
Radiotherapy
• External beam radiation delivered in several fraction total tumor dose-
30-35Gy at 1.8 Gy per fraction

• Regression of angiofibroma slow

• Take 2-3 yrs for reduction of tumor size

• Local control rate-80-85%

External beam radiotherapy
• Generally reserved for larger and/or unressectable tumors and tumors
that are life threatening do to their location.
• Reason for limited use of radiation:
• Carcinogenic side effects
• Growth retardation
• Temporal lobe radionecrosis
• Panhypopituitarism
• Cataracts
• Radiation induced keratopathy
Chemotherapy
• Chemotherapy alternative therapy
• Unresectable tumor had chemotherapy for palliation
• Doxorubicin
• Decarbazine
• Vincristine
Surgical Approaches

1. Trans-palatal approach (Wilson) : Small tumor in nasopharynx

2. Sublabial + Trans-palatal approach (Sardana): large tumor of

nose + PNS + nasopharynx

3. Intranasal endoscopic approach :small tumour in nose / PNS /

nasopharynx , pterygopalatine fossa and even for larger tumors.

• Lateral Rhinotomy with medial maxillectomy
• Transnasal/Transantral:
 Mid Facial Degloving with Maxillary Osteotomies
Weber Ferguson inscision
• Denkers Approach
• Infratemporal fossa with or without craniotomy: extension into
infratemporal fossa
Pre-op reduction of tumor vascularity

1.Embolization of feeding arteries: with Gelfoam

2. Oestrogen therapy: Diethylstilbestrol (2.5 - 5 mg orally t.i.d. for 3 - 6 wk)

3. Testosterone receptor blocker: Flutamide(250mg tds for 6 weeks)

4. Pre-operative radiotherapy

5. Cryotherapy of tumor
PREOPERATIVE EMBOLIZATION
• 24 to 72 hours preoperatively
• Gelfoam or polyvinyl alcohol foam
• Gelfoam: resorbed in approximately 2 weeks
• Polyvinyl alcohol: more permanent
• Efficacy
• Stage I patients reduced from 840cc to 275cc blood loss
• Complications
• Brain and ophthalmic artery embolization
• Facial nerve palsy
• Skin and soft tissue necrosis
DSA before and after embolization
ENDONASAL ENDOSCOPIC
TECHNIQUES
• Reduced intraoperative blood loss,
• Fewer postoperative complications and
• Reduced length of hospital stay.

Not recommended in situations of :

broad skull base infiltration,
 extensive blood supply from the internal carotid artery,
 encasement of the internal carotid artery and brain infiltration .
Strategy of endoscopic resection :
1.Opening of the maxillary sinus and exposure of its posterior wall are
performed early during surgery.
Size of the tumor dictates the size of the medial maxillectomy.
The bony posterior wall of the maxillary sinus is removed with
‘through cutting’ punches or drills avoiding trauma to the periosteum
in the pterygopalatine fossa before resection of the bone is completed
Sphenopalatine and maxillary arteries
are identified and clipped or
coagulated.
Early control of main feeding vessel
assists further tumor resection.
An alternative in early JAs is to push
the tumor gently in a medial direction
out of the pterygopalatine and
infratemporal fossae and to identify
the main feeding vessel.
In advanced JAs, the tumor can be
pushed medially with a finger placed
externally at the lateral border of the
maxillary sinus below the jaw
2. Resection of the posterior nasal septum is done to widen the access
and to define tumor margins at the nasal septum.
3. The anterior sphenoid sinus wall is removed in order to expose the
tumor within the sphenoid sinus. For this purpose, bilateral opening of
the sphenoid sinus is usually necessary.
4.Finally, the tumor is dissected directly off the pterygoid base,
pterygoid canal regions and the clivus.
5.It is of utmost importance to drill at the pterygoid base and the clivus
at the end of surgery in order to avoid residual tumor.
Trans-palatal approach
• A curved incision bowed forwards
is made with a sharp knife between
the tuberosities, keeping internal to
the greater palatine foramina.
• incision which extends just in front
of the posterior margin of the hard
palate: for assess to postnasal space
• incision should be carried well
anteriorly: for assess to nasal
passage
Elevation of flaps
• the edge of the mucoperiosteal
flap on the anterior side of the
incision.
• The posterior flap is then
separated completely from the
undersurface of the hard palate.
• small right-angled dissector is
used to separate the flap from the
posterior margin of the-bone
Separation of soft palate:
• Mucosa on the upper surface of
the palate is divided transversely
and the postnasal space examined.
• Anteriorly: exposure may be
obtained by a midline incision
through the mucoperiosteum and
removal of as much bone of hard
palate as possible.
• Posteriorly: extended
posterolateral just medial to the
pterygomandibular raphe and the
pterygoid hamulus
Exposure of postnasal space:
• Depression of the soft palate will
give adequate exposure of the
postnasal space in view of
the posterior edge of the nasal
septum, the posterior ends of the
turbinates, the roof and lateral
walls of the postnasal space, and
the Eustachian orifices.
Complications
• Recurrence (younger pt more likely recurrence)
• Surgically induced infraorbital nerve sensory deficit(midfacial
degloving)
• Prolonged nasal crusting
• Ocular problems
• Displacement of globe due to loss of bony support
• Ophthalmoplegia
• Visual loss
Late complications
• Relatively common
• Following radiotherapy
• Growth retardation
• Panhypopituitarism
• Temporal lobe necrosis
• Cataracts
• Radiation keratopathy
• Skin, thyroid, nasopharyngeal malignancies(first 10-15 yrs
Management of recurrent tumor
1. Observation: may regress spontaneously
2. Revision surgery and removal: if recurs even after revision surgery
radiotherapy may be considered
3. Radiation: reduces blood supply and tumor subsides over time.
• Thank you……