Angiofibroma

angiofibroma

1. Angiofibroma Dr. Krishna Koirala Nov 20, 2017

2. Benign, highly vascular and locally aggressive tumour of nasopharynx which occurs exclusively in prepubertal and adolescent males Accounts for 0.05% of all head and neck neoplasms Friedberg (1940) : “Angiofibroma”

3. Synonyms Angiofibroma Juvenile Nasopharyngeal angiofibroma Nasopharyngeal fibroma Monday, November 20, 2017 3

4. Age of onset - second decade (7-19 years ) Mean age at diagnosis : 14 years May regress in late teens but may persist into adulthood Rare after 25 years of age Monday, November 20, 2017 4

5. Site of origin Close proximity to the posterior attachment of the middle turbinate near the superior border of sphenopalatine foramen ? from nonchromaffin paraganglionic cells of the terminal branches of maxillary artery 5

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8. Theories Of Origin Hormonal : occurrence in adolescent males Desmoplastic response of the nasopharyngeal periosteum or embryonic fibrocartilage between the basiocciput and the basisphenoid Hamartomas testosterone Angiofibroma Nest cells (undifferentiated Epitheloid) Vestiges of atrophied stapedial artery 8

9. Hamartoma  Benign, focal malformation that resembles a neoplasm in the tissue of its origin Not a malignant tumor, grows at the same rate as the surrounding tissues C omposed of tissue elements normally found at that site which are growing in a disorganized mass Monday, November 20, 2017 9

10. Pathophysiology Starts adjacent to the sphenopalatine foramen Large tumors - bilobed or dumbbell shaped : (one portion of the tumor filling the naso -pharynx and other portion extending to the pterygopalatine fossa)

11. Spread Anterior growth Nasal cavity (filled on one side , septum deviates to the other side), maxillary sinus Superior growth Sphenoid sinus, cavernous sinus , pituitary fossa, optic chaisma , middle cranial fossa Anterior skull base  Middle cranial fossa

12. Lateral spread Pterygopalatine fossa  Pterygomaxillary fissure  infratemporal fossa  Cheek Greater wing of the sphenoid  middle fossa dura Infraorbital fissures  Orbit (Proptosis ,optic nerve atrophy ) Posterior Nasopharynx 12

13. Symptoms Nasal obstruction (80-90 %) Most frequent symptom Epistaxis (45-60 %) Mostly unilateral and recurrent : painless, profuse, unprovoked Headache (25 %) Blocked paranasal sinuses, Intracranial Facial swelling (10 - 18 %)

14. Other symptoms Unilateral rhinorrhea Anosmia/ hyposmia Rhinolalia clausa Deafness, otalgia Swelling of the palate Deformity of the cheek Monday, November 20, 2017 15

15. Signs Nasal /Nasopharyngeal mass (80%) Orbital mass (15%) , Proptosis (10 -15%) Cheek swelling and trismus (infratemporal fossa involvement) Frog face deformity Serous otitis media (ET blockage) Cranial nerve involvement (II, III, IV, V VI)

16. Monday, November 20, 2017 17 Characteristic Presentation : Teenage or young adult male with recurrent epistaxis , nasal mass and nasal obstruction

17. Investigations Plain x-ray of Nose and PNS Haziness of the sinuses, bone erosion CT scan of Nose and PNS (CECT) Extent / vascularity of tumor Holman Miller sign ( Anterior bowing of posterior wall of maxillary antrum) Bone erosion / Widening of sphenopalatine foramen

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19. Magnetic resonance imaging (MRI) Delineate and define the soft tissue extent in cases of intracranial involvement Angiography (DSA) Extent ,tumor blush, feeding arteries Tumor Biopsy : Contraindicated Vessels are thin walled, lack elastic fibers, absent or incomplete smooth muscle (cause for excessive bleeding) Monday, November 20, 2017 21

20. Monday, November 20, 2017 22 Hypertrophic maxillary artery is the main feeder

21. Other Investigations CBC, Urine R/E, ESR, Bleeding and Clotting profile ECG X-ray chest Blood group and cross match Monday, November 20, 2017 23

22. Staging Stage I: Tumor limited to Nasal cavity or nasopharynx with no bony destruction Stage II: Tumor invading pterygopalatine fossa or PNS Stage III: Tumor invading infratemporal fossa /orbit or parasellar region Stage IV: Tumor invading cavernous sinus/ optic chaisma /pituitary fossa 24

23. Differential Diagnosis Other causes of nasal obstruction Antrochoanal polyp, teratoma, encephalocele, dermoids, inverted papilloma, rhabdomyosarcoma, squamous cell carcinoma Other causes of epistaxis Systemic or local Other causes of proptosis or orbital swellings   Monday, November 20, 2017 25

24. Treatment Options Surgery Gold standard Radiotherapy Reserved for unresectable tumor, intracranial extension , recurrent cases 3000 – 3500 cGy in 15 -18# over 3 - 3.5 wks Proton stereotactic radiotherapy

25. Chemotherapy Recurrent tumors with previous surgery and radiation Hormone therapy To reduce vascularity before surgery Monday, November 20, 2017 27

26. Surgical Approaches Intranasal Endoscopic Transpalatal Transmaxillary Extended Denker’s approach Midfacial degloving Extended lateral rhinotomy Infratemporal fossa Anterior Subcranial Image guided Surgery (Recent Advance)

27. Preop. reduction of tumor vascularity Embolization of feeding arteries 24 to 72 hours pre operative Gelfoam (resorbed in approximately 2 weeks) Polyvinyl alcohol foam (more permanent ) Estrogen Therapy Diethylstilbestrol 2.5 mg PO TDS for 3-6 wks (Cellular contraction, increase in collagen and fibroblasts  decreases bleeding, reduces size)

28. Testosterone Receptor blocker Flutamide Radiotherapy Proton stereotactic RT Cryotherapy Monday, November 20, 2017 30

29. Embolization

30. Selection of Surgical Approach

31. Intranasal endoscopic Approach Small tumor in nose, PNS , nasopharynx, pterygopalatine fossa and even for large tumors Newer technique 33

32. Transpalatal Approach (Wilson) Small tumor in Nasopharynx Monday, November 20, 2017 34

33. Transpalatal + Sublabial Approach( Sardana) Large tumor of Nose/PNS/ Nasopharynx Transmaxillary Approach For tumors extending to pterygopalatine fossa Extended Lateral rhinotomy, mid facial degloving , Denker’s Monday, November 20, 2017 35

34. Monday, November 20, 2017 36 Midfacial degloving

35. Infratemporal fossa approach with or without craniotomy For tumors extending to infratemporal fossa/ intracranial extension

36. Anterior subcranial approach Intracranial extension Image guided surgery Small/ medium size tumors Monday, November 20, 2017 38