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


Radkowski et al [4], and Onerci et al [5] have proposed staging
Juvenile nasopharyngeal angiofibroma (JNA) occurs primarily or classification systems for JNA. Previously, we used Fisch's
in adolescent males and accounts for 0.05% of all head and system to study the diagnosis and management of JNAs [6].

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
134 AM ER IC AN JOUR NA L OF OTOLARYNGO LOGY 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

Table 1 Staging or typing systems for juvenile nasopharyngeal angiofibroma.


Type or Fisch 1983 Chandler Sessions Radkowski Onerci Revised
stage et al. 1984 et al. 1981 et al. 1996 et al. 2006

1 Limited to Tumor confined IA Limited to nose Same as in Nose, NV, sinus, Localized in nasal
nasopharynx to NV and/or NV Sessions et al. or minimal ext. cavity, nasopharynx,
and nasal IB One or more Same as in to PMF sinus, PMF. Minimal
cavity sinuses Sessions et al. extension in ITF, orbit,
or cranial fossa.
II PMF, maxillary, Tumor ext. into IIA Minimal ext. Same as in Maxillary sinus, Localized in ITF, cheek,
ethmoidal, and nasal cavity or into PMF, full PMF Sessions et al. full occupation deep or minimal ACF
sphenoidal sphenoidal IIB PMF with or Same as in of PMF, ext. to extension, minimal
sinuses sinus without erosion Sessions et al. anterior cranial MCF extension. With or
of orbital bones fossa, and limited without cavernous
IIc ITF with or Or posterior to ext. to ITF sinus
without cheek pterygoid plates and ICA compression,
but dura mater intact
III ITF, orbit and Antrum, III Intracranial IIIA Erosion of Deep. ext. into From PMF and superior
parasellar region ethmoidal extension skull base, cancellous bone at orbital fissure extending
remaining sinus, PMF, ITF, minimal base of pterygoid into MCF as a large
lateral orbit, and/or intracranial or body and GWS, gourd-shaped lobe.
to cavernous cheek IIIB Extensive significant lateral
sinus intracranial ext. to ITF or to
with or without pterygoid plates
cavernous posteriorly or
sinus orbital region,
cavernous sinus
obliteration
IV Cavernous sinus, Intracranial Intracranial ext.
optic chiasm, or tumor between pituitary
pituitary fossa gland and ICA, tumor
region localization lateral
to ICA, middle fossa
ext., and extensive
intracranial ext.

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 introducing our treatment recommendation and the prog-
proposed typing and staging protocols can be difficult. Based nosis of our patients. Meanwhile, we present six cases here
on our retrospective research of 51 cases, we prefer to classify to characterize this problem and its solution sufficiently.
JNAs into three types, simplifying clinical treatment. Table 1 Type I tumors were removed by endoscopy via the nasal
contains our classification schemes for comparing with other cavity approach, type II JNAs by a transantralinfratemporal
classification schemes in the literatures. The Table 2 is used for 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 Tumor size Case Surgical Blood Median blood Result Follow-up
approach loss (ml) loss (ml)

I Localized in nasal cavity, nasopharynx, 16 Transnasal cavity 201500 437 Removed by first 117 years
sinus, PMF. Minimal extension in ITF, approach (n = 15) or repeated
orbit, or cranial fossa. (n = 1) operation
II Localized in ITF, cheek, deep or minimal 29 Transantral 1008000 1893 Removed by first 125 years
ACF extension, minimal MCF extension. infratemporal (n = 24) or repeated
With or without cavernous sinus and fossanasal (n = 5) operation
ICA compression, but dura mater intact cavity approach
III From PMF and superior orbital fissure 6 EC & IC approach 15005200 1975 Residual tumor in 524 years
extending into MCF as a large MCF (n = 6)
gourd-shaped lobe.

