Juvenile Nasopharyngeal angiofibroma

 The 2nd most common benign tumor of the nasopharynx

 But it is a rare disease accounting for less than 0.05% of all head and neck tumors
(benign tumor of the nasopharynx are rare)

Definition:

1. Benign: non metastatic

2. locally aggressive
3. slowly growing
4. non-encapsulated
5. vasoformative neoplasm: blood vessels lacking smooth muscles in fibrous
tissue
6. covered by the nasopharyngeal mucosa

Epidemyology:
 Seen almost exclusively in male
 More common among adolescents but not invariably.
 Testosterone dependant
 association with familial adenomatous polyposis has been reported

Origin:

 Superior margin of the sphenopalatine foramen.

Symptoms:

1. Progressive Unilateral nasal obstruction: the most common presentation

2. Recurrent sever Epistaxis
3. Unilateral middle ear effusion
4. Proptosis
5. Diplopia
6. cheek swelling with or without pain
7. trismus
8. sinusitis

Exam: nasal mass (80%)

Clinical exam should include nasal endoscopic exam

Life threatening causes:

 bleeding
 intracranial extension:
 occurs in 10%- 36% of all cases
 pituitary, anterior, and middle cranial fossa being the most common sites of
invasion
 Invasion of the skull base by expansion and bone resorption rather than by
cellular infiltration

Extensions of Nasopharyngeal angiofibroma:

1. Nasal cavity
2. Paranasal sinuses: all sinuses except the frontal
3. Pterygomaxillary fossa, infratemporal fossa and cheek.
4. Orbits through:
a. inferior orbital fissure
b. Superior orbital fissure.
5. Cranial cavity:
a. Middle cranial fossa is the most common.

routes of entry:

(i) By erosion of floor of middle cranial fossa, anterior to foramen lacerum. The
tumour lies lateral to carotid artery and cavernous sinus.

(ii) Through sphenoid sinus, into the sella. Tumour lies medial to carotid artery.

(iii) Anterior cranial fossa (through ethmoid roof or cribriform plate).

Pathology:

 Grossly:

 Well-circumscribed, sessile, lobulated/smooth purple-red masses.

 Histologically:

 Fibrous stroma + blood vessels

 Blood vessels: lack of smooth muscle + elastic:
o tumor's hemorrhagic propensity after even minimal manipulation.
o No response to adrenaline

Hormone receptors:

 75% of tumor have androgen receptors

 Small number has progesterone receptors
 No estrogen receptors
 Overproduction of insulin like growth factor is ass with higher risk of recurrence
and poorer prognosis

Radiological imaging:

CT with IV contrast

 the best radiological modality

 antral sign/ Holman-Miller sign ( pathognomonic sign):
Anterior bowing of the posterior wall of the maxillary sinus
 Bowing of the pterygoid plate posteriorly
 Widening of the sphenoplatine foramen

MRI in needed in case of assessment of intra-cranial and intra-orbital extension

 T1 : intermediate signal
 T2 : heterogeneous signal : flow voids appear dark.( salt and pepper
appearance)
 T1 C+ (Gd) - shows prominent enhancement
Diagnostic test: carotid angiography

 delineates the vascular supply

Feeding vessels:
a) external carotid artery system (most commonly) 94%
1. internal maxillary
2. ascending pharyngeal arteries
b) internal carotid artery can contribute to the blood supply of:
1. large tumors
2. intraorbital extension
3. intracranial extension.

Bilateral supply for:

1. Large lesions
2. those that approach the midline

 Therefore, angiography should include the internal and external carotid

systems bilaterally.

 used in cases of doubt about the diagnosis

 it is not necessary to confirm the diagnosis
 Its main indication: to provide preoperative embolization.
 Surgery ideally follows embolization within 24 hours to:
 take advantage of the maximal hemostatic effect
 avoid the inflammatory reaction produced by the embolization

Complications:
 Cerebral embolization because of the presence of anastomosis
that connect the internal and external carotid systems.
 Visual loss can result from accidental embolization of the
ophthalmic artery.

Note: diagnosis is done via CT with IV contrast and confirmed by the diagnostic test
(angiography)

But staging depends on combination of information obtained via CT & MRI

 Staging depends on the extent of the disease not the actual size of the tumor

general description a b c
stage I (post nares/nasopharyngeal vault) Limited involvement of at
least 1 sinus
stage II pterygopalatine fossa min lateral extension full occupation extension into the infra-
temporal fossa/post to
the pterygomaxillary
stage III skull base and intracranial min extension extensive
extension

Stage 1a=stage 1 fisch

Stage 1b+ 2a+b + bone erosion=stage 2

stage 3 stage4
infra-temporal cavernous sinus thrombosis
orbit optic chiasma
para-sellar pituitary sella
Fisch classification is more practical
Treatment:

 Surgery :
1. The treatment of choice
2. surgical approach is determined by:
1. tumor location
2. Extent
3. surgical expertise
 All patients should undergo preoperative embolization of feeding vessels,
usually within 24 hours of surgery, and early endoscopic control of the
internal maxillary artery before tumor mobilization

a. Endoscopic trans-nasal resection:

 used for tumor limited tumor (Fisch stage I,II,SOME 3): nasopharynx, nasal
cavity, sinuses, pterygoid fossa
 b. Open Approach:
1. Midfacial degloving: the most commonly used for large tumor
2. Infratemporal approach: for intra-cranial extension
3. Lateral rhinotomy:
4. Trans-palatal

 radiation therapy:
 Indications:
1. advanced disease with significant intracranial extension (cause it
has internal carotid blood supply)
2. Poor surgical candidate
3. Recurrent angiofibroma

 response to radiotherapy is very slow :the effect may not be fully apparent
for 1 to 2 years

 Tumors that extend through the sella and insinuate between the pituitary
medially and internal carotid laterally are especially hazardous and difficult
to manage surgically and need combination therapy

Recurrence

 Common feature of JNAs

 Rates ranging from 30% - 46%(in scott 25% regardless of the approach)

 Recurrence usually becomes evident within 2-3 years of the initial resection.

 Higher risk of recurrence:

1. Sphenoid involvement: 93% of recurrences occurred in patients with

radiologic evidence of sphenoid diploe invasion through the pterygoid
canal and that the recurrence rate per patient was proportional to the
degree of sphenoid invasion.
2. Age: the younger the higher is the risk
3. Advancement of the tumor
4. Timing of surgery, either during the aggressive growth phase or latent
phase of the tumor, may be partially responsible for the variable local
failure rates despite resection under similar conditions.
5. Pre-operative angiography: increase the risk of Recurrence

 Small residual tumor may be regress spontaneously

 Residual tumor is usually asymptomatic
 All patients with residual disease are at risk of future regrowth or recurrence.
For these patients, lifetime monitoring and assessment is necessary.
 Serial interval MRI should become a standard of care for these patients.
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