JUVENILE NASOPHARYNGEAL ANGIOFIBROMA

JUVENILE NASOPHARYNGEAL ANGIOFIBROMA,JNA, ANGIOFIBROMA

1. JUVENILE NASOPHARYNGEAL ANGIOFIBROMA Moderator-Dr.Narayanaswamy Presenter -Dr.Razal

2. DEFINITION JNA is a histologically benign , but locally invasive neoplasm which is an uncommon and extremely vascular tumour that arises in the tissues within the sphenopalatine foramen. Synonyms : Angiofibroma, Juvenile angiofibroma, Juvenile nasal angiofibroma(JNA).

3. HISTORY JNA was documented since the time of Hippocrates (4 BC) . Shaheen in 1930 reported the first female patient with juvenile nasopharyngeal angiofibroma . Hondousa recorded the youngest JNA patient (8 years ) Figi and Davis (1950) emphasized the role of surgery in the management of JNA . Histopathological studies of JNA tissue was extensively done by Harma (1959).

4. INCIDENCE JNA accounts for less than 0.5 percent of all head and neck tumors JNA is more common in Americans and Middle east region. Exclusively adolescent males are affected.  The reported rate of incidence varies from 1/6000 ( Harma 1959) to 1/50,000 (Hondousa etal 1954). 

5. AETIOPATHOGENESIS The exact nature of the tumor and its etiology is not well known.  Various theories have been propounded to explain the etiopathogenesis of JNA.

6. THEORIES Ringertz theory : This theory was proposed by Ringertz in 1938.  He believed that JNA always arise from the periosteum of the skull base.

 Som & Neffson (1940): believed that inequalities in the growth of bones forming the skull base resulted in hypertrophy of the underlying periosteum in response to hormonal influence.

 Bensch & Ewing (1941): thought that the tumor probably arise from embryoninc fibro cartilage between the basi occiput and basi sphenoid.

 Brunner (1942) : Suggested an origin from conjoined pharyngobasilar  and buccopharyngeal fascia. 


7. THEORIES Marten et al (1948)- resulted from deficiency of androgens or over activity of estrogens and that the hormonal stimulation is responsible for angiomatous components seen in JNA.
 
Sternberg (1954) :JNA could be a type of haemangioma ( cutaneous haemangioma seen in children which regresses with age).
 
Osborn (1959) :They proposed that the swelling could be due to either a hamartoma or residual fetal erectile tissue which were subject to hormonal influences.
 
 Girgis & Fahmy (1973) : He Observed cell nests of undifferentiated epitheloid cells at the growing edge of angiofibromas. T his appearance was more or less similar to that of paraganglioma .  They considered JNA to be a paraganglionoma .  


8. Recent studies shows other possible factors are, 75% of patients have androgen receptors present. VEGF found in endothelial and stromal cells. Over expression of IGF –II was found in JNA and also associated with recurrence and poor prognosis. Mutations of beta catenin have found in sporadic and recurrent JNA. FAP have 25 times more chance to develop JNA. THEORIES

9. SITE OF ORGIN Earlier assumed that Vault of the Nasopharynx was the most likely site because of the broad attachment to the skull Choana Presently ,Tumor arises from the Posterior part of Nasal cavity-Close to superior margin of the sphenopalatine foramen

10. HISTOPATHOLOGY Macroscopic : Rounded, Spongy, Nodular (nodularity increases with age), Non Encapsulated tumour, red pink or tan grey in appearance covered by nasopharyngeal mucosa. The intact membrane covering the tumour is deep red in color as usually seen in younger patients.

11. Microscopic : vascular spaces of varying shapes and sizes within a stroma of fibrous tissue.  The relative proportions of the vascular and the stromal components change with the age of the swelling.  whereas in long standing tumors collagen predominates.  In some cases  pseudostratified columnar epithelium is seen side by side with the metaplastic squamous epithelium HISTOPATHOLOGY

12. BLOOD SUPPLY Maxillary artery Unnamed branches from internal carotid artery Anterior pharyngeal artery

13. SPREAD OF TUMOR JNA Spreads to Nasal cavity-Nasal Obstruction,Epistaxis , Nasal Dischage Nasopharynx - Pushes soft palate downwards Pterygopalatine fossa Maxillary,ethmoid,Sphenoid sinuses Middle cranial fossa and Anterior cranial fossa

14. CLINCIAL FEATURES The two cardinal symptoms of angiofibroma are NASAL OBSTRUCTION and intermittent unprovoked EPISTAXIS .   The nasal obstruction is so complete causing stasis of secretions and sepsis become inevitable.  Patients may even have hyposmia or anosmia . Chronic Anemia is thus a feature of an established JNA . The voice of the patient acquires a nasal intonation .  If the swelling enlarges to force the soft palate down, the voice may become plummy .  
 Blockage of Eustachian tube orifice is also common causing deafness and otalgia .  Headache is not uncommon in long standing cases. If present it could be attributable to chronic sinusitis in some patients.  Intracranial extension of the mass could also be the cause for headache in these patients. 
 


