Dr Sanjiv Kumar, Contributed by :- Dr Sanjiv Kumar, MS(ENT)
std, Patna, India For more presentations, please visit
www.nayyarENT.comwww.nayyarENT.com Juvenile Nasopharyngeal
ANGIOFIBROMA 7/23/2012 www.nayyarENT.com 1
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% 7/23/2012 www.nayyarENT.com 3
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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 7/23/2012 www.nayyarENT.com 4
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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 7/23/2012 www.nayyarENT.com
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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)
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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. 7/23/2012
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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. 7/23/2012 www.nayyarENT.com 8
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Diagnosis 7/23/2012 www.nayyarENT.com 9
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Diagnosis History Physical Exam Radiological study CT Scan MRI
Angiogram 7/23/2012 www.nayyarENT.com 10
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Classical Presentation Nasopharyngeal mass in teenage or young
adult exclusively in male. Unilateral progressive Nasal obstruction
(80-90%). Recurrent unilateral epistaxis (45-60%) 7/23/2012
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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 7/23/2012 www.nayyarENT.com
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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 7/23/2012 www.nayyarENT.com 13
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Appearance Smooth lobulated mass in the nasopharynx or lateral
nasal wall Pale, purplish, red-gray, or beefy red Compressible
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Differential diagnosis of mass in nose and nasopharynx
Hemangioma Choanal polyp Nasopharyngeal carcinoma Angiomatous polyp
Nasopharyngeal cyst Hemangiopericytoma Rhabdomyosarcoma Chordoma
Juvenile nasopharyngeal angiofibroma 7/23/2012 www.nayyarENT.com
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Radiology 7/23/2012 www.nayyarENT.com 16
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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 7/23/2012 www.nayyarENT.com
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Holman-Miller sign 7/23/2012 www.nayyarENT.com 18
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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. 7/23/2012 www.nayyarENT.com 19
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Coronal CT Widening of left sphenopalatine foramen Lesion fills
left choanae Extends into sphenoid sinus 7/23/2012
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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
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Staging 7/23/2012 www.nayyarENT.com 23
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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. 7/23/2012 www.nayyarENT.com 24
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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 7/23/2012 www.nayyarENT.com 25
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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 7/23/2012 www.nayyarENT.com
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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 7/23/2012
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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 7/23/2012 www.nayyarENT.com 28
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Treatment 7/23/2012 www.nayyarENT.com 29
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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 7/23/2012 www.nayyarENT.com 30
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Surgical Approaches Endoscopic transnasal Transpalatal Denker
approach Facial translocation Medial maxillectomy Infratemporal
fossa with or without craniotomy 7/23/2012 www.nayyarENT.com
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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.
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Surgical Approaches Endoscopic transnasal Transpalatal Denker
approach Facial translocation Medial maxillectomy Infratemporal
fossa with or without craniotomy 7/23/2012 www.nayyarENT.com
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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 7/23/2012
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Endoscopic Transnasal Middle turbinectomy may be performed for
improved exposure 7/23/2012 www.nayyarENT.com 35
Surgical Approaches Endoscopic transnasal Transpalatal Denker
approach Facial translocation Medial maxillectomy Infratemporal
fossa with or without craniotomy 7/23/2012 www.nayyarENT.com
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Transpalatal Soft palate is split and retracted 7/23/2012
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Transpalatal Hard palate resection for enhanced exposure
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Transpalatal Palatine bone and inferior aspect of pterygoid
plate resected 7/23/2012 www.nayyarENT.com 41
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Surgical Approaches Endoscopic transnasal Transpalatal Denker
approach Facial translocation Medial maxillectomy Infratemporal
fossa with or without craniotomy 7/23/2012 www.nayyarENT.com
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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
7/23/2012 www.nayyarENT.com 43
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Surgical Approaches Endoscopic transnasal Transpalatal Denker
approach Facial translocation Medial maxillectomy Infratemporal
fossa with or without craniotomy 7/23/2012 www.nayyarENT.com
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Midface Degloving with Maxillary Osteotomies Gingivobuccal
incision Nasal intercartilaginous incisions with transfixion
incision 7/23/2012 www.nayyarENT.com 45
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Surgical Approaches Endoscopic transnasal Transpalatal Denker
approach Facial translocation Medial maxillectomy Infratemporal
fossa with or without craniotomy 7/23/2012 www.nayyarENT.com
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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
7/23/2012 www.nayyarENT.com 48
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7/23/2012 www.nayyarENT.com 49
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Surgical Approaches Endoscopic transnasal Transpalatal Denker
approach Facial translocation Medial maxillectomy Infratemporal
fossa with or without craniotomy 7/23/2012 www.nayyarENT.com
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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 7/23/2012 www.nayyarENT.com 51
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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 7/23/2012 www.nayyarENT.com 52
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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
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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 7/23/2012 www.nayyarENT.com 54
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Surgical Approach 7/23/2012 www.nayyarENT.com 55
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Surgical Technique Approach (65 pts) EndoscopicOpen Expected
Blood Loss 225 ml 1250 ml Complications130 Length of Stay 2 days 5
days Recurrence Rate 0 % 24 % 7/23/2012 www.nayyarENT.com 56
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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. 7/23/2012 www.nayyarENT.com
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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 7/23/2012 www.nayyarENT.com 58
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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 7/23/2012 www.nayyarENT.com 59
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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
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External Beam Radiation 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 7/23/2012 www.nayyarENT.com 61
Slide 62
External Beam Radiation 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
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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? 7/23/2012
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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 7/23/2012 www.nayyarENT.com 64
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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 7/23/2012 www.nayyarENT.com 65
Recurrence Rates Post-operative Stage I and II = 7% Stage III =
39.5% Tumor stage extracranial vs. intracranial tumor Extracranial
= 5% Intracranial = 50% 7/23/2012 www.nayyarENT.com 67
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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 7/23/2012 www.nayyarENT.com 68
Slide 69
Thank You For more presentations, please visit
www.nayyarENT.comwww.nayyarENT.com 7/23/2012 www.nayyarENT.com
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