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Nasopharyngeal Angiofibroma. Dr. Vishal Sharma. Definition. Benign tumor of nasopharynx (?), locally invasive, extremely vascular & occurs in adolescent males. Hamartomatous nidus of vascular tissue, dependent on testosterone. Synonyms: nasopharyngeal fibroma,

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definition
Definition
  • Benign tumor of nasopharynx (?), locally invasive, extremely vascular & occurs in adolescent males.
  • Hamartomatous nidus of vascular tissue, dependent on testosterone.
  • Synonyms:nasopharyngeal fibroma,

angiofibroma

site of origin
Site of origin

Arises in posterior nasal cavity, near superior

border of sphenopalatine foramen

pathology
Pathology

Gross: Sessile, bi-lobed, rubbery, red-pink or gray in colour. Histology:Encapsulated, composed of vascular tissue & fibrous stroma. Vessels are thin-walled, lack elastic fibers & smooth muscle (this leads to uncontrolled bleeding).

spread
Spread

Anterior:Nasal cavity + paranasal sinus

Posterior:Nasopharynx

Lateral:goes to Pterygopalatine fossa

1. Infratemporal fossa  cheek

2. Inferior orbital fissure  orbit

spread20
Spread

Superior:1. Sphenoid sinus

 Middle cranial fossa

 Cavernous sinus

 Optic chiasma

 Pituitary fossa

2. Skull base

 Middle cranial fossa

symptoms
Symptoms

1. Nasal obstruction(80-90%)with denasal

speech (rhinolalia clausa)

2. Epistaxis (50-60%): Persistent, Painless,

Profuse, Paroxysmal, Unprovoked

3. Headache (25%)

4. Facial swelling (20%): cheek & palatal swelling

signs
Signs

1. Nasal or Nasopharyngeal mass (80%)

2. Frog-face deformity:

proptosis + nasal bridge broadening

3. Otitis media with effusion: due to E.T. blockage

4. Trismus: involvement of pterygoid muscle

5. Involvement of II, III, IV, VI cranial nerve

c t scan p n s with contrast
C.T. scan P.N.S. with contrast
  • Extent of tumor
  • Anterior bowing of posterior maxillary wall (Miller Holman’s antral sign)
  • Tumor enhancement
  • Bone destruction
other investigations
Other Investigations

 M.R.I.:for intra-cranial involvement

 Digital Subtraction Angiography (D.S.A.):a. extent of tumor b. tumour blush (due to increasedvascularity)c. feeding arteries for embolization

 Biopsy: contraindicated (profuse bleeding)

differential diagnosis
Differential diagnosis
  • Rhabdomyosarcoma
  • Antrochoanal polyp
  • Teratoma
  • Dermoid
  • Encephalocoele
  • Inverting papilloma
  • Squamous cell carcinoma
staging
Staging

Stage I: Tumor limited to nasal cavity or

nasopharynx with no bony destruction Stage II:Tumor invading pterygopalatine

fossa or paranasal sinusesStage III: Tumor invading infratemporal

fossa or orbit or parasellar region Stage IV:Tumor invading cavernous sinus

or optic chiasma or pituitary fossa

pre op reduction of tumor vascularity
Pre-op reduction of tumor vascularity

1. Embolization of feeding arteries: with Gelfoam

2. Oestrogen therapy:Diethylstilbestrol (2.5 - 5

mg orally t.i.d. for 3 - 6 wk)

3. Testosterone receptor blocker:Flutamide

4. Pre-operative radiotherapy

5. Cryotherapy of tumor

denker s incision
Denker’s incision

Caldwell Luc incision extended medially till midline

surgical approaches
Surgical approaches

1. Trans-palatal approach (Wilson)

small tumour in nasopharynx

2. Sublabial + Trans-palatal approach (Sardana) large tumour of nose + PNS + nasopharynx

3. Intranasal endoscopic approach small tumour in nose / PNS / nasopharynx

surgical approaches45
Surgical approaches

4. Transmaxillary approach via:

 Extended lateral rhinotomy incision

 Midfacial degloving incision

 Denker’s extended Caldwell-Luc incision

 Le Fort 1 osteotomy approach

Done for extension into pterygopalatine fossa

surgical approaches46
Surgical approaches

5. Infratemporal fossa approach (Fisch) extension into infratemporal fossa

6. Anterior subcranial approach intracranial & orbital extension

7. Image-guided, endoscopic, laser-assisted

removal (latest): small / medium size tumors

proton stereotactic radiation therapy p s r t
Proton Stereotactic Radiation Therapy (P.S.R.T.)

Synonym:Gamma knife surgery

Used for: 1. Intracranial extension

2. Recurrence after surgery

  • Single relatively high dose of radiation delivered precisely to a small area to kill tumorcells
  • Minimal injury to adjacent nerves & brain tissue