Documenti di Didattica
Documenti di Professioni
Documenti di Cultura
Nasopharyngeal
Angiofibroma
Alok Thakar*
MS, FRCSEd
Ashutosh Hota*
MS
S Pookamala*
MS
Abstract
Juvenile nasopharyngeal angiofibroma (JNA) continues to provide clinical and surgical challenges
consequent to its high vascularity, tendency to intracranial extension, and propensity to postsurgical recurrence. This review encompasses the current concepts with regard to its origin and
progression, and discusses the principles of pre-surgical preparation and surgical excision.
Keywords
Introduction
*
Department of
Otolaryngology and HeadNeck Surgery,
All India Institute of
Medical Sciences,
New Delhi
Correspondence:
Alok Thakar
Additional Professor of
Otolaryngology and
Head-Neck Surgery,
All India Institute of Medical
Sciences,
Ansari Nagar,
New Delhi 110029
India
Email: drathakar@
gmail.com
Histology
Histologically, the tumour is unencapsulated
and consists of a collagenous tissue stroma
interspersed with wide vascular spaces
lined by a single endothelial layer. These
arterial vascular channels are unusual in not
manifesting a complete muscular layer in the
vessel wall (Figure 1). The lack of muscle layer
in the vessels is claimed to be the histological
explanation for the profuse epistaxis noted
in this condition, as it is supposed that these
arterial vessels are deficient in their ability
to contract in response to tissue injury and
bleeding.7
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Nasopharyngeal Angiofibroma
lateral extension leads to the tumour exiting via the pterygomaxillary fissure to expand into the infra temporal fossa.6
Superior extension to the sphenoid sinus is by erosion
of its floor and anterior wall (Figure 4B). The tumour may
also extend posteriorly into the vidian canal and therein erode
medially into the sphenoid sinus.
Intracranial Extension Intracranial extension of JNA
has been reported to occur in about 20%.10 Intracranial JNA
is almost always extradural, and usually to the middle cranial
fossa (peri-cavernous). Extension into the middle cranial fossa
is usually by one of three routes9 (Figure 5 A, B, C):
1. Tumour extension via the inferior orbital fissure into
the orbit (Figure 2B), and subsequent posterior
extension of orbital tumour via the superior orbital
fissure into the middle cranial fossa.8,9 (Figure 5A tumour location antero-lateral to cavernous sinus).
This is the most common route for intracranial
extension.
2. Expansion of tumour in the sphenoid sinus with
erosion of its lateral wall.9 (Figure 5B- tumour location
medial to cavernous sinus)
3. Expansion of tumour into the pterygoid base and the
cancellous diploe of the sphenoid bone, and further
superior erosion of the greater wing of sphenoid with
extension into the middle cranial fossa.8,9 (Figure 5Ctumour location lateral and inferior to cavernous sinus).
Figure 2: (A, B) Axial and Coronal MR scans demonstrating the salt and pepper appearance of JNA, and tumour extensions from the
pterygopalatine fossa into the nasopharynx, nose, infratemporal fossa and posterior orbit.
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Nasopharyngeal Angiofibroma
Figure 3: CT images demonstrating posterior displacement of the pterygoid base (3A) and erosion of the pterygoid base (3B). Figure 3a
also displays anterior bowing of the posterior maxillary wall and expansion of the pterygo-palatine fossa ( Holman Miller sign), and figure 3b
additionally displays the erosion of the ipsilateral vidian canal.
Clinical features
Figure 4: Typical and diagnostic radiological signs of JNA include tumour enhancement with contrast, (4A), expansion of the pterygomaxillary fissure with anterior bowing of the postero-lateral maxillary wall (4A); .erosion of the sphenoid floor with direct tumour extension
from the nasopharynx to the sphenoid (4B), and erosion of the vidian canal by the tumour (4B). The contra-lateral vidian canal is well
visualized (white arrow).
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Nasopharyngeal Angiofibroma
Figure 5: Typical routes of JNA spread to the middle cranial fossa. The most frequent extension to the MCF is tumour extension via the
superior orbital fissure (5A). Other routes of extension are by erosion of the lateral sphenoidal wall (5B) and by invasion and erosion of the
cancellous bone in the body and greater wing of sphenoid (5C).
Classification
Various systems of classification exist for angiofibroma. The
Radkowski`s classification (Table 1)12 is currently popular,
and increasing stages have been correlated with incremental
rise in tumour recurrences.13
Investigations
Imaging
IB-
Tumour involving posterior nares and/or nasopharyngeal vault with involvement of at least one Para nasal sinus
Stage II:
IIA-
IIB-
Full occupation of the pterygo-maxillary fossa with or without superior erosion of orbital bones
IIC-
IIIA-
Erosion of the base of skull (middle cranial fossa/ base of pterygoids)-minimal intracranial extension
IIIB-
Extensive intracranial extension with or without extension into the cavernous sinus.
