Juvenile Nasopharyngeal ANGIOFIBROMA

Juvenile Nasopharyngeal Angiofibroma

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

For more presentations, please visit www.nayyarENT.com

Juvenile Nasopharyngeal ANGIOFIBROMA 7/23/2012www.nayyarENT.com1Juvenile Nasopharyngeal AngiofibromaBenign highly vascular tumor

Locally invasive, submucosal spread

Vascular supply most commonly from internal maxillary arteryAlso: Ascending pharyngeal, Ascending palatine, Internal carotid, external carotid, common carotid,

7/23/2012www.nayyarENT.com2JNA 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%7/23/2012www.nayyarENT.com3OriginIt 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

7/23/2012www.nayyarENT.com4Routes of SpreadMedial growthNasal cavityNasopharynxLateral growthPterygopalatine fossa Vertical expansion through inferior orbital fissure to orbit possible Infratemporal fossaSuperior expansion through pterygoid process may involve middle cranial fossaLateral and posterior walls of sphenoid sinus can be erodedCavernous sinus may be involvedPituitary may be involved

It tends to extend along natural foramina and fissures not invading bone but often eroding it by pressure atrophy7/23/2012www.nayyarENT.com5HistologyMyofibroblast is cell of originConsist of proloferating, irregular vascular channels within fibrous stroma.Pseudocapsule made of fibrous tissueBlood 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)7/23/2012www.nayyarENT.com6GeneticsOverexpression 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.

7/23/2012www.nayyarENT.com7Genetics 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 receptorTransformation 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.

7/23/2012www.nayyarENT.com8Diagnosis7/23/2012www.nayyarENT.com9DiagnosisHistoryPhysical ExamRadiological studyCT ScanMRIAngiogram7/23/2012www.nayyarENT.com10Classical PresentationNasopharyngeal mass in teenage or young adult exclusively in male.Unilateral progressive Nasal obstruction (80-90%).Recurrent unilateral epistaxis (45-60%)

7/23/2012www.nayyarENT.com11Other JNA Symptoms

Other common symptoms --Swelling Of The CheekConductive hearing Loss and secretory otitis media secondary to Eustachian tube blockDacrocystitsRhinorrheaHard And Soft Palate DeformityHyposmia Or Anosmia

7/23/2012www.nayyarENT.com12Other JNA symptoms contiue..Advanced Lesions May CausesFacial pain,orbital proptosis, diplopia, visual loss is due to invasion of orbit and cavernous sinus. Headache due to blockage of PNSCranial Neuropathy

7/23/2012www.nayyarENT.com13AppearanceSmooth lobulated mass in the nasopharynx or lateral nasal wall

Pale, purplish, red-gray, or beefy red

Compressible 7/23/2012www.nayyarENT.com14Differential diagnosis of mass in nose and nasopharynxHemangiomaChoanal polypNasopharyngeal carcinomaAngiomatous polypNasopharyngeal cystHemangiopericytomaRhabdomyosarcomaChordomaJuvenile nasopharyngeal angiofibroma7/23/2012www.nayyarENT.com15Radiology7/23/2012www.nayyarENT.com16Radiological StudiesPlain 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 thesphenoid sinus -Anterior bowing of the posterior wall of themaxillary antrum (Holman-Miller Sign)

-Widening of thepterygomaxillar fissureandpterygopalatine fossa -Erosion of the medial pterygoid plate

7/23/2012www.nayyarENT.com17Holman-Miller sign

7/23/2012www.nayyarENT.com18Radiological studies continueCT ScanExcellent for delineating bony changesLesion enhances with contrast on CTLobulated 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

AngiogramEvaluation of feeding blood vessels, for selective embolisation.

