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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

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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, 7/23/2012 www.nayyarENT.com 2

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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% 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 5

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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) 7/23/2012 www.nayyarENT.com 6

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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 www.nayyarENT.com 7

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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 www.nayyarENT.com 11

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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 12

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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 7/23/2012 www.nayyarENT.com 14

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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 15

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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 17

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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 www.nayyarENT.com 20

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External Carotid Arteriogram Feeding vessel = Internal Maxillary Artery 7/23/2012 www.nayyarENT.com 21

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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 7/23/2012 www.nayyarENT.com 22

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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 26

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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 www.nayyarENT.com 27

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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 31

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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. 7/23/2012 www.nayyarENT.com 32

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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 33

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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 www.nayyarENT.com 34

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Endoscopic Transnasal Middle turbinectomy may be performed for improved exposure 7/23/2012 www.nayyarENT.com 35

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Endoscopic Transnasal Middle meatus antrostomy Resection of posterior maxillary wall 7/23/2012 www.nayyarENT.com 36

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Endoscopic Transnasal Sphenopalatine artery ligation Tumor resection from pterygopalatine fossa 7/23/2012 www.nayyarENT.com 37

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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 38

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Transpalatal Soft palate is split and retracted 7/23/2012 www.nayyarENT.com 39

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Transpalatal Hard palate resection for enhanced exposure 7/23/2012 www.nayyarENT.com 40

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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 42

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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 44

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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 46

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Maxillectomy Maxillary osteotomies Sagittal osteotomy 7/23/2012 www.nayyarENT.com 47

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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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Surgical Approaches Endoscopic transnasal Transpalatal Denker approach Facial translocation Medial maxillectomy Infratemporal fossa with or without craniotomy 7/23/2012 www.nayyarENT.com 50

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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 7/23/2012 www.nayyarENT.com 53

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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 57

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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 7/23/2012 www.nayyarENT.com 60

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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

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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 7/23/2012 www.nayyarENT.com 62

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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 www.nayyarENT.com 63

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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

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Surveillance Frequent physical examinations CT Scan / MRI 7/23/2012 www.nayyarENT.com 66

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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% 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

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Thank You For more presentations, please visit www.nayyarENT.comwww.nayyarENT.com 7/23/2012 www.nayyarENT.com 69