Angiofibroma by Sanjiv Kumar

Juvenile Nasopharyngeal
ANGIOFIBROMA
Contributed by :- Dr Sanjiv Kumar, MS(ENT) std, Patna, India
For more presentations, please visit www.nayyarENT.com
1 www.nayyarENT.com 7/23/2012
Juvenile Nasopharyngeal Angiofibroma
Benign highly vascular tumor
Locally invasive, submucosal spread
Vascular supply most commonly from internal maxillary

artery
Also: Ascending pharyngeal, Ascending palatine, Internal
carotid, external carotid, common carotid,
JNA Facts and Statistics
< 0.5% of all head and neck tumors
Occurring almost exclusively in males
Average age of onset = 15 years (10-25)
Intracranial Extension between 10-20%
Recurrence Rates as high as 50%
Origin
It takes origin from the superior lip of the sphenopalatine
foramen (at posterolateral nasal wall) at the junction of the
pterygoid process of the sphenoid bone and the sphenoid
process of the palatine bone.
some believe it to originate from pterygopalatine fossa
Routes of Spread
Medial growth
Nasal cavity
Nasopharynx
Lateral growth
Pterygopalatine fossa
Vertical expansion through inferior orbital fissure to orbit possible
Infratemporal fossa
Superior expansion through pterygoid process may involve middle cranial fossa
Lateral and posterior walls of sphenoid sinus can be eroded
Cavernous sinus may be involved
Pituitary may be involved
It tends to extend along natural foramina and fissures not invading bone but often eroding it by
pressure atrophy
Histology
Myofibroblast is cell of origin
Consist of proloferating, irregular vascular channels within
fibrous stroma.
Pseudocapsule made of fibrous tissue
Blood vessels lack a smooth muscle & elastic fibre-cause for
sustained bleeding. (irregular or incomplete smooth muscle coat
is present in large vessel near origin point of JNA)
Has vascular and stromal component.
Stromal component is made of plump cells (mainly spindle cell
that give rise to varying amount of collagen & also by stellate
cell)
Genetics
Overexpression of IGF-2 is found in JNA (53%) associated with tendency to
recurrence & poor prognosis.
IGF-2 is situated at chromosome 11p-site for the target for genomic imprinting
so expressing paternal allele only..
Angiogenic growth factor (VEGF) found in both vascular and stromal component
of JNA.But VEGF expression donot seem to bear any relation to the stage of the
JNA; ie, its degree of aggressiveness
JNA also a/w 25 times more frequently in patients with FAP(a/w germline
mutation in APC gene on chr. 5q) which is involved in sporadic & recurrent JNA.
Although evidence of adenomatous polyposis coli (APC) gene mutations is not
found in stromal component of JNA.
APC gene regulate beta catenin pathway.
Beta catenin influence cell to cell adhesion and also acts as coactivator of androgen
receptor increased sensitivity of androgen on tumour.
Genetics continue..
At molecular genetic level, involvement of 13q detected,
suggesting link with spindle cell lipoma & some
myofibroblastoma.
Tumour has androgen receptor (in 75% cases) which is
present in vascular and stromal component and progesteron
receptor but no oestrogen receptor
Transformation of fibroblasts into endothelial cells caused by
the angiogenic capacity of the c-MYC protein building up an
immature vascular network appears possible in JNAs.
Diagnosis
Diagnosis
History
Physical Exam
Radiological study
CT Scan
MRI
Angiogram
Classical Presentation
Nasopharyngeal mass in teenage or young adult exclusively

in male.
Unilateral progressive Nasal obstruction (80-90%).
Recurrent unilateral epistaxis (45-60%)
Other JNA Symptoms
Other common symptoms --

Swelling Of The Cheek
Conductive hearing Loss and secretory otitis media
secondary to Eustachian tube block
Dacrocystits
Rhinorrhea
Hard And Soft Palate Deformity
Hyposmia Or Anosmia
Other JNA symptoms contiue..
Advanced Lesions May Causes
Facial pain,orbital proptosis, diplopia, visual loss is due to
invasion of orbit and cavernous sinus.
Headache due to blockage of PNS
Cranial Neuropathy
Appearance
Smooth lobulated mass in the nasopharynx or lateral nasal