7/23/2012www.nayyarENT.com19Coronal CTWidening of left sphenopalatine foramen

Lesion fills left choanae

Extends into sphenoid sinus

7/23/2012www.nayyarENT.com20External Carotid Arteriogram

Feeding vessel = Internal Maxillary Artery7/23/2012www.nayyarENT.com21Blood Supply of these tumours is usually byExternal carotid artery: majorityinternal maxillary arteryascending pharyngeal arterypalatine arteries

Internal carotid artery: less common, usually in larger tumourssphenoidal branchesophthalmic artery

7/23/2012www.nayyarENT.com22Staging7/23/2012www.nayyarENT.com23Exact 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.7/23/2012www.nayyarENT.com24Fisch Staging1.Tumour limited to the nasopharyngeal cavity; bone destruction negligible or limited to the sphenopalatine foramen2. Tumour invading the pterygopalatine fossa or the maxillary, ethmoid or sphenoid sinus with bone destruction3. Tumour invading the infratemporal fossa or orbital region: (a) without intracranial involvement (b) with intracranial extradural (parasellar) involvement4. 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 chiasm7/23/2012www.nayyarENT.com25Radkowski Staging -19961a-Limited to the nose and nasopharyngeal area1b-Extension into one or more sinuses2a-Minimal extension into pterygopalatine fossa2b-Occupation of the pterygopalatine fossa without orbital erosion2c-Infratemporal fossa extension without cheek or pterygoid plate involvement3a-Erosion of the skull base (middle cranial fossa or pterygoids)3b-Erosion of the skull base with intracranial extension with or without cavernous sinus involvement7/23/2012www.nayyarENT.com26nerci 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

7/23/2012www.nayyarENT.com27 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

7/23/2012www.nayyarENT.com28Treatment7/23/2012www.nayyarENT.com29Treatment OptionsSurgeryGold standardRadiation therapyReserved for unresectable, life-threatening tumorsChemotherapyRecurrent tumors with previous surgery and radiationHormone therapyEstrogens and antiandrogens used to decrease tumor size and vascularity7/23/2012www.nayyarENT.com30Surgical ApproachesEndoscopic transnasalTranspalatalDenker approachFacial translocationMedial maxillectomyInfratemporal fossa with or without craniotomy

7/23/2012www.nayyarENT.com31Preoperative Embolization24 to 72 hours preoperatively to avoid collateral vascularisationMost 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 EfficacyStage I patients reduced from 840cc to 275cc blood lossComplicationsophthalmic artery embolizationFacial nerve palsySkin 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 strokesome authors consider preoperative embolization to provide no benefit, or even to increase the risk of recurrence.

7/23/2012www.nayyarENT.com32Surgical ApproachesEndoscopic transnasalTranspalatalDenker approachFacial translocationMedial maxillectomyInfratemporal fossa with or without craniotomy

7/23/2012www.nayyarENT.com33Endoscopic Transnasal

Resection preserves both the anatomy and physiology of the nose, requires less rehabilitation days after surgery, and is highly successful for selected patients7/23/2012www.nayyarENT.com34Endoscopic TransnasalMiddle turbinectomy may be performed for improved exposure

7/23/2012www.nayyarENT.com35Endoscopic TransnasalMiddle meatus antrostomyResection of posterior maxillary wall

7/23/2012www.nayyarENT.com36Endoscopic TransnasalSphenopalatine artery ligationTumor resection from pterygopalatine fossa


7/23/2012www.nayyarENT.com38TranspalatalSoft palate is split and retracted

7/23/2012www.nayyarENT.com39TranspalatalHard palate resection for enhanced exposure

7/23/2012www.nayyarENT.com40Transpalatal

Palatine bone and inferior aspect of pterygoid plate resected


7/23/2012www.nayyarENT.com42Denker ApproachItis 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 antrostomyRemoval of ascending process of maxillaRemoval of inferior half of lateral nasal wall


7/23/2012www.nayyarENT.com44Midface Degloving with Maxillary OsteotomiesGingivobuccal incisionNasal intercartilaginous incisions with transfixion incision


7/23/2012www.nayyarENT.com46MaxillectomyMaxillary osteotomiesSagittal osteotomy

7/23/2012www.nayyarENT.com47Alternative Approaches to Nasal Cavities and Paranasal SinusesLateral RhinotomyWeber-Ferguson incisionWeber-Ferguson with Lynch extensionWeber-Ferguson with lateral subciliary extensionWeber-Ferguson with subciliary extension and supraciliary extension7/23/2012www.nayyarENT.com48


