JUVENILE NASOPHARYNGEAL ANGIOFIBROMA Surgical approaches & newer treatment options

Detail description of pathology & management of JNA.

1. JNA : SURGICAL APPROACHES & NEWER
TREATMENT OPTIONS
DR UTKAL MISHRA
AIIMS, BHOPAL

2. JUVENILE NASOPHARYNGEAL ANGIOFIBROMA
Most common benign tumorof nasopharynx.
Seen almost exclusively in AdolescentMalesof 10-20 years
It is encapsulated , slow-growing,vasculartumor
Although benign it is locally aggressive and has a high recurrence
rate

3. EPIDEMIOLOGY
Accounts for 0.05 to 0.5% of all head & neck tumours.
Intracranial extension found in 20 %cases.
Incidence –1/6000
Harmaet alto 1/50,000
Hondousaet al
In India incidence is increasing.

4. PATHOLOGY
Gross : -Sessile, Firm, Lobulated, Pink –Red in colour
Histology : -
1.Encapsulated, composed of vascular tissue & fibrous stroma.
2.Vessels are thin-walled, endothelium lined with no muscle or elastic coat.

5. THEORIES OF ORIGIN
Ringertztheory: JNA always arose from the periosteumof the skull base.
Bensch& Ewing (1941): Origin from embryonincfibro cartilage between the basiocciputand basisphenoid.
Brunner (1942): Origin from conjoined pharyngobasilarand buccopharyngealfascia.
Marten (1948): Tumorsresulted from deficiency of androgens or over activity of estrogens
Sternberg(1954): Hamartoma
Osborn (1959): Hamartomatousorigin
Girgis& Fahmy(1973): They considered JNA to be a paraganglionoma.
Mild & Mauristheory: Origin from midline erectile tissue/ androgen dependent hamartoma

6. SITE OF ORIGIN
Most common site -Superior Margin Of SphenopalatineForamen
Pterygoidwedge
Vidians canal
Basisphenoid

7. EXTRANASOPHARYNGEAL ANGIOFIBROMA
Do not originate from the area around the sphenopalatineforamen.
Common in older Females
Less vascular
Commonest site –Maxillary sinus
Other sites –Ethmoid sinus, Inferior Turbinates, Frontal Recess, Tonsil, RMT

8. MOLECULAR ANALYSIS
Androgen receptors -75%
VEGF–80%
Progesterone receptors
SOMATOSTATIN Receptor (SSTR 2)
IGF II
APC gene -25 times more frequent in FAP patients
ß catenin
CD 34
Loss of expression of GSTM 1

9. CLINICAL FEATURES
Commonest Symptom -Profuse, Unprovoked,Recurrentand Spontaneous
Epistaxis.
Progressive nasal obstruction and denasalspeech
Conductive hearing loss and otitismedia with effusion.
Mass in the nasopharynx, Palatal Bulge
Broadening of Nasal Bridge, Proptosis, Swelling of Cheek

10. EXAMINATION OF NOSE
Smooth Reddish Lobulatedmass filling the nasal cavity & choana at times.
Accumulations of secretions anterior to mass –CHOANAL BANKING EFFECT
DNS to contralatertalside may be present.

11. SPREAD OF JNA
Sphenopalatine
foramen
Pterygopalatine
fossa
Infratemporal
fossa
Inferior orbital fissure
Orbit
Maxillary sinus
Cheek
Sphenoid sinus
Middle Cranial
fossa
Pituitary
Cavernous sinus
Nasal
cavity
Nasopharyn
x

12. SIGNIFICANCE OF PTERYGOID WEDGE
It is defined as the anterior junction of the medial & lateral pterygoidplates.
Involvement of pterygoidwedge is found in 99% cases.
Pterygoidwedge is the Epicenter of tumour.
Most common site of residual & recurrent disease –pterygoidwedge (45%)
Most important step in JNA surgery to prevent recurrence -Drilling of pterygoidwedge

14. FISCHSTAGING
Courtesy : Scott Browns Otolaryngology & Head & Neck Surgery 7
th
edition

15. RADKOWSKI STAGING
Courtesy : Scott Browns Otolaryngology & Head & Neck Surgery 7
th
edition

16. OTHER STAGING SYSTEMS
Andrews staging
Chandlier’sstaging
Session’s staging
Onercistaging
Tondonstaging

18. DIAGNOSIS
BIOPSY CONTRAINDICATED
Investigation of choice –Contrast Enhanced CT scan
MRI –Intracranial extension, Orbit, Infratemporalfossa
Carotid Angiography with Embolization

20. HOLMAN MILLER SIGN

21. HONDOUSA SIGN
HONDOUSA SIGN –Widening of gap between ramusof
mandible & maxillary body

22. RAM HARAN SIGN
RAM HARAN SIGN –Quadrilateral appearance of pterygoidwedge

23. CHOP STICK SIGN
CHOP STICK SIGN–Post op appearance of medial & lateral pterygoidplates as
two separate sticks due to drilling & removal of pterygoidwedge.

