Nasopharyngeal Angiofibroma

NASOPHARYNGEAL ANGIOFIBROMA
  • It is a histologically benign but locally aggressive vascular tumor that grows in the back of the nasal cavity.
  • It is the most common benign neoplasm of nasopharynx.
  • It is a highly vascular tumor.It consists of fibrous tissue and blood vessels,
  • Blood supply of the tumor most commonly arises from the internal maxillary artery. 
  • The exact cause is unknown. As the tumour is predominantly seen in adolescent males in the second decade of life, it is thought to be testosterone dependent.
  • Such patients have a hamartomatous nidus of vascular tissue in the nasopharynx and this is activated to form angiofibroma when male sex hormone appears.
  • Most common site is posterior part of nasal cavity close to the margin of sphenopalatine foramen.
  • It may expand into the surrounding areas like nasal cavity,sphenoid sinus,pterygopalatine fossa,maxillary sinus,orbit and cranial cavity.
CLINICAL PRESENTATION OF NASOPHARYNGEAL ANGIOFIBROMA
  • Sex : Seen almost exclusively in males (testosterone dependent).
  • Age : 10-20 years (2nd decade).
  • Clinical features: - Most common symptom is profuse and recurrent epistaxis Progressive nasal obstruction
  • Other features depending upon the extension of the tumour:
  1. Denasal speech(Rhinolalai clausa)
  2. Conductive hearing loss and serous otitis media due to eustachian tube blockage
  3. Broadening of nasal bridge
  4. Soft palate may bulge due to a large mass in late stages
  5. Proptosis
  6. Frog-face deformity
  7. Swelling of cheek
  8. Involvement of cranial nerves II, Ill, IV, VI
INVESTIGATIONS IN A CASE OF NASOPHARYNGEAL ANGIOFIBROMA
  • Radiography:it shows a soft tissue shadow in the nasopharynx.
  • Lateral view shows forward displacement of the posterior wall of maxilla.
  • Angiography and CT Scan with contrast deomonstrate the extent and vascularity of the tumor.
  • Biopsy should never be attempted as it may lead to profuse bleeding.
  • Instead,Excision biopsy is performed.
TREATMENT OF NASOPHARYNGEAL ANGIOFIBROMA
Surgery
  • Surgical excision is the treatment of choice.
  • Before surgery at least 2-3 liters of blood should be given. 
  • Preoperative embolization and estrogen therapy or cryotherapy reduce blood loss in surgery.
  • Hypotensive Anesthesia in nasopharyngeal angiofibroma is/are given by Propofol and Phentolamine.
Approach
  1. Wilson's Transpalatine approach—done for tumor confined to nasopharynx.
  2. Lateral rhinotomy approach—done for large tumors involving, nasal cavity, paranasal sinuses and orbit. 
Other approaches:
  1. Sardana's approach - Transpalatine + Sublabial.
  2. Extended Lateral Rhinotomy
  3. Intracranial combined with Extracranial.
  4. Infratemporal fossa approach.
Radiation
  • It is sometimes advised to reduce the vascularity of the tumor.
  • It may be considered in cases with intracranial extension.
Hormonal Therapy
  • It is doubtful if tumour regression occurs with this therapy.
Exam Question
  • Nasopharyngeal angiofibromas are most frequently seen in the second decade.
  • Hypotensive Anesthesia in nasopharyngeal angiofibroma is/are given by Propofol and Phentolamine.
  • Nasopharyngeal fibroma is the most common benign tumor of nasopharynx.
  • Clinical features of nasopharyngeal angiofibroma are Adolescent male and Epistaxis and nasal obstruction is the cardinal symptom.
  • A 14 years boy presented with repeated epistaxis, and a swelling in cheek.Diagnosis is nasopharyngeal angiofibroma and Contrast CT scan should be done to see the extent.
  • Surgery is treatment of choice in juvenile Nasopharyngeal Angiofibroma.
  • The most probable diagnosis in a 14-year old boy presenting with history of frequent nasal bleeding and Hb found to be 6.4 g/dL and peripheral smear showing normocytic 
  • hypochromic anemia is Juvenile nasopharyngeal angiofibroma.
  • Most probable diagnosis in a child with unilateral nasal obstruction along with a mass in cheek and profuse and recurrent epistaxis is Nasopharyngeal Angiofibroma.
  • Hormonal etiology in implicated in Nasopharyngeal Angiofibroma.
  • Radiotherapy may be given in Nasopharyngeal Angiofibroma.

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