JUVENILE

NASOPHARYNGEAL
ANGIOFIBROMA
MODERATOR-DR.NARAYANASWAMY
PRESENTER-DR.RAZAL
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).
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).
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).
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.
THEORIES
THEORIES
THEORIES
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.
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
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.
HISTOPATHOLOGY
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
BLOOD SUPPLY
• Maxillary artery
• Unnamed branches from internal carotid artery
• Anterior pharyngeal artery
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
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
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
CLINCIAL FEATURES
•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
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
Surgical Approaches to Juvenile Nasopharyngeal Angiofibroma - Case Report and Literature Review, Journal of Otolaryngology,
2006 Vol. 10 Num. 2.
DIFFERENTIAL DIAGNOSIS
•Rhinosporidiosis
•Inverted papilloma
•Nasal polyps
•Nasopharyngeal cyst
•Carcinoma
•Neurofibroma
•Pyogenic granuloma
•Adenoid hypertrophy
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
 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,
INVESTIGATIONS
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
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
MRI
•Helpful especially to see the extension
of the soft tissue tumour into the
cranium, orbit and infra-temporal
fossa.
CAROTID ANGIOGRAM
CAROTID ANGIOGRAM-the level of vascularity and has to
be done before attempting embolization
TREATMENT
Depends mainly on the extent of the lesion.
1. Surgery
2. Radiation
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.
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.
SURGERY

Stage Fisch I, 2 and some type 3 tumours are suitable for endoscopic resection.
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.
RADIATION
•Unresectable tumors
•External beam radiation
•Intracranial extensions and
recurrent tumors
•Dose range: 30-46gy
•No report on Gama knife therapy
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
THANK YOU