Acta Radiologica

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Radiological Findings in Angiofibroma

B. Schick & G. Kahle

To cite this article: B. Schick & G. Kahle (2000) Radiological Findings in Angiofibroma, Acta
Radiologica, 41:6, 585-593, DOI: 10.1080/028418500127345956

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Acta Radiologica 41 (2000) 585–593 Copyright C Acta Radiologica 2000
Printed in Denmark ¡ All rights reserved
AC TA R A D I O L O G I C A
ISSN 0284-1851

Review Article

RADIOLOGICAL FINDINGS IN ANGIOFIBROMA

B. S1 and G. K2

1
Department of Ear, Nose and Throat Diseases, Head-, Neck- and Facial Plastic Surgery, Communication Disorders, and
2
Institute of Radiology, University Hospital Fulda, Academic Teaching Hospital at the University of Marburg, Marburg,
Germany.

Abstract
Surgery after pre-operative embolization has become the main treatment mo- Key words: Head and neck,
dality in angiofibroma therapy. As surgical planning is based on precise pre- angiofibroma; CT; MR imaging;
operative tumour evaluation, knowledge of the characteristic growth patterns paediatric.
is of great interest. Analysis of tumour extension and blood supply, as well as
methods of controlling intra-operative bleeding, help in determining the appro- Correspondence: Bernhard Schick,
priate surgical approach. Though benign, angiofibroma demonstrates a locally Department of Ear-, Nose- and
aggressive nature. This fibrovascular tumour is characterised by typical radio- Throat Diseases, University Hospital
logical findings and by predictable growth patterns. The tumour extension and Fulda, University of Marburg,
blood supply can be accurately determined by CT, MR imaging and angiogra- Pacelliallee 4, D-36043 Fulda,
phy. With classic radiological findings, no pre-operative biopsy is necessary in Germany. FAX π49 661 84 6002.
most angiofibromas. Advances in radiological imaging have contributed to im-
proved surgical planning and tumour resection. The surgeon is able to select Accepted for publication 26 April
the least traumatic approach with secure haemostatic control, which is also 2000.
critical for avoiding the disturbance of facial skeletal growth in this group of
young patients. Embolization, pre-operative autologous donation and the cell
saver system for immediate retransfusion of the collected blood after filtration,
are important tools for dealing with blood loss in angiofibroma surgery as they
minimize homologous blood transfusion.

Angiofibromas represent less than 0.5% of all head
and neck tumours (8). They occur almost exclus-
ively in adolescent males and were therefore pre-
viously termed juvenile angiofibroma. The age of
diagnosis most commonly ranges between 14 and
25 years (10) and manifestation in other age
groups or females is rare. The tissue of origin is
still unclear. Angiofibromas usually arise in the re-
gion of the posterior nares in close relationship to
the sphenopalatine foramen (4). Although benign,
they show aggressive growth behaviour and spread
through natural foraminas and fissures (8).
Surgery is the main treatment modality (20). Ex-
act determination of the site of origin, extension
and blood supply of this fibrovascular tumour is
required prior to surgery. Due to the characteristic
endothelial-lined vascular spaces with little or no
smooth muscle layers, angiofibromas tend to bleed
profusely. Therefore, methods to reduce intra-op-
erative blood loss (pre-operative embolization, cell
saver) have attracted much attention as they mini-
mize the need for transfusion during surgery.
In this article we present the specific radiological
findings in angiofibromas based on experience in
dealing with this rare tumour over an 18-year
period.
Tumour imaging
From June 1, 1980 until December 31, 1998, 36
angiofibromas were treated at the University Hos-
pital Fulda. Thirty-six CT and 22 MR examina-

