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Available online at www.sciencedirect.com

www.elsevier.com/locate/amjoto

Nasopharyngeal angiofibroma: A concise classification system

and appropriate treatment options
Zixiang Yi, MDa,, Zheming Fang, MDb , Gongbiao Lin, MDa , Chang Lin, MDa ,
Wenhui Xiao, MDa , Zhichun Li, MDa , Jinmei Cheng, MDa , Aidong Zhou, MDa
a

Department of Otolaryngology, First Affiliated Hospital of Fujian Medical University, Fuzhou, China
Department of Imaging, First Affiliated Hospital of Fujian Medical University, Fuzhou, China

ARTI CLE I NFO

A BS TRACT

Article history:

Objectives: To describe a clear and simplified classification system for juvenile

Received 28 August 2012

nasopharyngeal angiofibroma (JNA), and to describe suitable management options.

Study design: Retrospective medical record review.
Methods: The clinical and imaging materials of 51 cases of JNA diagnosed at our hospital
between 1981 and 2011 were collected and studied. Based on our experiences, we prefer to
divide JNAs into three types. Type I includes JNAs fundamentally localized to the nasal
cavity, paranasal sinus, nasopharynx, or pterygopalatine fossa. Type II is a JNA extending
into the infratemporal fossa, cheek region, or orbital cavity, with anterior and/or minimal
middle cranial fossa extension but intact dura mater. Type III is a calabash-like massive
tumor lobe in the middle cranial fossa. The management and prognosis for the three types
of JNA were compared and evaluated.
Results: Among cases of type I JNA (n = 16), the entire mass was removed by the initial
operation in 15 cases and by a repeat operation in 1 case. Among cases of type II JNA (n = 29),
the entire mass was removed by the first operation in 24 cases and by repeat operation in 5
cases. In cases of type III JNA (n = 6), the huge calabash-like lobe in the middle cranial fossa
could not be completely excised; 4 cases underwent radiotherapy and 2 cases were lost to
follow-up.
Conclusions: 1) The transnasal cavity approach with endoscopic guidance is suitable for
type I JNA resection. 2) The transantralinfratemporal fossanasal cavity combined
approach is reliable for resection of a type II JNA, which extends into the deep anterior
cranial fossa and/or minimally into the middle cranial fossa, with intact dura mater. 3) The
complete removal of a type III JNA is difficult, even through a combined extracranial and
intracranial approach. Radiotherapy is useful for treating the residual intracranial tumor.
The successful or failed experiences of 6 typical cases prove that this revised classification
system is reasonable and reliable.
2013 Elsevier Inc. All rights reserved.

1.

Introduction

Juvenile nasopharyngeal angiofibroma (JNA) occurs primarily

in adolescent males and accounts for 0.05% of all head and
Corresponding author.
E-mail address: yizixiang@sina.com (Z. Yi).
0196-0709/$ see front matter 2013 Elsevier Inc. All rights reserved.
http://dx.doi.org/10.1016/j.amjoto.2012.10.004

neck neoplasms. Sessions et al [1], Fisch [2], Chandler et al [3],

Radkowski et al [4], and Onerci et al [5] have proposed staging
or classification systems for JNA. Previously, we used Fisch's
system to study the diagnosis and management of JNAs [6].

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Table 1 Staging or typing systems for juvenile nasopharyngeal angiofibroma.

Type or
stage
1

II

Fisch 1983

Chandler
et al. 1984

Limited to
nasopharynx
and nasal
cavity

Tumor confined
to NV

PMF, maxillary,
ethmoidal, and
sphenoidal
sinuses

Tumor ext. into

nasal cavity or
sphenoidal
sinus

Sessions
et al. 1981
IA

III

ITF, orbit and

parasellar region
remaining
lateral
to cavernous
sinus

IV

Cavernous sinus, Intracranial

optic chiasm, or tumor
pituitary fossa
region

Antrum,
ethmoidal
sinus, PMF, ITF,
orbit, and/or
cheek

Onerci
et al. 2006

Same as in
Sessions et al.
Same as in
Sessions et al.