Stage III:
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Nasopharyngeal Angiofibroma
appearing as dark flow voids.9 The tumour enhances
strongly with gadolinium and tumour extensions are clearly
demonstrated. MR is especially useful for assessment of intra
cranial tumours abutting the cavernous sinus and the ICA,
and for follow up evaluations for residual/recurrent tumour.
Angiography: Angiography is of diagnostic value with
its characteristic tumour blush. It provides information
about the major vascular supply and allows for pre-operative
embolisation. The major arterial supply to these tumours is
typically the ipsilateral internal maxillary artery, with occasional
additional vessels from ascending pharyngeal artery, and
branches from cavernous ICA or contra lateral external carotid
system14 (Figure 6).
Treatment
Surgical Treatment
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Nasopharyngeal Angiofibroma
Table 2: Commonly used surgical approaches for excision of
Juvenile Nasopharyngeal Angiofibroma.
Nasal Endoscopic
Trans palatal
Trans palatal approach
Trans palatal with sub labial extension
Trans maxillary
Lateral rhinotomy / Medial maxillectomy
Midfacial degloving
Le-fort I osteotomy approach
Maxillary swing / Facial Translocation approach
Lateral skull base approach
Pre auricular sub temporal infra-temporal approach
Infratemporal approach Type C
Combination of any two approaches
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and further may aid disease clearance from the recesses in the
sphenoid bone.22,32
Limits of endoscopic surgery: The endoscopic approach
limits the free space to work around the periphery of tumour.
The limited access through the nasal aperture restricts the
role of an assistant in providing a clear surgical field and in
providing traction on the tumour to aid its dissection.32 Even
minimal bleeding can obscure the surgical view. Involvement
of the cavernous sinus, or tumour extension toward the
internal carotid artery through the foramen lacerum are
associated with a risk of uncontrollable bleeding and probably
represents the limits of endoscopy.31,32,34
Radiotherapy
Residual/recurrent tumour
Conclusion
Conflict of Interest
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Nasopharyngeal Angiofibroma
Key Points
JNA is a highly vascular benign tumour with characteristic patterns of spread. The peculiarity of it being restricted to adolescent males,
and also its deep location and spread in the central skull base, has provided for intellectual and surgical challenges with regard to its
origins and treatment.
Current concepts localize its origins to the vidian canal and pterygoid base. The tumour has been demonstrated to manifest receptors
for testosterone, and also for estrogen and progesterone.
Diagnosis is based on the characteristic CT and MR findings demonstrating typical patterns of spread. Tissue biopsy and angiography are
not currently considered necessary for establishing a pre-operative diagnosis.
Surgical excision is the treatment of choice. Radiation therapy may be used for the rare situations with significant intracranial spread
wherein surgical excision is deemed as high risk.
Residual and recurrent disease following surgical excision has been frequent in previous times. Improved radiology in current times
allows for a complete assessment of disease extensions, and the selection of appropriate surgical approaches for improved surgical
excisions.
Surgical excisions may be improved and surgical residual disease minimized by attention to pre-surgical optimization (tumour shrinkage
by anti-androgen therapy, and pre-operative embolisation), and the surgical principles of adequate tumour exposure, control of vascular
supply, lateral to medial dissection/ sub-periosteal dissection, and post excision drilling of the pterygoid base and vidian canal.
The evolution of endoscopic techniques and instrumentation in recent times has provided for its application as a low morbidity option
for early stage tumours. Further evolution of expertise and experience is expanding its role for larger stage tumours.
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CPD Assessment
Approved by ENT-UK for Continuing Professional Development
Satisfactory completion of the CPD assessment related to any single article, that is attaining
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only claim credit commensurate with the extent of their participation in the activity.
Nasopharyngeal Angiofibroma
1. The propensity for intractable and massive
haemmorrage in JNA is ascribed to
a. Sequestration of platelets in the venous channels
b. High flow arteriovenous communications
c. Consumption coagulopathy and metabolic acidosis
d. Inadequate muscular contraction in intra-tumoral arterial
vessels.
2.
a.
b.
c.
d.
9.
a.
b.
c.
d.
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CPD Assessment
www.theotorhinolaryngologist.co.uk
Diagnostic Quiz Questions
1. Pre operative imaging of 12 year male with
nasopharygeal angiofibroma revealed localized
tumor involving the right posterior nose and
nasopharynx and lateral extension posterior to the
pterygoid plates.
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