24. MRI
Characteristic –Salt & Pepper appearance due to flow voids
It aids in differentiation of tumourin –Orbit , Cavernous sinus , Middle cranial fossa , Infratemporal
region

25. MRI

26. DIGITAL SUBSTRACTION ANGIOGRAPHY
Commonest feeding vessel –Internal Maxillary Artery
In large tumours–
1.Ascending Pharyngeal Artery
2.ContralateralECA branches
3.ICA branches -Ophthalmic, Meningo-hypophyseal, Vidian Artery

28. EMBOLIZATION
Planned 24-48 hrs before surgery to avoid revascularization.
No anesthesia required for cooperative patients
Done under DSA guidance.

29. DISADVANTAGE
Advantage –Reduction in blood loss, Less operative time, Improved visualisationof tumourmargins
Disadvantage –
1.Neurological complications, -Stroke, Cranial N. palsy, Blindness
2.Recurrence
3.Friable
4.Obscure tumourfront in cracks & crevices.

30. TYPES
2 types –
1.TRANSARTERIAL EMBOLIZATION WITH PVA
2.DIRECT PERCUTANEOUS EMBOLIZATION WITH ONYX–
Advantage: Solidifies slowly & infiltrates small vessels with excellent penetration of parenchyma

32. TREATMENT MODALITIES
Surgery –Treatment of choice
Radiotherapy
Hormonal therapy
Chemotherapy

33. PRINCIPLES OF JNASURGERY
Analyze the coronal CT thoroughly & plan the approach.
Adequate tumourexposure.
Don’t touch the tumouruntil feeding vessels are controlled.
Drilling of pterygoidwedge is must.

34. ANAESTHETIC CONSIDERATIONS
TIVA –Ramifentanyl+ Propofol
Controlled hypotension by Nitroglycerine infusion
Maintain MAP → 60 –70 mm Hg
Positioning –Reverse Trendelenberg position

35. SURGICAL
APPROACHES
ENDOSCOPIC
APPROACH
OPEN
APPROACH

36. TREATMENT

37. ENDOSCOPIC APPROACH
INDICATIONS -
Fisch1& II tumours
FischIII tumourswith limited medial invasion of infratemporalfossa

38. BINOSTRIL 4 HANDED SURGERY
1 stdescribed by –MAYet al in 1990.
Posterior septectomydone as 1
st
step.
Requires 2 surgeons
Surgeon 1 –Holds endoscope at 11 o clock position + Irrigation
Surgeon 2 –Suction same nostril + Instruments opposite nostril

39. ENDOSCOPIC ENDONASALTECHNIQUE
Nose is prepared with 4% Cocaine & adrenaline 1:10,000
Resection of anterior end of middle turbinate
Anterior ethmoidectomy + Removal of medial wall of maxillary sinus
Removal of posterior wall of maxillary antrum to achieve complete lateral exposure of tumor
LigatingSPA + DPA
Dissection continues till rostrum of sphenoid
Tumor is peeled inferiorly
Drilling of basisphenoid & pterygoidwedge to remove residual tumour.

40. MODIFIED DENKERS APPROACH

41. THE FOUR-PORT BRADOOTECHNIQUE
4 ports –
(A) The ipsilateral nostril.
(B) The contralateralnostril after doing a posterior septectomy.
(C) An antralwindow in the canine fossa.
(D) An incision of one inch in the gingivobuccalsulcus adjacent to the last molar.
Advantage–Avoids removal of frontonasalprocess of maxilla

42. POST OP MANAGEMENT
Merocelpack removed after 48 hrs.
Saline irrigation started after pack removal
Endoscopic cleaning of nose every weekly until crusting subsides.
CECTdone after 36 hrs to rule out residual disease.

