Juvenile Nasopharyngeal ANGIOFIBROMA

1. Juvenile Nasopharyngeal ANGIOFIBROMA Contributed by :- DrSanjiv Kumar, MS(ENT) std, Patna, India For more presentations, please visit www.nayyarENT.com www.nayyarENT.com

2. 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, www.nayyarENT.com

3. 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% www.nayyarENT.com

4. 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 pterygopalatinefossa www.nayyarENT.com

5. 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 www.nayyarENT.com

6. 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) www.nayyarENT.com

7. 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/wgermline mutation in APC gene on chr. 5q) which is involved in sporadic & recurrent JNA. Although evidence of adenomatouspolyposis 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. www.nayyarENT.com

8. 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. www.nayyarENT.com

9. Diagnosis www.nayyarENT.com

10. Diagnosis • History • Physical Exam • Radiological study • CT Scan • MRI • Angiogram www.nayyarENT.com

11. Classical Presentation • Nasopharyngeal mass in teenage or young adult exclusively in male. • Unilateral progressive Nasal obstruction (80-90%). • Recurrent unilateral epistaxis (45-60%) www.nayyarENT.com

12. 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 www.nayyarENT.com

13. Other JNA symptoms contiue….. Advanced Lesions May Causes • Facial pain,orbitalproptosis, diplopia, visual loss is due to invasion of orbit and cavernous sinus. • Headache due to blockage of PNS • Cranial Neuropathy www.nayyarENT.com

14. Appearance • Smooth lobulated mass in the nasopharynx or lateral nasal wall • Pale, purplish, red-gray, or beefy red • Compressible www.nayyarENT.com

15. Differential diagnosis of mass in nose and nasopharynx • Hemangioma • Choanal polyp • Nasopharyngeal carcinoma • Angiomatous polyp • Nasopharyngeal cyst • Hemangiopericytoma • Rhabdomyosarcoma • Chordoma • Juvenile nasopharyngeal angiofibroma www.nayyarENT.com

16. Radiology www.nayyarENT.com

17. 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 www.nayyarENT.com

18. Holman-Miller sign www.nayyarENT.com

19. 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 antrumanteriorly • 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. www.nayyarENT.com

20. Coronal CT • Widening of left sphenopalatine foramen • Lesion fills left choanae • Extends into sphenoid sinus www.nayyarENT.com

21. External Carotid Arteriogram Feeding vessel = Internal Maxillary Artery www.nayyarENT.com

22. 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 www.nayyarENT.com

23. Staging www.nayyarENT.com

24. 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. www.nayyarENT.com

25. Fisch Staging 1.Tumour limited to the nasopharyngeal cavity; bone destruction negligible or limited to the sphenopalatine foramen 2. Tumour invading the pterygopalatinefossa or the maxillary, ethmoid or sphenoid sinus with bone destruction 3. Tumour invading the infratemporalfossa 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 www.nayyarENT.com

26. 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 www.nayyarENT.com

27. 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 www.nayyarENT.com

28. 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 infratemporalfossa, no residual vascularity following embolisation (IV) Skull base erosion, orbit, infratemporalfossa, Residual vascularity (V) Intracranial extension, residual vascularity M: medial extension L: lateral extension www.nayyarENT.com

29. Treatment www.nayyarENT.com

30. 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 www.nayyarENT.com

31. Surgical Approaches • Endoscopic transnasal • Transpalatal • Denker approach • Facial translocation • Medial maxillectomy • Infratemporalfossa with or without craniotomy www.nayyarENT.com

32. 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. www.nayyarENT.com

33. Surgical Approaches • Endoscopic transnasal • Transpalatal • Denker approach • Facial translocation • Medial maxillectomy • Infratemporal fossa with or without craniotomy www.nayyarENT.com

34. 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 www.nayyarENT.com

35. Endoscopic Transnasal • Middle turbinectomy may be performed for improved exposure www.nayyarENT.com

36. Endoscopic Transnasal • Middle meatus antrostomy • Resection of posterior maxillary wall www.nayyarENT.com

37. Endoscopic Transnasal • Sphenopalatine artery ligation • Tumor resection from pterygopalatine fossa www.nayyarENT.com

38. Surgical Approaches • Endoscopic transnasal • Transpalatal • Denker approach • Facial translocation • Medial maxillectomy • Infratemporal fossa with or without craniotomy www.nayyarENT.com

39. Transpalatal • Soft palate is split and retracted www.nayyarENT.com

40. Transpalatal • Hard palate resection for enhanced exposure www.nayyarENT.com

41. Transpalatal • Palatine bone and inferior aspect of pterygoid plate resected www.nayyarENT.com

42. Surgical Approaches • Endoscopic transnasal • Transpalatal • Denker approach • Facial translocation • Medial maxillectomy • Infratemporal fossa with or without craniotomy www.nayyarENT.com

43. 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 midfacialdegloving technique. • Wide anterior antrostomy • Removal of ascending process of maxilla • Removal of inferior half of lateral nasal wall www.nayyarENT.com

44. Surgical Approaches • Endoscopic transnasal • Transpalatal • Denker approach • Facial translocation • Medial maxillectomy • Infratemporal fossa with or without craniotomy www.nayyarENT.com

45. MidfaceDegloving with Maxillary Osteotomies • Gingivobuccal incision • Nasal intercartilaginous incisions with transfixion incision www.nayyarENT.com

46. Surgical Approaches • Endoscopic transnasal • Transpalatal • Denker approach • Facial translocation • Medial maxillectomy • Infratemporal fossa with or without craniotomy www.nayyarENT.com

47. Maxillectomy • Maxillary osteotomies • Sagittal osteotomy www.nayyarENT.com

48. 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 www.nayyarENT.com

49. www.nayyarENT.com

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