Juvenile Nasopharyngeal ANGIOFIBROMA Contributed by Dr Sanjiv Kumar

Juvenile Nasopharyngeal ANGIOFIBROMA Contributed by : - Dr Sanjiv Kumar, MS(ENT) std, Patna, India For more presentations, please visit www. nayyar. ENT. com 1 www. nayyar. ENT. com 7/23/2012

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, 2 www. nayyar. ENT. com 7/23/2012

JNA Facts and Statistics �< 0. 5% of all head and neck tumors �Occurring almost exclusively in males �Average of onset = 15 years (10 -25) �Intracranial Extension between 10 -20% �Recurrence Rates as high as 50% 3 www. nayyar. ENT. com 7/23/2012

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 4 www. nayyar. ENT. com 7/23/2012

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 5 often eroding it by pressure atrophy www. nayyar. ENT. com 7/23/2012

Histology �Myofibroblast is cell of origin �Consist of proloferating, irregular vascular channels 6 within fibrous stroma. �Pseudocapsule made of fibrous tissue �Blood vessels lack a smooth muscle & elastic fibrecause 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) www. nayyar. ENT. com 7/23/2012

Genetics � Overexpression of IGF-2 is found in JNA (53%) associated with � � � 7 tendency to recurrence & poor prognosis. IGF-2 is situated at chromosome 11 p-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. 5 q) 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 7/23/2012 www. nayyar. ENT. com androgen on tumour.

Genetics continue…. . � At molecular genetic level, involvement of 13 q 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. 8 www. nayyar. ENT. com 7/23/2012

Diagnosis 9 www. nayyar. ENT. com 7/23/2012

Diagnosis �History �Physical Exam �Radiological study �CT Scan �MRI �Angiogram 10 www. nayyar. ENT. com 7/23/2012

Classical Presentation �Nasopharyngeal mass in teenage or young adult exclusively in male. �Unilateral progressive Nasal obstruction (80 -90%). �Recurrent unilateral epistaxis (45 -60%) 11 www. nayyar. ENT. com 7/23/2012

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 12 www. nayyar. ENT. com 7/23/2012

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 13 www. nayyar. ENT. com 7/23/2012

Appearance �Smooth lobulated mass in the nasopharynx or lateral nasal wall �Pale, purplish, red-gray, or beefy red �Compressible 14 www. nayyar. ENT. com 7/23/2012

Differential diagnosis of mass in nose and nasopharynx �Hemangioma �Choanal polyp �Nasopharyngeal carcinoma �Angiomatous polyp �Nasopharyngeal cyst �Hemangiopericytoma �Rhabdomyosarcoma �Chordoma �Juvenile nasopharyngeal angiofibroma 15 www. nayyar. ENT. com 7/23/2012

Radiology 16 www. nayyar. ENT. com 7/23/2012

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 17 www. nayyar. ENT. com 7/23/2012

Holman-Miller sign 18 www. nayyar. ENT. com 7/23/2012

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. 19 www. nayyar. ENT. com 7/23/2012

Coronal CT �Widening of left sphenopalatine foramen �Lesion fills left choanae �Extends into sphenoid sinus 20 www. nayyar. ENT. com 7/23/2012

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

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 22 www. nayyar. ENT. com 7/23/2012

Staging 23 www. nayyar. ENT. com 7/23/2012

�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. 24 www. nayyar. ENT. com 7/23/2012

Fisch Staging 25 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 www. nayyar. ENT. com 7/23/2012 fossa or optic chiasm

Radkowski Staging -1996 � 1 a-Limited to the nose and nasopharyngeal area � 1 b-Extension into one or more sinuses � 2 a-Minimal extension into pterygopalatine fossa � 2 b-Occupation of the pterygopalatine fossa without orbital erosion � 2 c-Infratemporal fossa extension without cheek or pterygoid plate involvement � 3 a-Erosion of the skull base (middle cranial fossa or pterygoids) � 3 b-Erosion of the skull base with intracranial extension with or without cavernous sinus involvement 26 www. nayyar. ENT. com 7/23/2012

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 internal carotid artery, tumor localization lateral to ICA, middle fossa extension, and extensive intracranial extension 27 www. nayyar. ENT. com 7/23/2012

Snyderman et al. -2010 (I) No significant extension beyond the site of origin and remaining medial to the midpoint of the pterygopalatine space 28 (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 www. nayyar. ENT. com L: lateral extension 7/23/2012

Treatment 29 www. nayyar. ENT. com 7/23/2012

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 30 www. nayyar. ENT. com 7/23/2012

Surgical Approaches �Endoscopic transnasal �Transpalatal �Denker approach �Facial translocation �Medial maxillectomy �Infratemporal fossa with or without craniotomy 31 www. nayyar. ENT. com 7/23/2012