Nose, NV, sinus,

or minimal ext.
to PMF

IIA Minimal ext.

into PMF, full PMF
IIB PMF with or
without erosion
of orbital bones
IIc ITF with or
without cheek

Same as in
Sessions et al.
Same as in
Sessions et al.

Maxillary sinus,
full occupation
of PMF, ext. to
anterior cranial
fossa, and limited
ext. to ITF

III

IIIA Erosion of
skull base,
minimal
intracranial
IIIB Extensive
intracranial
with or without
cavernous
sinus

IB

Limited to nose
and/or NV
One or more
sinuses

Radkowski
et al. 1996

Intracranial
extension

Or posterior to
pterygoid plates

Revised

Deep. ext. into

cancellous bone at
base of pterygoid
or body and GWS,
significant lateral
ext. to ITF or to
pterygoid plates
posteriorly or
orbital region,
cavernous sinus
obliteration
Intracranial ext.
between pituitary
gland and ICA, tumor
localization lateral
to ICA, middle fossa
ext., and extensive
intracranial ext.

Localized in nasal
cavity, nasopharynx,
sinus, PMF. Minimal
extension in ITF, orbit,
or cranial fossa.
Localized in ITF, cheek,
deep or minimal ACF
extension, minimal
MCF extension. With or
without cavernous
sinus
and ICA compression,
but dura mater intact
From PMF and superior
orbital fissure extending
into MCF as a large
gourd-shaped lobe.

NV, nasopharyngeal vault; PMF, pterygomaxillary fossa; ITF, infratemporal fossa; ICA, internal carotid artery; GWS, greater wing of the
sphenoid; ext., extension.

However, we feel that defining some JNAs using the previously

proposed typing and staging protocols can be difficult. Based
on our retrospective research of 51 cases, we prefer to classify
JNAs into three types, simplifying clinical treatment. Table 1
contains our classification schemes for comparing with other
classification schemes in the literatures. The Table 2 is used for

introducing our treatment recommendation and the prognosis of our patients. Meanwhile, we present six cases here
to characterize this problem and its solution sufficiently.
Type I tumors were removed by endoscopy via the nasal
cavity approach, type II JNAs by a transantralinfratemporal
fossanasal cavity combined approach via an extended

Table 2 Revised classification of 51 cases of nasopharyngeal angiofibroma, surgical approach, blood loss, results, and
follow-up.
Type
I

II

III

Tumor size

Case

Surgical
approach

Localized in nasal cavity, nasopharynx,

sinus, PMF. Minimal extension in ITF,
orbit, or cranial fossa.
Localized in ITF, cheek, deep or minimal
ACF extension, minimal MCF extension.
With or without cavernous sinus and
ICA compression, but dura mater intact
From PMF and superior orbital fissure
extending into MCF as a large
gourd-shaped lobe.

16

Transnasal cavity
approach

29

Transantral
infratemporal
fossanasal
cavity approach
EC & IC approach

Blood
loss (ml)

Median blood
loss (ml)

Result

Follow-up

201500

437

117 years

1008000

1893

Removed by first
(n = 15) or repeated
(n = 1) operation
Removed by first
(n = 24) or repeated
(n = 5) operation

15005200

1975

Residual tumor in
MCF (n = 6)

125 years

524 years

PMF, pterygomaxillary fossa; ITF, infratemporal fossa; MCF, middle cranial fossa; ACF, anterior cranial fossa; ICA, internal carotid artery; EC & IC
approach, combined extracranial and intracranial approach.

AM ERI CAN JOUR NAL OF OTOLARY NG OLOG Y H EA D A N D N EC K ME D IC IN E AN D S U RG ER Y 34 ( 20 1 3 ) 1 3 31 4 1

CaldwellLuc incision, and type III JNAs by a combined

intracranial and extracranial approach. However, open
craniotomy for resection of a calabash-like huge tumor lobe
in the middle cranial fossa remains difficult. Complementary
radiotherapy is indicated for intracranial tumor remnants in
such cases.

2.

