Specialist ENT NASOPHARYNGEAL ANGIOFIBROMA

NASOPHARYNGEAL ANGIOFIBROMA

Definition: They are benign, very vascular and biologically aggressive tumors, originating almost exclusively from posterior nasal and nasopharyngeal region in adolescent males. Aetiology:
Age Usually occurs in the 2nd decade. Sex Exclusively common in males. Theories of origin:
  1. Fibroblastic Theory: This theory is suggestive of an abnormal growth or response of connective tissues such as embryonic occipital plate, fascia basalis, periosteum of posterior nasopharyngeal wall prior to ossification at 25 Years.
  2. Hormonal Theory: Angiofibromas are hormone dependent tumors due to estrogen and androgen imbalance.
  3. Hamartomatous Theory:  It suggests of proliferation of vascular aberrant erectile tissue under hormonal influence.
  4. Ringertz: Tumour arise from periosteum of the nasal vault.
  5. Som and Neffson: Inequalities in the growth of bones forming skull base results in hypertrophy of the underlying periosteum.
  6. Bensch and Ewing: Tumour arises from the embryonic fibrocartilage between            basi-occiput and basi-sphenoid.
  7. Brunner: Tumor arises from conjoined pharyngobasilar and buccopharyngeal fascia.
  8. Osborn: He suggested that this tumor is either a hamartoma or residues of fetal erectile tissue under hormonal influences.
  9. Girgis and Fahmy: They suggested the presence of paragangliomatous tissue around the terminal part of maxillary artery in the pterygopalatine fossa.
Pathogenesis: The tumor usually arises from the sphenopalatine foramen or the posterior wall of the nasal cavity with adjoining superolateral wall. The blood vessels are devoid of tunica media, hence the bleeding is torrential. Blood supply: Major arterial supply is from the ipsilateral internal maxillary artery. Collateral supply is from the ascending pharyngeal artery and branches of internal carotid artery.
Spread of JNA:
Specialist ENT Screenshot 2020 11 28 Ganpati nose
Clinical features:
Symptoms:
  1. Nasal obstruction.
  2. Epistaxis.
  3. Facial swelling.
  4. Eustachian tube blockage.
  5. Rhinolalia.
Signs:
  1. Reddish purple mass in one nostril which bleeds profusely on touch.
  2. Proptosis.
  3. Soft palate pushed forward by mass.
  4. Pallor.
Specialist ENT Screenshot 2020 11 28 Ganpati nose
Investigation: 1) Haemogram. 2) CT Scan is the important diagnostic test to know the extent and vascularity of tumor. It can be performed along with angiography. It usually shows:
  1. Nasopharyngeal mass.
  2. Widening of pterygopalatine fissure.
  3. Enlargement of superior orbital fissure.
  4. Distortion of nasal septum with erosion of the paranasal sinuses.
3) M.R.I contrast should be done to assess the following
  1. a) Morphology of the lesion.
  2. b) Soft tissue involvement.
  3. c) Intracranial extension.
4) Digital Subtraction Angiography (D.S.A) is done for accurate evaluation of vascularity of the lesion. It helps to diagnose the feeder vessel and pre-operative embolization to reduce vascularity.
Specialist ENT Screenshot 2020 11 28 Ganpati nose
Staging of JNA:
Radkowski staging:
Stage Description
IA Involvement limited to the nose and or nasopharynx.
IB Extension into one or more sinus.
IIA Minimal extension into the pterygopalatine fossa.
IIB Occupying the entire pterygopalatine fossa with or without erosion of the orbital apex.
IIC Involvement of the infratemporal fossa with or without extension to the cheek or posterior to the Pterygoid plates.
IIIA Erosion of the skull base (the middle cranial fossa/ base of the pterygoids); minimal intracranial extension.
IIIB Erosion of skull base; Extensive intracranial extension with or without cavernous sinus invasion.
Andrew staging: 
Stage Description
I Tumor limited to nasal cavity and nasopharynx.
II Tumor extension into pterygopalatine fossa, maxillary, sphenoid or ethmoid sinuses.
IIIA Extension into orbit or infratemporal fossa without intracranial extension.
IIIB Stage III A with small extradural intracranial (parasellar) involvement.
IVA Large extradural intracranial or intradural extension.
IVB Extension into cavernous sinus, pituitary or optic chiasma.
Fisch staging:
Stage Description
I Tumor limited to the nasopharyngeal cavity, bone destruction negligible or limited to the sphenopalatine foramen.
II Tumor invading the pterygopalatine fossa or the maxillary, ethmoid or sphenoid sinus with bone destruction.
IIIA Tumor invading the infratemporal fossa or orbital region without intracranial involvement.
IIIB Tumor invading the infratemporal fossa or orbital region with intracranial extradural (parasellar) involvement.
IVA Intracranial intradural tumor without infiltration of the cavernous sinus, pituitary fossa or optic chiasma.
IVB Intracranial intradural tumor with infiltration of the cavernous sinus, pituitary fossa or optic chiasma.
Newer Endoscopic staging system:
Stage Description
1 Nasal cavity, medial parapharyngeal fossa.
2 Paranasal sinuses, lateral parapharyngeal fossa; no residual vascularity.
3 Skull base erosion, orbit, infratemporal fossa; no residual vascularity.
4 Skull base erosion, orbit, infratemporal fossa; residual vascularity.
5 Intracranial extension, residual vascularity; medial extension; lateral extension.
Treatment: 1. Surgical excision by:
i. Transnasal maxillary approach.
ii. Transpalatal approach (Wilson’s approach).
iii. Lateral Rhinotomy.
iv. Sublabial mid-facial degloving approach.
v. Infratemporal fossa approach.
vi. Transmaxillary approach (Le fort-I).
vii. Maxillary swing.
viii. Endoscopic resection.
ix. Combined approaches.
A. Combined craniofacial approaches.
B. Combined transpalatal and sublabial (Sardana’s approach).
C. Combined transoral and transnasal endoscopic approach (recent concept). Pre-operative embolization of the tumor drastically reduces the intra-operative hemorrhage.
2. Radiation therapy in usually palliative.

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