585
 B. SCHICK AND G. KAHLE
Fig. 1. Axial CT after application of contrast medium indicates
typical angiofibroma location in the posterior nasal cavity with
extension to the nasopharynx.
Fig. 2. Bony destructions of the medial pterygoid plate caused
by angiofibroma in the early stage are seen on coronal CT.
tions were available for re-evaluation of specific tu-
mour growth patterns. CT at 2- to 4-mm in-
crements had been obtained in the axial plane
before and after contrast medium injection (Soma-
tom Plus, Siemens). Coronal and sagittal recon-
structions had been performed from the CT data.
The MR imaging protocol included axial, coronal
and sagittal investigations in T1- and T2-weighted
sequences (1.5 T, Philips). Gadolinium-DTPA was
used as contrast agent at MR imaging.
CT and MR imaging are used to determine the
586
tumour site and its extension with special attention
to skull base involvement, intracranial spread and
relationship to important vascular and neurologic
structures. While CT defines the bony changes in
detail, MR is superior in distinguishing neoplasia
from mucosal swelling, determining fluid retention
in the paranasal sinuses as well as evaluating more
accurately the intracranial extension.
Angiofibroma present as a hypervascular space
occupying lesion in the posterior nasal cavity (Fig.
1). Tumour extension into the nasopharynx is
common, which historically resulted in the er-
roneous conclusion that the site of origin was the
nasopharynx. This false assumption led to the
questionable term nasopharyngeal angiofibroma.
As the nasopharynx is only secondarily involved,
the term ‘‘nasopharyngeal’’ is a misnomer. Erosion
of the medial pterygoid plate (Fig. 2) and widening
of the sphenopalatine foramen (Fig. 3a) with tu-
mour spread into the pterygopalatine fossa (Fig.
3b) are early findings at CT. Anterior bowing of
the posterior maxillary wall – known as the Hol-
man-Miller sign – is a characteristic finding in
Fig. 3. Widening of the sphenopalatine foramen on axial CT
(a) and tumour spread into the pterygopalatine fossa on Gd-
enhanced axial MR image (b), being a common route of angio-
fibroma extension.
Fig. 4. Sagittal CT reconstruction after contrast medium appli-
cation shows destruction of the sphenoid sinus floor with an
angiofibroma filling the sphenoid sinus.
 RADIOLOGICAL FINDINGS IN ANGIOFIBROMA

area of the foramen lacerum is occasionally ob-

served (Fig. 6). More common is the spread into
the infratemporal fossa from the pterygopalatine
fossa (Fig. 7). Further lateral extension with in-
volvement of the cheek will present as facial swell-
ing. Invasion of the orbit can lead to proptosis and
optic nerve compression with visual disturbances
(Fig. 8). Intracranial spread is seen through the
roof of the infratemporal fossa (Fig. 9), the su-
perior orbital fissure, or the sphenoid sinus. Intra-
cranial extension is usually extradural (6).
Angiofibromas more often displace the dura or
may to some extent adhere to this important
boundary. Dural infiltration or involvement of the

Fig. 5. Axial CT indicates bony erosions of the clivus in angio-

fibroma.

Fig. 7. Contrast-enhanced axial CT shows infratemporal fossa

involvement of angiofibroma.

Fig. 6. Axial MR image after Gd-DTPA. Tumour growth pos-

terior to the pterygoid base with extension to the foramen la-
cerum is demonstrated as a rare finding in angiofibromas.

angiofibroma with invasion of the pterygopalatine

fossa (20). By destroying the sphenoid sinus floor,
angiofibromas are able to extend into the sphenoid
sinus (Fig. 4). Bony destruction of the clivus can
be observed (Fig. 5). These bony changes are rarely
caused by cellular infiltration, but are the result of
a growth pattern characterised by expansion. The
ipsilateral ethmoid and maxillary sinuses, as well
as the nasal cavities and contralateral sinuses, can
all be affected during further tumour growth. Fig. 8. Axial CT after contrast medium proves angiofibroma
Tumour extension along the central skull base extension to the orbit, causing visual impairment (1), and intra-
posterior to the pterygoid plates extending to the cranial angiofibroma spread (2).

587
 B. SCHICK AND G. KAHLE

imaging and angiography with such a high degree

Fig. 9. Contrast-enhanced coronal CT reconstruction. Intra-
cranial tumour extension through the roof of the infratemporal
fossa.
of certainty that pre-operative biopsy will usually
not be needed. In our series, a rather rare side-
effect of pre-operative biopsy was the development
of a pseudoaneurysm 6 weeks after biopsy had
been performed at another centre (Fig. 12a, b).
Apart from the typical site of origin at the lat-
eral margin of the posterior nares, angiofibromas
are seldom found in atypical sites. A review of the
literature dating from January 1980 to the end of
1997 revealed 46 unusually located angiofibromas,
requiring us to add three additional descriptions
of atypical tumour locations (16). We have seen a
fourth atypical location in the oropharynx (Fig.
13a, b) with no nasal or nasopharyngeal involve-
ment.

Classification of angiofibromas
Different classification systems relying on the
radiological findings have been proposed for

Fig. 10. Coronal MR image. Intradural tumour invasion with

compression of the left temporal lobe and additional encapsula-
tion of the left internal carotid artery as well as involvement of
the cavernous sinus in a case of an extended angiofibroma.

brain has to be considered a rare event (Fig. 10).