Materials, methods, and results

Between 1981 and 2011, 51 patients with JNA, aged 844

(mean, 22.3) years, were treated at the First Affiliated
Hospital of Fujian Medical University. Sixteen type I JNAs
were removed using the nasal cavity approach (under
endoscopic guidance in 12 cases). Twenty-nine type II lesions
were accessed via the transantralinfratemporal fossanasal
cavity combined approach; a CaldwellLuc incision was used
in 24 cases, and methods such as lateral rhinotomy or
Weber's incision were used in 5 cases. Six type III masses
were treated via the transantralinfratemporal fossanasal
cavity combined approach for the extracranial portion, and
via the temporal epidural approach (pterional craniotomy)
for the intracranial portion. However, the intracranial portions of these type III tumors could not be removed
completely, and 4 patients underwent radiotherapy (~ 40 Gy)
with a good prognosis. Two patients were hospitalized before
1997. At that time, digital subtraction angiography (DSA)
technology was not available in our hospital, and serious
intraoperative blood loss resulted in the presence of residual
tumor; these two patients were lost to follow-up. With the
approval of the Institutional Review Board, 51 JNAs are
described herein and 6 typical cases of successes or failures
are reported.

2.1.

Perioperative management

1. Imaging examinations were performed to evaluate tumor

size, arterial supply, and venous drainage.
Since 1996, computed tomography angiography (CTA) with
three-dimensional reconstruction, magnetic resonance
imaging (MRI), DSA, and other tests have been used to
evaluate tumor size, arterial supply, and venous drainage.
Two or three days after DSA-guided embolization of the
external carotid artery (ECA) tumor blood supply, suitable
surgical management was performed.
2. Ophthalmic examinations (routine visual examinations
and Schirmer's tear tests) were performed to evaluate
visual function.
3. Surgical cavities were filled as necessary with iodoform or
Vaseline gauze, NasoPore, or absorbable hemostatic gauze,
or biological glue spray was used.

2.2.

Surgical approach

Endoscopic nasal cavity approach for resection of type I JNA

1. Under endoscopy, the tumor in the nasal cavity, nasopharynx, sinus, or pterygomaxillary fossa was removed via
a trans-nasal cavity approach, provided it did not extend
into the deep intracranial and infratemporal fossa.

135

2. A type II JNA can be removed using a transantral

infratemporal fossanasal cavity combined approach via a
CaldwellLuc incision. Prior to 1986, we used this approach
with different incisions, such as lateral rhinotomy or
Weber's incision. To preserve the patient's physical
appearance, we have preferred to use an extended
CaldwellLuc incision (midfacial degloving) for this combined approach since 1986. Previously, we defined this
approach as a transantral approach via a midfacial
degloving incision [7,8], but it can be defined more
precisely as a transantralinfratemporal fossanasal cavity
combined approach via an extended CaldwellLuc incision
[6]. The length of the CaldwellLuc incision is determined
by the size of the tumor. The transantral approach offers
the best safe and minimally invasive access to the
infratemporal fossa, temporal fossa, and cheek region;
the transnasal cavity approach is the best way to access
the perinasal region, including the anterior and middle
cranial fossae. This approach can be used to directly
visualize and remove a type II JNA, allowing careful
sectioning of the tumor to prevent serious bleeding and
complications. The surgery can be divided into two main
steps: 1) use of the transantralinfratemporal fossa approach to strip the tumor from the maxillary sinus,
infratemporal and temporal fossae, and cheek region; and
2) use of the nasal cavity approach under endoscopic
guidance to strip the tumor from the nasal cavity,
nasopharynx, orbit, and sphenoidal sinus. Generally, the
tumor lobe in the infratemporal fossa, cheek, and temporal
fossa is a laterally expanding lesion extending from the
pterygomaxillary fissure. Hence, an isthmus can be observed at this site by imaging and intraoperative visualization. If this huge lateral lobe impedes the surgical field,
the isthmus must be tightly sutured and ligated before
removal to gain a wider operative field. Afterward,
endoscopy via the maxillary sinus and nasal cavity can be
used to aid the removal of tumor portions localized to the
pterygomaxillary fossa, nasopharynx, sphenoidal sinus,
orbital cavity, and anterior cranial fossa. If the optic nerve,
optic chiasm, and cavernous sinus are compressed and
displaced but the dura mater is intact, the tumor can still
be removed completely [8].
3. A tumor with broad intracranial extension can be
removed using a combined intracranial and extracranial
approach: the extracranial portion can be accessed via
the transantralinfratemporal fossanasal cavity combined approach, and the intracranial portion via a
pterional approach.
In 8/51 cases, the tumor extended widely into the cranial
fossa. The epidural pterional approach was used to explore
the intracranial lobe, but no dura mater perforation was
found. Two cases of type II JNA were accessed initially via the
epidural pterional approach, but ultimately required the use
of the transantralinfratemporal fossanasal cavity combined
approach for complete removal. Six cases with huge gourdshaped lobes extending into the middle cranial fossa could
not be completely removed. Two cases were lost to follow-up,
and four cases had intracranial tumor remnants that were
treated successfully with radiotherapy.