wall
Pale, purplish, red-gray, or beefy red
Compressible
Differential diagnosis of mass in nose
and nasopharynx
Hemangioma
Choanal polyp
Nasopharyngeal carcinoma
Angiomatous polyp
Nasopharyngeal cyst
Hemangiopericytoma
Rhabdomyosarcoma
Chordoma
Juvenile nasopharyngeal angiofibroma
Radiology
Radiological Studies
Plain film
-No longer play a role in the work up of a suspected JNA,
however they may still be obtained in some instances during
assessment of nasal obstruction, or symptoms of sinus obstructions.
Findings
-visualisation of a nasopharyngeal mass
-Opacification of the sphenoid sinus
-Anterior bowing of the posterior wall of the maxillary antrum
(Holman-Miller Sign)
-Widening of the pterygomaxillar fissure and pterygopalatine

fossa
-Erosion of the medial pterygoid plate
Holman-Miller sign
Radiological studies continue
CT Scan
Excellent for delineating bony changes
Lesion enhances with contrast on CT
Lobulated non encapsulated soft tissue mass is demonstrated centred on the
sphenopalatine foramen (which is often widened)
Bowing the posterior wall of the maxillary antrum anteriorly
MRI
Excellent at evaluating tumour extension into the orbit and
intracranial compartments.
Differentiate tumor from other soft tissue structures
Angiogram
Evaluation of feeding blood vessels, for selective embolisation.
Coronal CT
Widening of left
sphenopalatine foramen
Lesion fills left choanae
Extends into sphenoid sinus
External Carotid Arteriogram
Feeding vessel = Internal Maxillary Artery

Blood Supply of these tumours is usually by
External carotid artery : majority

internal maxillary artery
ascending pharyngeal artery
palatine arteries
Internal carotid artery : less common, usually in larger tumours

sphenoidal branches
ophthalmic artery
Staging
Exact extent or stage of the tumour can only be determined
by a combination of CT & MRI and this is vital when
planning for surgical resection.
Fisch Staging
1.Tumour limited to the nasopharyngeal cavity; bone destruction
negligible or limited to the sphenopalatine foramen
2. Tumour invading the pterygopalatine fossa or the maxillary, ethmoid
or sphenoid sinus with bone destruction
3. Tumour invading the infratemporal fossa or orbital region:
(a) without intracranial involvement
(b) with intracranial extradural (parasellar) involvement
4. Intracranial intradural tumour:
(a) without infiltration of the cavernous sinus, pituitary fossa or optic
chiasm
(b) with infiltration of the cavernous sinus, pituitary fossa or optic
chiasm
Radkowski Staging -1996
1a-Limited to the nose and nasopharyngeal area
1b-Extension into one or more sinuses
2a-Minimal extension into pterygopalatine fossa
2b-Occupation of the pterygopalatine fossa without orbital
erosion
2c-Infratemporal fossa extension without cheek or pterygoid plate
involvement
3a-Erosion of the skull base (middle cranial fossa or pterygoids)
3b-Erosion of the skull base with intracranial extension with or
without cavernous sinus involvement
nerci et al. -2006
(I) Nose, nasopharyngeal vault, ethmoidal-sphenoidal sinuses, or minimal

extension to PMF
(II) Maxillary sinus, full occupation of PMF, extension to the anterior cranial fossa,
and limited extension to the infratemporal fossa (ITF)
(III) Deep extension into the cancellous bone at the base of the pterygoid or the
body and the greater wing of sphenoid, significant lateral extension to the ITF
or to the pterygoid plates posteriorly or orbital region, cavernous sinus
obliteration
(IV) Intracranial extension between the pituitary gland and internal carotid artery,
tumor localization lateral to ICA, middle fossa extension, and extensive
intracranial extension
Snyderman et al. -2010
(I) No significant extension beyond the site of origin and remaining medial to the
midpoint of the pterygopalatine space
(II) Extension to the paranasal sinuses and lateral to the midpoint of the
pterygopalatine space
(III) Locally advanced with skull base erosion or extension to additional
extracranial spaces, including orbit and infratemporal fossa, no residual
vascularity following embolisation
(IV) Skull base erosion, orbit, infratemporal fossa, Residual vascularity
(V) Intracranial extension, residual vascularity
M: medial extension
L: lateral extension
Treatment
Treatment Options
Surgery
Gold standard
Radiation therapy
Reserved for unresectable, life-threatening tumors
Chemotherapy
Recurrent tumors with previous surgery and radiation
Hormone therapy
Estrogens and antiandrogens used to decrease tumor size and
vascularity
Surgical Approaches
Endoscopic transnasal
Transpalatal
Denker approach
Facial translocation
Medial maxillectomy
Infratemporal fossa with or without craniotomy
Preoperative Embolization
24 to 72 hours preoperatively to avoid collateral vascularisation
Most of the authors use resorbable particles such as gelfoam or dextran microspheres
or short duration non-absorbable such as Ivalon, ITC contour or Terbal,
polyvinylalcohol particles, which last longer and are more efficient
Efficacy
Stage I patients reduced from 840cc to 275cc blood loss
Complications
ophthalmic artery embolization
Facial nerve palsy
Skin and soft tissue necrosis
occlusion of the central retinal artery and consequent temporary blindness,
oronasal fistula due to tissue necrosis,
occlusion of the middle cerebral artery followed by stroke
some authors consider preoperative embolization to provide no benefit, or even to increase
the risk of recurrence.
Surgical Approaches
Transpalatal
Denker approach
Medial maxillectomy
Endoscopic Transnasal
Resection preserves both the anatomy and physiology of the