7/23/2012www.nayyarENT.com50Surgical PlanningSmaller 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 fossa7/23/2012www.nayyarENT.com51Surgical planning continue..Larger tumors (IIIA, IIIB)Lateral rhinotomyMidfacial 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

7/23/2012www.nayyarENT.com52Transnasal 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 infections7/23/2012www.nayyarENT.com53Changing TechniqueOn Retrospective chart review of surgical intervention

Marked shift towards endonasal procedures while tumor stages remained the sameEndonasal approach contraindicated in Stage IV and some Stage III casesMay be used in conjunction with other approach in these cases7/23/2012www.nayyarENT.com54Surgical Approach

7/23/2012www.nayyarENT.com55Surgical TechniqueApproach (65 pts)EndoscopicOpenExpected Blood Loss225 ml 1250 mlComplications130Length of Stay2 days5 daysRecurrence Rate0 %24 %7/23/2012www.nayyarENT.com56Surgical TechniqueTransnasal endoscopic approach can replace transpalatal approachBecouse 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 exposureGreater morbidity.7/23/2012www.nayyarENT.com57Surgical TechniqueSurgical limitations of endoscopic resection evaluated in literature review Extremely limited IIIA and IIIB may be approached endoscopicallyPreoperative embolization recommended, but some surgeons dont recomend7/23/2012www.nayyarENT.com58Gamma Knife Surgery2 case reports used as booster treatment for residual tumor after surgeryNo change in tumor size of one patient, regression in other patient

1 case report used as primary treatment modality successfully7/23/2012www.nayyarENT.com59External Beam RadiationRetrospective review of efficacy of radiation as primary treatment modality for JNA15 patients received 3000-3500 cGyRecurrence rate of 15%Conclusion-External beam radiation is effective mode of treatment of advanced JNA

7/23/2012www.nayyarENT.com60External Beam RadiationRetrospective review of efficacy of radiation as primary treatment modality for JNA27 patients received 3000-5500 cGyRecurrence rate of 15% 2-5 years post-treatmentExternal beam radiation is effective mode of treatment of advanced JNA7/23/2012www.nayyarENT.com61External Beam RadiationLong-term sequelae of concernGrowth retardation, panhypopituitarism, temporal lobe necrosis, cataracts, radiation keratopathy

Retrospective review reported 2 cases out of 55 patients developing secondary malignanciesThyroid carcinoma 13 years after receiving 3500cGyBasal cell carcinoma of skin 14 years after receiving 3500cGy initially, then 3000cGy for recurrence7/23/2012www.nayyarENT.com62ChemotherapyChemotherapy is alternative therapy unresectable tumor had chemotherapy for palliationAdriamycin, decarbazine, vincristine,actinomycin-d and cyclophosphamideExtensive regression of tumorPossible alternative to radiation?7/23/2012www.nayyarENT.com63Hormonal TherapyAndrogen and progesteron receptors have been identified with varying frequencies in JNAsSome JNAs lack these receptors

Limited utilityDelays surgeryFeminizing side effectsCardiovascular complications

7/23/2012www.nayyarENT.com64Hormonal TherapyTreatment with flutamide(potent nonsteroidal androgen receptor blocker), tumor shrinkage of up to 44 % was reported by Gates et al diethyl stilbestrolBefore and after measurement comparison made using CT scanNo statistically significant difference in sizeNo difference in blood lossNo advantage with treatment7/23/2012www.nayyarENT.com65SurveillanceFrequent physical examinations

CT Scan / MRI7/23/2012www.nayyarENT.com66Recurrence RatesPost-operativeStage I and II = 7%Stage III = 39.5%

Tumor stage extracranial vs. intracranial tumorExtracranial = 5%Intracranial = 50%

7/23/2012www.nayyarENT.com67ConclusionsRare, 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

7/23/2012www.nayyarENT.com68Thank You

For more presentations, please visit www.nayyarENT.com

7/23/2012www.nayyarENT.com69

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Juvenile Nasopharyngeal ANGIOFIBROMA