In cases of extensive intracranial tumour growth, Fig. 11. Coronal MR image after Gd-DTPA. Cystic tumour
the internal carotid artery can be affected and the portion in an angiofibroma.
tumour may finally end up in the cavernous sinus
(Fig. 10).
Cystic changes in the tumour are a rare radio-
logical finding (Fig. 11). In our department, cystic
areas were observed in 2 of 35 surgically treated
angiofibromas. Histological evaluation revealed
these to be degenerative cysts within the tumour
tissue in both cases. Cystic changes may therefore
be interpreted as a radiological sign of tumour re-
gression. This assumption was further supported
in our series by the observation of cystic tumour
regression in a huge angiofibroma after chemo-
therapy (15). One should be aware, that spon-
Fig. 12. After superselective embolization with polyvinyl alco-
taneous regression of angiofibroma has only rarely hol particles: an unusual finding of a pseudoaneurysm after
been documented in the literature (3). biopsy at Gd-enhanced coronal MR imaging (a) and at angio-
Angiofibroma can be diagnosed using CT, MR graphy (b).

588
 RADIOLOGICAL FINDINGS IN ANGIOFIBROMA
Fig. 13. A rare finding of angiofibroma in the oropharynx at
(a) axial CT after contrast medium application with (b) angio-
graphic proof of a hypervascular tumour fed from the maxillary
artery.
angiofibromas (Table 1). The one suggested by
J et al. (9) as early as 1980 did not find wide
use. The classification system by C et al.
(2) is based on that for nasopharyngeal cancer by
the American Joint Committee. The classification
given by F (6) and S et al. (17) reflect
more precisely on the growth pattern of angio-
fibromas. The classification system proposed by
A et al. (1) is a revision of the system intro-
duced by F (6). R et al. (14) sug-
gested a revised classification based on the system
proposed by S et al. (17). Tumour extension
posterior to the pterygoid plates as well as the dif-
ferentiation between minimial or extensive intra-
cranial tumour spread was added by R
et al. (14).
Angiography
Angiography is used to define the tumour blood
supply, the vascular composition of the tumour,
and its venous drainage. As the tumour usually
starts in the area of the posterior nasal cavity in
close relationship to the sphenopalatine foramen,
the sphenopalatine or maxillary artery are found
to feed the tumour in early stages (Fig. 14). Other
branches of the external carotid artery, especially
the ascending pharyngeal artery, participate in tu-
mour blood supply in more extended tumours.
Branches of the internal carotid artery (Fig. 15)
and the vertebral artery (Fig. 16) are additional
feeders in advanced tumours. As the tumour blood
supply can derive from both sides, the external and
internal carotid arteries as well as the vertebral ar-
tery should be examined bilaterally. An inhomo-
geneous and intensive blush is typical for angio-
fibromas. Angiography was available in 28 out of
our series of 36 angiofibromas. The feeding vessel
frequency in this series is listed in Table 2.
Pre-operative balloon-test occlusion of the inter-
nal carotid artery is advised in cases where im-
aging indicates a risk of intra-operative internal
carotid artery injury. This assesses collateral cer-
ebral blood supply (5, 21). When tolerated, the oc-
clusion test is carried out for 30 min in our routine
skull base practise. The patient is monitored
neurologically and single photon emission CT
(SPECT) analysis of brain perfusion is carried out
before and after the test occlusion.
Embolization
Angiofibroma resection presents the surgeon with
the potential problem of profuse bleeding and
methods of reducing this risk have attracted much
attention. Apart from the risk of infections with
homologous blood transfusions, the low tolerance
of blood loss in children makes such efforts even
more pertinent. Pre-operative embolization was in-
troduced to decrease the need of blood transfusion
and to facilitate tumour resection.
The role of pre-operative tumour embolization
has been controversial (18). Proponents of pre-op-
erative embolization stress its effectiveness in re-
ducing blood loss and a drier surgical field. They
point to better results and, in particular, lower
morbidity and mortality. Some authors, however,
found that embolization reduced blood loss only
in high-grade angiofibromas (12) or did not reduce
blood loss at all.
The improved bleeding control due to em-
bolization correlated either with a lower recurrence
rate or an increased incidence of incomplete tu-
mour resection due to failure to notice non-
bleeding tumour tissue during surgery (18). A criti-
cal discussion of the potential side effects of em-
bolization has been provided by P & C-
 (13).
Many contemporary surgeons recommend em-
bolization followed by tumour resection as the
treatment modality of choice (5, 8, 20). Because
angiofibromas are rare, most series are, however,
small. In addition, in both the embolized and the
non-embolized group, there are tumours of various
stages. As there have been no prospective studies,
statistical evaluation is difficult. In retrospective
analyses, U et al. (20) and S et
al. (18) have highlighted the effectiveness of pre-
operative embolization followed by surgery.
Superselective embolization of feeding vessels
arising from the external carotid artery (Fig. 17a,
b) is highly effective and safe (7). In 27 out of 35
surgically treated angiofibromas at our hospital,
superselective embolization of the tumour from ex-
ternal carotid artery feeders has been performed
without any side-effects. Anastomosis between
589
590
Johns et al., 1980
I. Disease confined to the
nasopharynx and nasal cavity
II. Disease involving nasopharynx
or nasal cavity with extension to
the pterygomaxillary fossa and/or
maxillary sinus
III. Involvement of more than the
anatomic sites listed in II, but
without intracranial involvement
IV. Intracranial extension
Chandler et al., 1984
I. Tumour confined to
nasopharynx
II. Tumour extending into
nasal cavity and/or sphenoid
sinus
III. Tumour extending into one
or more of the following:
antrum, ethmoid sinus,
pterygomaxillary fossa,
infratemporal fossa, orbit, and/
or cheek
IV. Tumour extending into
cranial cavity
Table 1
Synopsis of classification systems for angiofibromas
Fisch, 1983
I. Tumour limited to the
nasopharynx and nasocavity
with no bone destruction
II. Tumour invading the
pterygomaxillary fossa, the
maxillary, ethmoid and
sphenoid sinuses with bone
destruction
III. Tumour invading the
infratemporal fossa, orbit and
parasellar region remaining
lateral to the cavernous sinus
IV. Tumour with massive
invasion of the cavernous
sinus, the optic chiasmal region
or pituitary fossa
Andrews et al., 1989
I. Tumour limited to the
nasopharynx and nasal cavity.
Bone destruction negligible or
limited to the sphenopalatine
foramen
II. Tumour invading the
pterygomaxillary fossa or the
maxillary, ethmoid, or
sphenoid sinus with bone
destruction
IIIa. Tumour invading the
infratemporal fossa or orbital
region without intracranial
involvement
IIIb. Tumour invading the
infratemporal fossa or orbital
region with intracranial
extradural (parasellar)
involvement
Sessions et al., 1981 Radkowski et al., 1996
IA. Tumour limited to IA. Limited to nose and/or
posterior nares and/or nasopharyngeal vault
nasopharyngeal vault. No
paranasal sinus extension
IB. Same as IA but with IB. Extension into
extension into one or more Øone sinus
paranasal sinuses
IIA. Minimal lateral extension IIA. Minimal extension into
through the sphenopalatine pterygomaxillary fossa
foramen, into and including a
minimal part of the medial-
most part of the
pterygomaxillary fossa
IIB. Full occupation of the IIB. Full occupation of
pterygomaxillary fossa, pterygomaxillary fossa with or
displacing the posterior wall of without erosion of orbital bone
the maxillary antrum forewards.
Lateral and/or anterior
B. SCHICK AND G. KAHLE