nose, requires less rehabilitation days after surgery, and is
highly successful for selected patients
Middle turbinectomy may be performed for improved exposure
Middle meatus antrostomy

Resection of posterior maxillary wall
Sphenopalatine artery ligation

Tumor resection from pterygopalatine fossa
Surgical Approaches
Transpalatal
Denker approach
Medial maxillectomy
Transpalatal
Soft palate is split and retracted

Transpalatal
Hard palate resection for enhanced exposure

Transpalatal
Palatine bone and inferior aspect of pterygoid plate resected

Surgical Approaches
Transpalatal
Denker approach
Medial maxillectomy
Denker Approach
It is effective for angiofibromas confined to the nasal cavity and nasopharynx with small
extensions in the infratemporal fossa.
large tumor extension in the infratemporal fossa can be effectively approached in
combination with a midfacial degloving technique.
Wide anterior antrostomy

Removal of ascending process of maxilla
Removal of inferior half of lateral nasal wall
Surgical Approaches
Transpalatal
Denker approach
Medial maxillectomy
Midface Degloving with Maxillary
Osteotomies
Gingivobuccal incision
Nasal intercartilaginous incisions with transfixion incision
Surgical Approaches
Transpalatal
Denker approach
Medial maxillectomy
Maxillectomy
Maxillary osteotomies
Sagittal osteotomy
Alternative Approaches to Nasal
Cavities and Paranasal Sinuses
Lateral Rhinotomy
Weber-Ferguson incision
Weber-Ferguson with Lynch extension
Weber-Ferguson with lateral subciliary extension
Weber-Ferguson with subciliary extension and supraciliary
extension
Surgical Approaches
Transpalatal
Denker approach
Medial maxillectomy
Surgical Planning
Smaller tumors (IA, IB, IIA, IIB, IIC)
Trans-nasal endoscopic-tumors involving the ethmoid, maxillary,

or sphenoid sinus, the sphenopalatine foramen, nasopharynx,
pterygomaxillary fossa and have limited extension into the
infratemporal fossa are amenable to endoscopic resection.
Transpalatal-provides access to the nasopharynx, sphenoid,

sphenopalatine foramen and posterior nares. It avoid external scar and
does not effect the facial growth but oronasal fistula is a more common
side effect
Transantral: lesions extending laterally up to

pterygopalatine fossa
Surgical planning continue..
Larger tumors (IIIA, IIIB)
Lateral rhinotomy
Midfacial degloving- provides good exposure to the
maxillary antrum, nose, pterygopalatine fossa and infratemporal
fossa. There will be no deforming scar on face because of the
use of a sub labial incision, but needs extensive removal of
bones from the anterior, posterior, medial and lateral walls of
maxillary antrum
Extensive resection with higher morbidity
Limited resection with higher recurrence
Transnasal endoscopic technic has great advantage because it
preserves both the anatomy and physiology of the nose,
requires less rehabilitation days after surgery, requiring less
days of hospitalization and is less subject to hospital infections
Changing Technique
On Retrospective chart review of surgical intervention
Marked shift towards endonasal procedures while tumor stages

remained the same
Endonasal approach contraindicated in Stage IV and some Stage III
cases
May be used in conjunction with other approach in these cases
Surgical Approach
Surgical Technique
Approach (65 pts) Endoscopic Open
Expected Blood Loss 225 ml 1250 ml
Complications 1 30
Length of Stay 2 days 5 days
Recurrence Rate 0% 24 %
Surgical Technique
Transnasal endoscopic approach can replace transpalatal
approach
Becouse of less morbidity
Patients with IIA through IIIA previously treated with lateral

rhinotomy may be treated with transnasal endoscopic
approach
Tumors extending to infratemporal fossa require lateral