43. FOLLOW UP
Endoscopic examination of nose every 3 months
Routine CECT every year for at least 3 years

44. OPEN APPROACHES
1.Transpalatine
2.Transpalatine+ Sublabial(Sardana’sapproach)
3.Lateral rhinotomywith medial maxillectomy
4.Midfacialdeglovingapproach
5.Transmaxillary(Le Fort I) approach
6.Maxillary swing approach or facial translocation approach (Wei’s operation)
7.Infratemporalfossa approach
8.Intracranial–extracranialapproach

45. WILSONS TRANSPALATAL APPROACH
Indication-For tumourrestricted to nasopharynx
Advantage–Excellent cosmesis
Disadvantage–Limited exposure, Palatal fistula

46. WILSONS TRANSPALATAL APPROACH

47. LATERAL RHINOTOMY + MEDIAL MAXILLECTOMY
Suited for growth in nasal cavity extending to maxillary sinus, pterygopalatine
fossa, medial part of infratemporalfossa.
Advantage –Wide exposure, Feeding vessels easily controlled
Disadvantage –Scar, Bleeding

48. LATERAL RHINOTOMY + MEDIAL MAXILLECTOMY

49. MIDFACIALDEGLOVINGAPPROACH
4 INCISIONS –
1.Sublabialincision 3rd molar
2.Transfixationincision
3.Intercartilagenousincision
4.Circumvestibularincision
Commonest complication –Vestibular stenosis, InfraorbitalN.
Injury

50. LE FORT 1 OSTEOTOMY
Wide access to Nasopharynx, Maxillary sinus, Sphenoid
sinus
Complication –Malocclusion, Necrosis of maxilla

51. MAXILLARY SWING OR FACIAL TRANSLOCATION ( WEI’S)

53. HEMOSTASIS IN JNA
Reverse trendelenbergposition with 20
0
head elevation –Improves venous drainage from brain.
Direct pressure
Ligaclips
Bipolar forceps
Warm saline irrigation 40
0
c
1:1000 topical adrenaline
Surgicel
Floseal–Bovine Collagen + Human Thrombin

54. MANAGEMENT OF ICAINJURY
Don’t panic Don’t pack
Use 2 suctions
1 –2 cm
3
muscle harvested from thigh or abdomen
Crushed & placed over bleeding point for atleast3-5 min. → Activates platelet fibrin plug
Reinforce with surgicel
If still not controlled → Endovascular intervention by angiography team

55. 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% Xylocainepack in PPF , ITF
If occurs –Stop all manipulation, IV Crystalloids, wait for 10-15 min

56. EARLY POST OP
Nasal Crusting
Orbital hematoma
Infraorbitalnerve paraesthesia

57. LATE COMPLICATIONS
Alarcollapse –Modified denkersdue to drilling of pyriformaperture
Vestibular stenosis
Fistula of palate
Caroticocavernousfistula
Recurrence

58. RECURRENCE
Defined as subsequent tumourafter negative immediate post op scan at 36 hours
Incidence –32 %
Factors responsible-
1.Extensive Disease
2.Young Age
3.Pre op Embolization
4.InexpriencedSurgeon
MOST IMPORTANT STEP TO PREVENT RECURRENCE –Drilling the cancellousbone of pterygoidwedge

60. INDICATIONS
Extensive primary disease with intracranial extension
Unresectable residual disease
Medically unfit

61. TYPES
Megavoltage EBRT
IMRT
GAMMA KNIFE & CYBER KNIFE

62. DOSE
3000 to 5500 cGyin 15–18 fractions is delivered in 3–3.5 weeks.
Tumour regression is very slow (over 2-3 year).
Tumorregression by radiation vasculitisand occlusion of vessels by perivascularfibrosis.

63. COMPLICATIONS
Occular–Cataract, Glaucoma, Endophthalmitis, Optic N. Atrophy
Cranial N. Palsy
Pan Hypopituitarism
Temporal lobe necrosis
Malignant transformation of JNA
Xerostomia, Hyposmia, Crusting

64. HORMONAL THERAPY
Flutamide-10mg/kg/day in 3 divided doses x 6 weeks –44%tumourshrinkage
Diethylstilbestrol –5 mg TID
Bevacizumab–MabagainstVEGF
Sirolimus/ Rapamycin

65. CHEMOTHERAPY
Doxorubicin
Dacarbazine
Vincristine
Dactinomycin
Cyclophophamide
Cisplatine

66. THANK
YOU