IVa. Intracranial intradural
tumour without infiltration of
the cavernous sinus, pituitary
fossa, or optic chiasm
IVb. Intracranial intradural
tumour with infiltration of the
cavernous sinus, pituitary
fossa, or optic chiasm
displacement of branches of
the maxillary artery. Superior
extension may occur, eroding
the orbital bones
IIC. Extension through the
pterygomaxillary fossa into
the cheek and temporal fossa
III. Intracranial extension
IIC. Infratemporal fossa with
or without cheek or posterior to
pterygoid plates
IIIA. Erosion of skull base –
minimal intracranial
IIIB. Erosion of skull base –
extensive intracranial with or
without cavernous sinus
 RADIOLOGICAL FINDINGS IN ANGIOFIBROMA

Attempts to embolize feeding vessels arising

from the internal carotid artery are associated with
the risk of severe intracranial complications and
hemiparesis and blindness are possible severe side
effects. Embolization should therefore only be car-
ried out by experienced radiologists. The benefits
associated with reducing the tumour blood supply
from the internal carotid artery in addition to
selective embolization from branches of the exter-
nal carotid artery in large angiofibromas must be
judged to clearly outweigh the risks.
As an alternative to transvascular embolization,
direct intratumoral embolization with the advan-
tage of a lower risk of neurological complications
has been recommended (19). This technique has,
in small series, led to a decreased intra-operative
blood loss. Direct pre-operative intratumoral em-

Fig. 14. Tumour blood supply from the maxillary artery.

