Nasopharyngeal Angiofibroma

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:

  • Denasal speech(Rhinolalai clausa)
  • Conductive hearing loss and serous otitis media due to eustachian tube blockage
  • Broadening of nasal bridge
  • Soft palate may bulge due to a large mass in late stages
  • Proptosis
  • Frog-face deformity
  • Swelling of cheek
  • 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

  • Wilson’s Transpalatine approach—done for tumor confined to nasopharynx.
  • Lateral rhinotomy approach—done for large tumors involving, nasal cavity, paranasal sinuses and orbit. 

Other approaches:

  • Sardana’s approach – Transpalatine + Sublabial.
  • Extended Lateral Rhinotomy
  • Intracranial combined with Extracranial.
  • 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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