15. CLINCIAL FEATURES Diplopia may occur secondary to the erosion of the mass into the cranial cavity and causing pressure on the optic chiasma . Proptosis and “ frog-face”. Failing vision -tenting of the optic nerve by the tumor . Swelling of the cheek,Hearing loss, Broadening of nasal bridge

16. Anterior rhinoscopy : shows the presence of abundant  purulent nasal secretions together with bowing of nasal septum to the uninvolved side.  Posterior rhinosocpy : in a cooperative patient shows a pink or red mass filling the Nasopharynx.   Due to the bulk of the lesion it may not be always possible to ascertain the site of origin accurately. Probing should not be done CLINCIAL FEATURES

17. Andrade NA, Andrade JSC, Silva PDM, Oliveira VB, Andrade BB. Nasopharyngeal Angiofibroma: Review of the Genetic and Molecular Aspects . Int. Arch. Otorhinolaryngol . 2008;12(3):442-449

18. Surgical Approaches to Juvenile Nasopharyngeal Angiofibroma - Case Report and Literature Review, Journal of Otolaryngology, 2006  Vol. 10   Num. 2.

19. DIFFERENTIAL DIAGNOSIS Rhinosporidiosis Inverted papilloma Nasal polyps Nasopharyngeal cyst Carcinoma Neurofibroma Pyogenic granuloma Adenoid hypertrophy

20. STAGING OF THE TUMOR FISCH STAGING CLASSIFICATION : TYPE 1 : Tumour limited to the nasopharyngeal cavity; bone destruction negligible or limited to the sphenopalatine foramen TYPE 2 : Tumor extension into the pterygopalatine fossa, or maxillary, sphenoid or ethmoid sinuses with bone destruction. TYPE 3 : Tumour invading the infratemporal fossa or orbital region: 3a without intracranial involvement 3b with intracranial extradural (parasellar) involvement TYPE 4 : Intracranial intradural tumour: 4a without infiltration of the cavernous sinus, pituitary fossa or optic chiasm 4b with infiltration of the cavernous sinus, pituitary fossa or optic chiasm

21. CURRENT STAGINGS Carl H. Snyderman, MD; Harshita Pant, BMBS, PhD; Ricardo L. Carrau, MD; et al:A New Endoscopic Staging System for Angiofibroma Arch Otolaryngol Head Neck Surg . 2010;136(6):588-594. doi:10.1001/archoto.2010.83 ,

22. INVESTIGATIONS

23. X-RAY Xray of the nasopharynx (lateral view) and paranasal sinuses ( occipito -mental view) will show the presence of soft tissues mass. Holman Miller Sign/ Antral Sign -seen in the plain Xray - lateral skull that would show anterior bowing of the posterior wall of the maxillary sinus

24. CT SCAN CT SCAN is particularly helpful to find the extent of the tumor. In addition CT scan with contrast will show the vascularity of the tumor. Showes a dumbell shaped mass extending from Nasal cavity /NP to PTF

25. MRI Helpful especially to see the extension of the soft tissue tumour into the cranium, orbit and infra-temporal fossa .

26. CAROTID ANGIOGRAM CAROTID ANGIOGRAM- the level of vascularity and has to be done before attempting embolization

27. TREATMENT Depends mainly on the extent of the lesion.  1. Surgery 2. Radiation

28. SURGERY-PRE OP Preoperative Embolization In surgical management is controversial.(Complications) Blood supply is predictable(usually Internal maxillary artery). Recurrence rate is more in case of Preoperative Embolization. Preoperative chemotherapy Estrogens have been reported to induce shrinkage.(But effects are variable) Estrogen therapy delayes sugery and secondary feminizing in boys. Non steroidal androgen receptor blocker FLUTAMIDE is also used.

29. APPROCHES Endoscopic Resection Transpalatal Le Fort 1 osteotomy Lateral rhinotomy Midfacial degloving Maxillary swing Infratemporal fossa(1980s) Extensive JNA are resected through skull base with combined approach with Neurosurgeons.

30. SURGERY Stage Fisch I, 2 and some type 3 tumours are suitable for endoscopic resection.

31. ENDOSCOPIC ENDONASAL APPROCH After induction of general anesthesia nose packed with vasoconstrictor solution. The anterior end of middle turbinate is resected. Uncinectomy and wide middle meatal antrostomy An Anterior ethmoidectomy together with removal of the medial wall of maxillary sinus gives access to posterior wall of antrum. Then removal Posterior wall will expose the tumor. Through out the procedure Bipolar diathermy or ligaclips to control bleeding from feeding vessels.

32. RADIATION Unresectable tumors External beam radiation Intracranial extensions and recurrent tumors Dose range: 30-46gy No report on Gama knife therapy

33. COMPLICATIONS Recurrence The age of patient, younger will develop recurrence In advanced disease and in experienced surgeons Invasion of the basisphenoid Pain , bleeding, infection, hyposmia, synechiae, orbital injury, loss of vision, cerebrospinal fluid leak-Endoscopic Approch Infra orbital nerve deficit-Mid facial degloving Radiotherapy Growth retardation ,Temporal bone necrosis,Catracts Thyroid malignancy

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