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.
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CaldwellLuc incision, and type III JNAs by a combined 2. A type II JNA can be removed using a transantral
intracranial and extracranial approach. However, open infratemporal fossanasal cavity combined approach via a
craniotomy for resection of a calabash-like huge tumor lobe CaldwellLuc incision. Prior to 1986, we used this approach
in the middle cranial fossa remains difficult. Complementary with different incisions, such as lateral rhinotomy or
radiotherapy is indicated for intracranial tumor remnants in Weber's incision. To preserve the patient's physical
such cases. appearance, we have preferred to use an extended
CaldwellLuc incision (midfacial degloving) for this com-
bined approach since 1986. Previously, we defined this
2. Materials, methods, and results approach as a transantral approach via a midfacial
degloving incision [7,8], but it can be defined more
Between 1981 and 2011, 51 patients with JNA, aged 844 precisely as a transantralinfratemporal fossanasal cavity
(mean, 22.3) years, were treated at the First Affiliated combined approach via an extended CaldwellLuc incision
Hospital of Fujian Medical University. Sixteen type I JNAs [6]. The length of the CaldwellLuc incision is determined
were removed using the nasal cavity approach (under by the size of the tumor. The transantral approach offers
endoscopic guidance in 12 cases). Twenty-nine type II lesions the best safe and minimally invasive access to the
were accessed via the transantralinfratemporal fossanasal infratemporal fossa, temporal fossa, and cheek region;
cavity combined approach; a CaldwellLuc incision was used the transnasal cavity approach is the best way to access
in 24 cases, and methods such as lateral rhinotomy or the perinasal region, including the anterior and middle
Weber's incision were used in 5 cases. Six type III masses cranial fossae. This approach can be used to directly
were treated via the transantralinfratemporal fossanasal visualize and remove a type II JNA, allowing careful
cavity combined approach for the extracranial portion, and sectioning of the tumor to prevent serious bleeding and
via the temporal epidural approach (pterional craniotomy) complications. The surgery can be divided into two main
for the intracranial portion. However, the intracranial por- steps: 1) use of the transantralinfratemporal fossa ap-
tions of these type III tumors could not be removed proach to strip the tumor from the maxillary sinus,
completely, and 4 patients underwent radiotherapy (~ 40 Gy) infratemporal and temporal fossae, and cheek region; and
with a good prognosis. Two patients were hospitalized before 2) use of the nasal cavity approach under endoscopic
1997. At that time, digital subtraction angiography (DSA) guidance to strip the tumor from the nasal cavity,
technology was not available in our hospital, and serious nasopharynx, orbit, and sphenoidal sinus. Generally, the
intraoperative blood loss resulted in the presence of residual tumor lobe in the infratemporal fossa, cheek, and temporal
tumor; these two patients were lost to follow-up. With the fossa is a laterally expanding lesion extending from the
approval of the Institutional Review Board, 51 JNAs are pterygomaxillary fissure. Hence, an isthmus can be ob-
described herein and 6 typical cases of successes or failures served at this site by imaging and intraoperative visuali-
are reported. zation. If this huge lateral lobe impedes the surgical field,
the isthmus must be tightly sutured and ligated before
2.1. Perioperative management removal to gain a wider operative field. Afterward,
endoscopy via the maxillary sinus and nasal cavity can be
1. Imaging examinations were performed to evaluate tumor used to aid the removal of tumor portions localized to the
size, arterial supply, and venous drainage. pterygomaxillary fossa, nasopharynx, sphenoidal sinus,
Since 1996, computed tomography angiography (CTA) with orbital cavity, and anterior cranial fossa. If the optic nerve,
three-dimensional reconstruction, magnetic resonance optic chiasm, and cavernous sinus are compressed and
imaging (MRI), DSA, and other tests have been used to displaced but the dura mater is intact, the tumor can still
evaluate tumor size, arterial supply, and venous drainage. be removed completely [8].
Two or three days after DSA-guided embolization of the 3. A tumor with broad intracranial extension can be
external carotid artery (ECA) tumor blood supply, suitable removed using a combined intracranial and extracranial
surgical management was performed. approach: the extracranial portion can be accessed via
2. Ophthalmic examinations (routine visual examinations the transantralinfratemporal fossanasal cavity com-
and Schirmer's tear tests) were performed to evaluate bined approach, and the intracranial portion via a
visual function. pterional approach.
3. Surgical cavities were filled as necessary with iodoform or
Vaseline gauze, NasoPore, or absorbable hemostatic gauze, In 8/51 cases, the tumor extended widely into the cranial
or biological glue spray was used. fossa. The epidural pterional approach was used to explore
the intracranial lobe, but no dura mater perforation was
2.2. Surgical approach found. Two cases of type II JNA were accessed initially via the
epidural pterional approach, but ultimately required the use
Endoscopic nasal cavity approach for resection of type I JNA of the transantralinfratemporal fossanasal cavity combined
approach for complete removal. Six cases with huge gourd-
1. Under endoscopy, the tumor in the nasal cavity, naso- shaped lobes extending into the middle cranial fossa could
pharynx, sinus, or pterygomaxillary fossa was removed via not be completely removed. Two cases were lost to follow-up,
a trans-nasal cavity approach, provided it did not extend and four cases had intracranial tumor remnants that were
into the deep intracranial and infratemporal fossa. treated successfully with radiotherapy.
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2.3. Typical case reports: experiences and lesions of types