Fig. 15. Angiofibroma with angiographic proof of blood supply

from branches of the internal carotid artery.
Fig. 16. Angiography showing tumour blood supply from the
left vertebral artery in a large angiofibroma with intracranial
branches of the external and internal carotid ar- extension.
tery and vascular spasm have to be considered
when planning superselective embolization. Mi-
gration of embolization particles to the ophthal- Table 2
mic artery, the cerebral or vertebral arteries via an-
Angiographic findings of tumour blood supply in 28
astomosis or reflux of particles applied to the ex- angiofibromas
ternal carotid artery may cause severe ischaemic
Angiographic findings n
deficits. If the tumour is in proximity to the optic
nerve, swelling of the tumour as a result of em- Single external carotid artery 11
Joint of external carotid artery and vertebral artery 1
bolization may also lead to visual disturbances. Bilateral external carotid arteries 5
Cranial nerve palsy as well as necrosis of the Ipsilateral internal and external carotid arteries 3
skin and soft tissues have been reported as rare Bilateral external carotid arteries and one internal carotid 7
complications in angiofibroma embolization (11, artery
21). Postembolization fever, local pain and brady- Bilateral external carotid arteries, one internal carotid 1
artery and vertebral artery
cardia have been noted as minor side effects (11).

591
 B. SCHICK AND G. KAHLE
Fig. 17. Angiographic findings before (a) and after (b) super-
selective embolization with polyvinyl alcohol particles of an
angiofibroma fed from the maxillary artery.
Fig. 18. (a) Axial MR image of mucosal swelling at the confines
of the maxillary sinus three months after endonasal angio-
fibroma resection. (b) Axial MR imaging two years after
surgery indicates no tumour recurrence but a thin mucosal
cover of the maxillary sinus.
bolization may also be useful in situations where
the external carotid artery has already been ligated
during previous surgical treatment.
Cell saver and autologous blood banking
To achieve tumour resection without need for
homologous blood transfusion, the intra-operative
use of the cell saver system (4) and pre-operative
autologous blood donation (POAD) are important
supportive measures (5). After blood filtration, the
cell saver system allows for immediate transfusion
of blood lossed intra-operatively. We have used the
cell saver since 1989 in surgical treatment of 20
angiofibromas with an average autotranfusion vol-
ume of 3000 ml of collected blood. POAD should
be offered to every patient. Since 1995, 10 patients
opted for POAD. We were able to avoid homo-
logous blood transfusion in 20 out of 35 surgically
treated angiofibromas by use of embolization, cell
saver and autologous blood banking.
592
Postoperative follow-up
The effectiveness of surgical therapy is analysed by
endoscopic and radiological follow-up. Careful fol-
low-up is of special importance as recurrence has
been reported in up to 25% of the cases (3). MR
imaging is indicated as the first choice modality
for radiological follow-up. It offers the best soft
tissue definition and it also avoids irradiation of
young patients. To document the bony changes
after surgery, we performed an additional baseline
CT examination 3 months after surgery. In those
paranasal sinuses involved at surgery, mucosal
swelling was found in the postoperative course
(Fig. 18a). During the wound healing process,
granulation tissue shows contrast enhancement at
MR imaging. Differentiation between granulation
tissue and residual tumour may be difficult at the
first control examination. During further follow-
up, the question of residual tumour or reparative
changes is answered by comparison with earlier in-
vestigations (Fig. 18b). While stable or decreased
enhancement indicate fibrosis, increased enhance-
ment indicates tumour recurrence.
Conclusion
Angiofibroma presents as a rare tumour with a
predictable growth pattern and typical radiological
findings. The knowledge of the tumour behaviour
is of major interest to both the radiologist and the
surgeon. With classical radiological findings, a pre-
operative biopsy is usually not necessary nor ad-
visable. Advances in accurate determination of the
tumour extension by high-resolution CT and MR
imaging improve surgical planning and reduce the
risk of recurrence. In combination with angiogra-
phic evaluation of the tumour blood supply, the
least traumatic approach for complete resection
can be choosen. The aim of reducing intra-operat-
ive blood loss is achieved by pre-operative em-
bolization, the cell saver system and PAOD.
ACKNOWLEDGEMENT
We dedicate this article to Professor Dr. W. Draf on the oc-
casion of his 60th birthday.
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