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2.3.
IIII

Fig. 1 Case 1. (A) Axial computed tomographic (CT) image

showing a juvenile nasopharyngeal angiofibroma (JNA)
occupying the left nasal cavity. (B) Sagittal view showing the
JNA spreading into the nasopharynx. (C) Three-dimensional
reconstruction of CT angiography indicating that branches of
the bilateral external carotid arteries supplied the tumor. (D)
The totally removed tumor measured 5.5 3.5 3 cm.

Typical case reports: experiences and lesions of types

Case 1, type I: a JNA was completely removed by an

endoscopic transnasal cavity approach.
A 15-year-old boy was hospitalized because of a tumor
causing bilateral nasal obstruction. CTA showed that the
primary tumor was a spherical lesion in the outer wall of the
left nasal cavity, extending into the nasopharynx (Fig. 1).
Three days after DSA-guided embolism of the branches of the
bilateral ECAs supplying the mass, the tumor (5.5 3.5 3 cm)
was removed completely through a transnasal cavity approach under endoscopic guidance, causing the loss of about
100 ml blood. Follow-up more than 1.5 years the patient was
in good health.
Case 2, type II: a JNA extending deeply into the
infratemporal fossa with minimal erosion of the middle
cranial base was completely removed using a transantral
infratemporal fossanasal cavity combined approach.
A 17-year-old boy suffered from right nasal obstruction
and intermittent epistaxis for 3 years, and was hospitalized in
July 2008. The right nasal cavity contained a reddish tumor.
CT imaging demonstrated a tumor occupying the nasal cavity,
nasopharynx, pterygomaxillary fossa, and infratemporal
fossa. The medial and posterior walls of the right maxillary
sinus, as well as the right pterygoid process, were partially
damaged (Fig. 2). Three days after DSA-guided embolism of

Fig. 2 Case 2. (A) Endoscopic view of the tumor in the nasal cavity. (B) The tumor in the nasal cavity and infratemporal fossa,
with an isthmus at the pterygomaxillary fissure (red line); the skull base and sphenoidal sinus were minimally eroded. (C) The
posterior antral wall was pushed forward. (D) Tumor removal through an extended CaldwellLuc incision. (E) Completely
excised tumor. (F) Postoperative axial computed tomographic image.

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the bilateral ECAs, the tumor was removed using a transantral

infratemporal fossanasal cavity combined approach via an
extended CaldwellLuc incision. After the anterior wall of the
maxillary sinus was partially excised, a semicircular prominence was revealed on the posterior wall. The mucosa and bone
were stripped, revealing the smooth surface of the tumor in the
infratemporal fossa. Partial destruction of the greater wing of
the sphenoid and the superior orbital fissure were discovered
intraoperatively. The tumor pedicle was located in the region of
the pterygopalatine fossa and the sphenopalatine foramen. Due
to the restriction of tumor growth by the pterygomaxillary
fissure, an obvious isthmus had formed. Under direct vision and
careful separation, the 5 4 3-cm tumor was removed
completely. Intraoperative blood loss was about 1400 ml. Four
years later, the patient was in good condition.
Case 3, type II: initial surgical resection provided easier
resection of the tumor from peripheral tissue than would
repeated surgical resection. Although this primary tumor was
very large, the intraoperative blood loss was only 100 ml.
An 8.5-year-old boy suffered from repeated left nasal
bleeding for 5 years, with obstructive sleep apnea/hypopnea
syndrome (OSAHS). He was hospitalized on August 3, 2011.
Physical examination revealed slight swelling on the left side
of the face, and a red tumor with a smooth surface occupying
the left nasal cavity and spreading into the nasopharynx. CTA
showed a huge tumor in the infratemporal fossa, cheek
region, pterygomaxillary fossa, nasal cavity, and nasopharynx, with slight involvement of the orbital apex and middle
cranial fossa (Fig. 3). Two days after DSA-guided embolization
of the ECA branches supplying the tumor, a transantral