IIII

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 ap-
proach 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.
Fig. 1 Case 1. (A) Axial computed tomographic (CT) image A 17-year-old boy suffered from right nasal obstruction
showing a juvenile nasopharyngeal angiofibroma (JNA) and intermittent epistaxis for 3 years, and was hospitalized in
occupying the left nasal cavity. (B) Sagittal view showing the July 2008. The right nasal cavity contained a reddish tumor.
JNA spreading into the nasopharynx. (C) Three-dimensional CT imaging demonstrated a tumor occupying the nasal cavity,
reconstruction of CT angiography indicating that branches of nasopharynx, pterygomaxillary fossa, and infratemporal
the bilateral external carotid arteries supplied the tumor. (D) fossa. The medial and posterior walls of the right maxillary
The totally removed tumor measured 5.5 3.5 3 cm. 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
infratemporal fossanasal cavity combined approach via an extended CaldwellLuc incision was used to remove it. First,
extended CaldwellLuc incision. After the anterior wall of the the tumor lobe in the infratemporal fossa and cheek region
maxillary sinus was partially excised, a semicircular promi- was removed, and then endoscopy was used to guide removal
nence was revealed on the posterior wall. The mucosa and bone of the tumor lobe involving the pterygomaxillary fossa, nasal
were stripped, revealing the smooth surface of the tumor in the cavity, and sphenoidal sinus. Intraoperative observation
infratemporal fossa. Partial destruction of the greater wing of identified the tumor pedicle in the sphenopalatine foramen
the sphenoid and the superior orbital fissure were discovered and pterygopalatine fossa. Given the lack of scar tissue from a
intraoperatively. The tumor pedicle was located in the region of previous operation, complete tumor resection was easily
the pterygopalatine fossa and the sphenopalatine foramen. Due accomplished within 2.5 hours. The intraoperative blood loss
to the restriction of tumor growth by the pterygomaxillary was about 100 ml. The OSAHS disappeared after surgery. Fig. 3
fissure, an obvious isthmus had formed. Under direct vision and shows the completely excised tumor, which weighed 32.8 g.
careful separation, the 5 4 3-cm tumor was removed Follow-up more than one year, the boy was in good health.
completely. Intraoperative blood loss was about 1400 ml. Four Case 4, type II: a tumor extending widely into the anterior
years later, the patient was in good condition. and middle cranial fossae, but leaving the dura mater intact,
Case 3, type II: initial surgical resection provided easier was removed completely with limited blood loss.
resection of the tumor from peripheral tissue than would A 16-year-old boy suffered from right-sided relapsing
repeated surgical resection. Although this primary tumor was epistaxis and progressive nasal obstruction for 2 years, with
very large, the intraoperative blood loss was only 100 ml. right-sided severe headache and loss of vision for 2 weeks.
An 8.5-year-old boy suffered from repeated left nasal He was hospitalized in April 2005. CT and MRI showed a
bleeding for 5 years, with obstructive sleep apnea/hypopnea huge lobulated tumor in the nasal and nasopharyngeal
syndrome (OSAHS). He was hospitalized on August 3, 2011. region with extensive extrapharyngeal spreading and skull
Physical examination revealed slight swelling on the left side base erosion (Fig. 4). Three days after embolization of the
of the face, and a red tumor with a smooth surface occupying tumor-feeding arteries, a transantralinfratemporal fossa
the left nasal cavity and spreading into the nasopharynx. CTA nasal cavity combined approach via an extended Caldwell
