Transcript Juvenile Nasopharyngeal Angiofibroma

Juvenile Nasopharyngeal
ANGIOFIBROMA
Contributed by :- Dr Sanjiv Kumar, MS(ENT) std, Patna, India
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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,
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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%
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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
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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
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 It tends to extend along natural foramina and fissures not invading bone but often eroding it by
pressure atrophy
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Histology
 Myofibroblast is cell of origin
 Consist of proloferating, irregular vascular channels within
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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
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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.
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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.
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Diagnosis
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Diagnosis
 History
 Physical Exam
 Radiological study
 CT Scan
 MRI
 Angiogram
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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%)
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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
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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
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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
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Radiology
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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
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Holman-Miller sign
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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.
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Coronal CT
 Widening of left
sphenopalatine foramen
 Lesion fills left choanae
 Extends into sphenoid sinus
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External Carotid Arteriogram
Feeding vessel = Internal Maxillary Artery
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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
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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.
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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
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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
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Önerci 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
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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
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Treatment
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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
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Surgical Approaches
 Endoscopic transnasal
 Transpalatal
 Denker approach
 Facial translocation
 Medial maxillectomy
 Infratemporal fossa with or without craniotomy
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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 tem¬porary 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
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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
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Endoscopic Transnasal
 Middle turbinectomy may be performed for improved exposure
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Endoscopic Transnasal
 Middle meatus antrostomy
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 Resection of posterior maxillary wall
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Endoscopic Transnasal
 Sphenopalatine artery ligation
 Tumor resection from pterygopalatine fossa
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Surgical Approaches
 Endoscopic transnasal
 Transpalatal
 Denker approach
 Facial translocation
 Medial maxillectomy
 Infratemporal fossa with or without craniotomy
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Transpalatal
 Soft palate is split and retracted
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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
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Surgical Approaches
 Endoscopic transnasal
 Transpalatal
 Denker approach
 Facial translocation
 Medial maxillectomy
 Infratemporal fossa with or without craniotomy
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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
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Surgical Approaches
 Endoscopic transnasal
 Transpalatal
 Denker approach
 Facial translocation
 Medial maxillectomy
 Infratemporal fossa with or without craniotomy
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Midface Degloving with Maxillary
Osteotomies
 Gingivobuccal incision
 Nasal intercartilaginous incisions with transfixion incision
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Surgical Approaches
 Endoscopic transnasal
 Transpalatal
 Denker approach
 Facial translocation
 Medial maxillectomy
 Infratemporal fossa with or without craniotomy
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Maxillectomy
 Maxillary osteotomies
 Sagittal osteotomy
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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
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Surgical Approaches
 Endoscopic transnasal
 Transpalatal
 Denker approach
 Facial translocation
 Medial maxillectomy
 Infratemporal fossa with or without craniotomy
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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
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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
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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
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Surgical Approach
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Surgical Technique
Approach (65 pts)
Endoscopic
Open
Expected Blood Loss
225 ml
1250 ml
Complications
1
30
Length of Stay
2 days
5 days
0%
24 %
Recurrence Rate
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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
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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
don’t recomend…
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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
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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
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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?
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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
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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
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Surveillance
 Frequent physical examinations
 CT Scan / MRI
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Recurrence Rates
 Post-operative
 Stage I and II = 7%
 Stage III = 39.5%
 Tumor stage – extracranial vs. intracranial tumor
 Extracranial = 5%
 Intracranial = 50%
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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
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Thank You
For more presentations, please
visit www.nayyarENT.com
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