137

infratemporal fossanasal cavity combined approach via an

extended CaldwellLuc incision was used to remove it. First,
the tumor lobe in the infratemporal fossa and cheek region
was removed, and then endoscopy was used to guide removal
of the tumor lobe involving the pterygomaxillary fossa, nasal
cavity, and sphenoidal sinus. Intraoperative observation
identified the tumor pedicle in the sphenopalatine foramen
and pterygopalatine fossa. Given the lack of scar tissue from a
previous operation, complete tumor resection was easily
accomplished within 2.5 hours. The intraoperative blood loss
was about 100 ml. The OSAHS disappeared after surgery. Fig. 3
shows the completely excised tumor, which weighed 32.8 g.
Follow-up more than one year, the boy was in good health.
Case 4, type II: a tumor extending widely into the anterior
and middle cranial fossae, but leaving the dura mater intact,
was removed completely with limited blood loss.
A 16-year-old boy suffered from right-sided relapsing
epistaxis and progressive nasal obstruction for 2 years, with
right-sided severe headache and loss of vision for 2 weeks.
He was hospitalized in April 2005. CT and MRI showed a
huge lobulated tumor in the nasal and nasopharyngeal
region with extensive extrapharyngeal spreading and skull
base erosion (Fig. 4). Three days after embolization of the
tumor-feeding arteries, a transantralinfratemporal fossa
nasal cavity combined approach via an extended Caldwell
Luc incision was used to remove the tumor completely.
Intraoperative observation revealed that the tumor had
extended into the pterygomaxillary and infratemporal fossae, eroded about 2 3 cm of the inner wall of the right orbit,
destroyed much of the sella turcica and clivus, and adhered

Fig. 3 Case 3. (A) a indicates tumor in the infratemporal fossa and cheek region; b indicates tumor in the nasal cavity and
nasopharynx. The red line indicates the pterygomaxillary fissure region. (B) The tumor spread into the cheek and sphenoidal
sinus. (C) Swelling of the left cheek. (D) Postoperative computed tomographic image. (E) Completely removed tumor. (F)
Photograph of the patient 1 year after the operation.

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Fig. 4 Case 4. (A) Magnetic resonance image (MRI) showing a huge tumor occupying the right nasal cavity, pterygopalatine
fossa, and infratemporal fossa. (B) The tumor extended deeply into the anterior cranial fossa; although the dura mater was
intact, the clivus was destroyed and the optic chiasm and pituitary gland were displaced upward. (C) Computed tomographic
image showing the tumor deep in the cranial fossa, compressing the cavernous sinus and extending into the middle cranial
fossa through the oval foramen. (D and E) Postoperative MRIs showing that the tumor was completely removed. (F) The
lobulated tumor measured 6 7 8 cm.

to about 3 3 cm of the intact dura mater. The multilobulated

tumor measured 6 7 8 cm. Postoperative MRI showed no
tumor remnant. Seven years later, the patient was healthy
and attending university.
Case 5, type II: excision of a recurrent tumor resulted in
more intraoperative blood loss compared with primary tumor
removal due to the difficulty of separating the tumor from
peripheral scar tissue and blood vessels.
An 18-year-old male with a JNA underwent one operation
elsewhere and two in our hospital. CT showed that the tumor
involved the infratemporal fossa, orbital apex, and middle
cranial fossa. Due to scar tissue formation resulting from the
previous operation, the pterygoid venous plexus and the vein in
the pterygomaxillary fossa and pterygoid process region were
congested and tortuous (Fig. 5). DSA showed that small
branches of the cavernous segment of the internal carotid
artery (ICA) and the ophthalmic artery supplied blood to the
tumor. In August 2010, a transantralinfratemporal fossanasal
cavity combined approach via an extended CaldwellLuc
incision was used to remove the tumor. Intraoperative observation identified the tumor pedicle in the pterygopalatine fossa
area. The pedicle was difficult to separate from the surrounding
tissue, and efforts to do so resulted in significant hemorrhage
from the tortuous engorged veins in the pterygopalatine fossa
region, could not be stopped by bipolar coagulation or
hemostatic electrocautery. The total blood loss was 5000 ml.
The surgery was suspended. Two weeks later, during the
iodoform and Vaseline gauze dressing over the surgical cavity