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 840 cc to 275 cc blood loss � Complications �ophthalmic artery embolization �Facial nerve palsy �Skin and soft tissue necrosis � occlusion of the central retinal artery and consequent tem¬porary 32 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. www. nayyar. ENT. com 7/23/2012

Surgical Approaches �Endoscopic transnasal �Transpalatal �Denker approach �Facial translocation �Medial maxillectomy �Infratemporal fossa with or without craniotomy 33 www. nayyar. ENT. com 7/23/2012

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 34 www. nayyar. ENT. com 7/23/2012

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

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

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

Surgical Approaches �Endoscopic transnasal �Transpalatal �Denker approach �Facial translocation �Medial maxillectomy �Infratemporal fossa with or without craniotomy 38 www. nayyar. ENT. com 7/23/2012

Transpalatal �Soft palate is split and retracted 39 www. nayyar. ENT. com 7/23/2012

Transpalatal �Hard palate resection for enhanced exposure 40 www. nayyar. ENT. com 7/23/2012

Transpalatal � Palatine bone and inferior aspect of pterygoid plate 41 www. nayyar. ENT. com resected 7/23/2012

Surgical Approaches �Endoscopic transnasal �Transpalatal �Denker approach �Facial translocation �Medial maxillectomy �Infratemporal fossa with or without craniotomy 42 www. nayyar. ENT. com 7/23/2012

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 43 www. nayyar. ENT. com 7/23/2012

Surgical Approaches �Endoscopic transnasal �Transpalatal �Denker approach �Facial translocation �Medial maxillectomy �Infratemporal fossa with or without craniotomy 44 www. nayyar. ENT. com 7/23/2012

Midface Degloving with Maxillary Osteotomies �Gingivobuccal incision �Nasal intercartilaginous incisions with transfixion 45 incision www. nayyar. ENT. com 7/23/2012

Surgical Approaches �Endoscopic transnasal �Transpalatal �Denker approach �Facial translocation �Medial maxillectomy �Infratemporal fossa with or without craniotomy 46 www. nayyar. ENT. com 7/23/2012

Maxillectomy �Maxillary osteotomies �Sagittal osteotomy 47 www. nayyar. ENT. com 7/23/2012

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 48 www. nayyar. ENT. com 7/23/2012

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

Surgical Planning �Smaller tumors (IA, IB, IIA, IIB, IIC) Ø 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. Ø 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 Ø Transantral: lesions extending laterally up to 51 pterygopalatine fossa www. nayyar. ENT. com 7/23/2012

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 52 www. nayyar. ENT. com 7/23/2012

�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 53 www. nayyar. ENT. com 7/23/2012

Changing Technique �On Retrospective chart review of surgical intervention Ø Marked shift towards endonasal procedures while tumor stages remained the same Ø Endonasal approach contraindicated in Stage IV and some Stage III cases �May be used in conjunction with other approach in these cases 54 www. nayyar. ENT. com 7/23/2012

Surgical Approach 55 www. nayyar. ENT. com 7/23/2012

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 56 www. nayyar. ENT. com 7/23/2012

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 57 www. nayyar. ENT. com . 7/23/2012

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… 58 www. nayyar. ENT. com 7/23/2012

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 59 www. nayyar. ENT. com 7/23/2012

External Beam Radiation �Retrospective review of efficacy of radiation as primary treatment modality for JNA � 15 patients received 3000 -3500 c. Gy �Recurrence rate of 15% �Conclusion-External beam radiation is effective mode of treatment of advanced JNA 60 www. nayyar. ENT. com 7/23/2012

External Beam Radiation �Retrospective review of efficacy of radiation as primary treatment modality for JNA � 27 patients received 3000 -5500 c. Gy �Recurrence rate of 15% 2 -5 years post-treatment �External beam radiation is effective mode of treatment of advanced JNA 61 www. nayyar. ENT. com 7/23/2012

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 3500 c. Gy �Basal cell carcinoma of skin 14 years after receiving 3500 c. Gy initially, then 3000 c. Gy for recurrence 62 www. nayyar. ENT. com 7/23/2012

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? 63 www. nayyar. ENT. com 7/23/2012

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 64 www. nayyar. ENT. com 7/23/2012

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 65 www. nayyar. ENT. com 7/23/2012

Surveillance �Frequent physical examinations �CT Scan / MRI 66 www. nayyar. ENT. com 7/23/2012

Recurrence Rates �Post-operative �Stage I and II = 7% �Stage III = 39. 5% �Tumor stage – extracranial vs. intracranial tumor �Extracranial = 5% �Intracranial = 50% 67 www. nayyar. ENT. com 7/23/2012

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 68 www. nayyar. ENT. com 7/23/2012

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