rhinotomy and degloving for optimal exposure
Greater morbidity
57 www.nayyarENT.com . 7/23/2012
Surgical Technique
Surgical limitations of endoscopic resection evaluated in
literature review
Extremely limited IIIA and IIIB may be approached
endoscopically
Preoperative embolization recommended, but some surgeons
dont recomend
Gamma Knife Surgery
2 case reports used as booster treatment for residual tumor after
surgery
No change in tumor size of one patient, regression in other patient
1 case report used as primary treatment modality successfully
External Beam Radiation
Retrospective review of efficacy of radiation as primary
treatment modality for JNA
15 patients received 3000-3500 cGy
Recurrence rate of 15%
Conclusion-External beam radiation is effective mode of
treatment of advanced JNA
Retrospective review of efficacy of radiation as primary
treatment modality for JNA
27 patients received 3000-5500 cGy
Recurrence rate of 15% 2-5 years post-treatment
External beam radiation is effective mode of treatment of
advanced JNA
Long-term sequelae of concern
Growth retardation, panhypopituitarism, temporal lobe
necrosis, cataracts, radiation keratopathy
Retrospective review reported 2 cases out of 55 patients

developing secondary malignancies
Thyroid carcinoma 13 years after receiving 3500cGy
Basal cell carcinoma of skin 14 years after receiving 3500cGy
initially, then 3000cGy for recurrence
Chemotherapy
Chemotherapy is alternative therapy
unresectable tumor had chemotherapy for palliation
Adriamycin, decarbazine, vincristine,actinomycin-d and
cyclophosphamide
Extensive regression of tumor
Possible alternative to radiation?
Hormonal Therapy
Androgen and progesteron receptors have been identified
with varying frequencies in JNAs
Some JNAs lack these receptors
Limited utility
Delays surgery
Feminizing side effects
Cardiovascular complications
Hormonal Therapy
Treatment with flutamide(potent nonsteroidal androgen
receptor blocker), tumor shrinkage of up to 44 % was
reported by Gates et al
diethyl stilbestrol
Before and after measurement comparison made using CT
scan
No statistically significant difference in size
No difference in blood loss
No advantage with treatment
Surveillance
Frequent physical examinations
CT Scan / MRI
Recurrence Rates
Post-operative
Stage I and II = 7%
Stage III = 39.5%
Tumor stage extracranial vs. intracranial tumor

Extracranial = 5%
Intracranial = 50%
Conclusions
Rare, benign, vascular tumor found almost exclusively in young

males
Surgery is the gold standard with a trend towards endoscopic

approaches
Frequent follow-up after treatment is necessary
Thank You
For more presentations, please

visit www.nayyarENT.com

Angiofibroma by Sanjiv Kumar

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Juvenile Nasopharyngeal

Contributed by :- Dr Sanjiv Kumar, MS(ENT) std, Patna, India

For more presentations, please visit www.nayyarENT.com

Locally invasive, submucosal spread

Vascular supply most commonly from internal maxillary

Occurring almost exclusively in males

Average age of onset = 15 years (10-25)

Intracranial Extension between 10-20%

Recurrence Rates as high as 50%

some believe it to originate from pterygopalatine fossa

Nasopharyngeal mass in teenage or young adult exclusively

Other common symptoms --

Smooth lobulated mass in the nasopharynx or lateral nasal

Pale, purplish, red-gray, or beefy red

-Widening of the pterygomaxillar fissure and pterygopalatine

Lesion fills left choanae

Extends into sphenoid sinus

Feeding vessel = Internal Maxillary Artery

External carotid artery : majority

Internal carotid artery : less common, usually in larger tumours

(I) Nose, nasopharyngeal vault, ethmoidal-sphenoidal sinuses, or minimal

Resection preserves both the anatomy and physiology of the

Middle turbinectomy may be performed for improved exposure

Middle meatus antrostomy

Sphenopalatine artery ligation

Soft palate is split and retracted

Hard palate resection for enhanced exposure

Palatine bone and inferior aspect of pterygoid plate resected

Wide anterior antrostomy

Trans-nasal endoscopic-tumors involving the ethmoid, maxillary,

Transpalatal-provides access to the nasopharynx, sphenoid,

Transantral: lesions extending laterally up to

Extensive resection with higher morbidity

Limited resection with higher recurrence

Marked shift towards endonasal procedures while tumor stages

Expected Blood Loss 225 ml 1250 ml

Length of Stay 2 days 5 days

Patients with IIA through IIIA previously treated with lateral

Tumors extending to infratemporal fossa require lateral

1 case report used as primary treatment modality successfully

Retrospective review reported 2 cases out of 55 patients

Frequent physical examinations

Tumor stage extracranial vs. intracranial tumor

Rare, benign, vascular tumor found almost exclusively in young

Surgery is the gold standard with a trend towards endoscopic

Frequent follow-up after treatment is necessary

For more presentations, please

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