was pulling out, the wound bleeding profusely again. The

patient was discharged with residual tumor tissue. In August
2011, he was rehospitalized, and imaging showed that the
tumor in the infratemporal fossa had narrowed since 2010, but
appeared to have spread into the nasal cavity and middle
cranial fossa. As was the case 1 year previously, the pterygoid
venous plexus and the vein in the pterygomaxillary fossa and
pterygoid process region were congested and tortuous. Moreover, small accessory branches of the cavernous segment of the
ICA supplied the tumor. Using a transantralinfratemporal
fossanasal cavity combined approach via an extended CaldwellLuc incision, reoperation was attempted. During separation of the tumor from the infratemporal and pterygomaxillary
fossae, a large amount of blood was lost due to vascular
adhesion. On separation of the tumor lobe from the middle
cranial fossa, rupture of the small branches of the ICA caused
significant hemorrhage. The total blood loss was about 5000 ml.
Postoperative CTA showed that the tumor had been totally
removed. One year later, the patient was in good condition.
Case 6, type III: a recurrent tumor with wide extension into
the middle cranial fossa was difficult to fully excise.
A 13-year-old boy had undergone removal of a JNA via a
transpalatal approach at another hospital 4 years previously.
Due to recurrence of the huge tumor, he was admitted to our
hospital in November 2007. Physical examination revealed
left-sided prominent eye and conjunctival hyperemia, bulging
of the left cheek, and compression and drooping of the soft
palate (Fig. 6). The patient also had OSAHS due to airway

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139

Fig. 5 Case 5. (A, B) Computed tomographic images from August 2010 showing a huge recurrent tumor in the nasal cavity,
nasopharynx, and infratemporal fossa. (D, E) The recurrent tumor 1 year after the failed operation. In A, B, D, and E, P1 indicates
the congested and dilated pterygoid venous plexus and P2 indicates the normal pterygoid venous plexus; the V and red
arrow indicate congested and dilated veins in the pterygopalatine fossa and pterygoid process region. (F) Branch of the middle
cerebral artery supplied the tumor (white arrow). (G, H) The tumor was completely removed, and no congested and dilated vein
was present in the infratemporal fossa, pterygopalatine fossa, and pterygoid process region. (C, I) Preoperative and
postoperative photographs.

obstruction by the mass. CTA showed that the huge recurrent

tumor had a calabash-like lobe extending through the
superior orbital fissure into the middle cranial fossa, and
another lobe extending deeply into the infratemporal fossa
and cheek region. The pterygoid venous plexus was enlarged
and tortuous. DSA showed feeding branches of the tumor
from: 1) the bilateral internal maxillary arteries, 2) the left
middle cerebral artery, 3) transmitting branches from the
cavernous portion of the bilateral ICAs, and 4) the left
ophthalmic artery.
In November 2007, the extracranial part of the tumor was
removed via a transantralinfratemporal fossanasal cavity
combined approach and the intracranial portion was excised
via a pterional approach. The operation lasted 10.5 hours, and
blood loss was 5200 ml. However, the calabash-like lobe in the

middle cranial fossa could not be fully removed, and residual

tumor tissue was left behind. Other complicating factors
included a concurrent epidural hematoma following the
craniotomy as well as an epidural abscess. The patient was
transferred for radiotherapy at 39.6 Gy. Two years later, CTA
showed significant reduction of the residual intracranial
tumor. The patient's left eye returned to the normal position.
He is currently a factory worker.

2.4.

Complications

1. One case of epidural hematoma occurred secondary to

pyogenic epidural abscess.
2. One case of excessive bleeding led to disseminated
intravascular coagulation.

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Fig. 6 Case 6. (A, B) Computed tomographic images of a huge recurrent tumor in the nasal cavity, nasopharynx, infratemporal
fossa cheek region, and middle cranial fossa. P1 indicates the normal pterygoid venous plexus, P2 indicates the congested and
dilated pterygoid venous plexus. (B) A large calabash-like lobe was present in the middle cranial fossa (red arrow). (C) The
middle cerebral artery supplied blood to the tumor (white arrow). (D) Preoperative photograph of the patient. (E) The
extracranial portion of the tumor was completely removed, and no congested and dilated pterygoid venous plexus was present
in the infratemporal fossa. (F, G) Intracranial residue after 2.5 years of radiotherapy (red arrow). (H) Photograph of the patient
after surgery and radiotherapy.

3.

Discussion

The previous classification or staging of JNAs is rather

complicated (Table 1), and the preferred treatment methods
also vary among experts. Bremer et al [9]. reported clinical
data from 150 cases collected over more than 40 years: cases
from 1945 to 1955 were treated with radiotherapy, those
from 1955 to 1971 were treated mainly using lateral
rhinotomy, and JNAs in all cases from 1971 to 1983 were
removed by surgical resection. Fisch [2] proposed the
classification of JNAs into four types. Tumors of type 1 and
II were removed through a transpalatine approach or lateral
rhinotomy, whereas tumors of type III and IV were removed
via the infratemporal fossa approach. Radkowski et al [4].
proposed that stage IIIa and IIIb tumors be removed through
midfacial degloving. Onerci et al [5]. reported 36 cases of
JNAs treated by resection via an external or endonasal
approach. Yi et al [10]. reported the use of a transantral
and nasal cavity combined approach to remove tumors
involving the pterygopalatine fossa, infratemporal fossa,
cheek, sinus, or other intracranial regions. Fisch [2],
Radkowski et al [4],. and Onerci et al [5]. have reported
cases in which the tumor involved the cavernous sinus, optic
chiasm, and pituitary fossa region. Our previous study
showed JNAs to be a vascular hamartoma that does not
infiltrate surrounding tissue, and if it causes deviation of

structures but the dura mater is intact, the tumor can be

removed using a transantral and nasal cavity combined
approach [6,8]. From a neurosurgical perspective and
according to gross anatomy and clinical and feasible
research, Philip et al [11]. stated that the transantral and
nasal cavity approach can be utilized for lesions in the
temporal fossa and in cases of exposure of the mandibular
nerve, middle meningeal artery, and even parts of the ICA
near the skull base. This report explains the important
significance for clinical practice.
According to our experience in treating 51 cases of JNA, we
have found that these tumors can be broadly divided into
three types. Type I tumors should be resected using an
intranasal endoscopic approach. Type II tumors can be
removed by a transantralinfratemporal fossanasal cavity
combined approach via an extended CaldwellLuc incision.
The advantages of this approach are obvious and reliable. For
example, selective removal of the medial, external (facial),
superior (orbital), or posterior (zygomatic or infratemporal)
surface of the antrum provides ideal access to the maxillary
and sphenoidal sinuses, infratemporal fossa, orbit, and cheek.
This approach avoids the piecemeal and blind dissection of
the tumor. Moreover, an extended CaldwellLuc incision can
avoid facial scarring and provides a wider surgical field. Type
III tumors require the use of an intracranial and extracranial
combined approach, and radiotherapy should be initiated if
residual tumor is present in the middle cranial fossa.

AM ERI CAN JOUR NAL OF OTOLARY NG OLOG Y H EA D A N D N EC K ME D IC IN E AN D S U RG ER Y 34 ( 20 1 3 ) 1 3 31 4 1

3.1.

Blood loss and tumor recurrence

According to our imaging findings, most feeding arteries

originate from the internal maxillary artery and the ascending
pharyngeal artery, and occasionally from the middle meningeal artery of the ipsilateral ECA. A large JNA is often supplied
by the contralateral ECA. Tumors that extend widely into the
middle cranial fossa are often supplied by branches of the
cavernous segment of the ICA, ophthalmic artery, and middle
cerebral artery. Because these small arterial branches cannot
be embolized, serious intraoperative blood loss may result in
tumor recurrence. In patients who have undergone an initial
operation, the tumor usually adheres to the surrounding
blood vessels, such as the pterygoid venous plexus or
sphenopalatine vein. The sphenopalatine vein, maxillary
vein, pterygoid venous plexus, and intracranial and extracranial venous branches communicate with each other with no
intervening valve. Thus, the surgeon should ideally obtain
direct visualization of the area and must use care when
separating the tumor pedicle to avoid rupture of the local
veins, inducing profound bleeding.
Cases 14 demonstrate that, regardless of tumor size, a
professional surgical technique can remove tumors of types I
and II. Furthermore, as in case 4, even a tumor with extensive
intracranial spreading can be removed completely if the dura
mater is intact.
In cases 5 and 6, local blood supply and blood drainage
were reduced simultaneously after the extracranial portion of
the tumor was removed from the infratemporal fossa and
pterygomaxillary fossa region. Imaging revealed no further
vein engorgement at the previous site of the lesion.

4.

Conclusions

The classification of JNAs into three types is relatively simple

and clear. The endoscopically guided nasal cavity approach
can used to remove a type I JNA. The transantral
infratemporal fossanasal cavity combined approach via an
extended CaldwellLuc incision is suitable for the removal of a
type II JNA. If the tumor is calabash-like and extends deeply
into the middle cranial fossa, full removal can be difficult, and
any intracranial tumor remnants should be treated with 40-

141

Gy radiotherapy [12]. Regardless of tumor size, after DSAguided embolization of ECA blood-supplying branches, the
operation should be performed under direct visualization, the
tumor should be carefully resected, and bleeding of the ICA
and the pedicle vein plays a crucial role in determining
surgical success and avoiding recurrence.

REFERENCES

[1] Sessions RB, Bryan RN, Nacierin RM, et al. Radiographic

staging of juvenile angiofibroma. Head Neck Surg 1981;3:
27983.
[2] Fisch UGO. The infratemporal fossa approach for nasopharyngeal tumors. Laryngoscope 1983;93:3644.
[3] Chandler JR, Goulding R, Moskowitz L, et al. Nasopharyngeal
angiofibroma: staging and management. Ann Otol Rhinol
Laryngol 1984;93:3229.
[4] Radkowski D, McGill T, Healy GB, et al. Angiofibroma.
Changes in staging and treatment. Arch Otolaryngol Head
Neck Surg 1996;122:1229.
[5] Onerci M, Oretmenolu O, Ycel T. Juvenile nasopharyngeal
angiofibroma: a revised staging system. Rhinology 2006;44:
3945.
[6] Lin C, Li Z-C, Cheng J-M, et al. Pathological features and
clinical managements of nasopharyngeal angiofibroma. Chin
J Otorhinolaryngeal Head Neck Surg 2008;43:7636.
[7] Yi Z, Li ZC, Lin C, et al. Huge lobulated juvenile angiofibroma:
sites of extension, selection of procedures for management.
Journal Otolaryngol 2003;32:2116.
[8] Yi ZX, Li ZC, Cheng JM, et al. Huge nasopharyngeal
angiofibroma with intracranial extension: change in the dura
mater and choice of surgical management. J Laryngol Otol
2007;121:110812.
[9] Bremer JW, Neel III HB, DeSanto LW, et al. Angiofibroma:
treatment trends in 150 patients during 40 years. Laryngoscope 1986;96:13219.
[10] Yi ZX, Yuan QG, Yang YG, et al. The surgical management of
nasopharyngeal angiofibroma invading the pterygopalatine
fossa infratemporal fossa and cheek. Chinese J Otorhinolog
1985;20:11223.
[11] Philip V, Guthikonda B, Brescia A, et al. Endoscopic approach
to the infratemporal fossa: anatomic study. Neurosurgery
2010;66:196202.
[12] Yi ZX, Huang JM, Lin C, et al. Radiation treatment of
nasopharyngeal angiofibroma postoperative residual. Chin J
Otorhinolaryngol Head Neck Surgery 2010;45:4278.