showed a huge tumor in the infratemporal fossa, cheek Luc incision was used to remove the tumor completely.
region, pterygomaxillary fossa, nasal cavity, and nasophar- Intraoperative observation revealed that the tumor had
ynx, with slight involvement of the orbital apex and middle extended into the pterygomaxillary and infratemporal fos-
cranial fossa (Fig. 3). Two days after DSA-guided embolization sae, eroded about 2 3 cm of the inner wall of the right orbit,
of the ECA branches supplying the tumor, a transantral 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 was pulling out, the wound bleeding profusely again. The
tumor measured 6 7 8 cm. Postoperative MRI showed no patient was discharged with residual tumor tissue. In August
tumor remnant. Seven years later, the patient was healthy 2011, he was rehospitalized, and imaging showed that the
and attending university. tumor in the infratemporal fossa had narrowed since 2010, but
Case 5, type II: excision of a recurrent tumor resulted in appeared to have spread into the nasal cavity and middle
more intraoperative blood loss compared with primary tumor cranial fossa. As was the case 1 year previously, the pterygoid
removal due to the difficulty of separating the tumor from venous plexus and the vein in the pterygomaxillary fossa and
peripheral scar tissue and blood vessels. pterygoid process region were congested and tortuous. More-
An 18-year-old male with a JNA underwent one operation over, small accessory branches of the cavernous segment of the
elsewhere and two in our hospital. CT showed that the tumor ICA supplied the tumor. Using a transantralinfratemporal
involved the infratemporal fossa, orbital apex, and middle fossanasal cavity combined approach via an extended Cald-
cranial fossa. Due to scar tissue formation resulting from the wellLuc incision, reoperation was attempted. During separa-
previous operation, the pterygoid venous plexus and the vein in tion of the tumor from the infratemporal and pterygomaxillary
the pterygomaxillary fossa and pterygoid process region were fossae, a large amount of blood was lost due to vascular
congested and tortuous (Fig. 5). DSA showed that small adhesion. On separation of the tumor lobe from the middle
branches of the cavernous segment of the internal carotid cranial fossa, rupture of the small branches of the ICA caused
artery (ICA) and the ophthalmic artery supplied blood to the significant hemorrhage. The total blood loss was about 5000 ml.
tumor. In August 2010, a transantralinfratemporal fossanasal Postoperative CTA showed that the tumor had been totally
cavity combined approach via an extended CaldwellLuc removed. One year later, the patient was in good condition.
incision was used to remove the tumor. Intraoperative obser- Case 6, type III: a recurrent tumor with wide extension into
vation identified the tumor pedicle in the pterygopalatine fossa the middle cranial fossa was difficult to fully excise.
area. The pedicle was difficult to separate from the surrounding A 13-year-old boy had undergone removal of a JNA via a
tissue, and efforts to do so resulted in significant hemorrhage transpalatal approach at another hospital 4 years previously.
from the tortuous engorged veins in the pterygopalatine fossa Due to recurrence of the huge tumor, he was admitted to our
region, could not be stopped by bipolar coagulation or hospital in November 2007. Physical examination revealed
hemostatic electrocautery. The total blood loss was 5000 ml. left-sided prominent eye and conjunctival hyperemia, bulging
The surgery was suspended. Two weeks later, during the of the left cheek, and compression and drooping of the soft
iodoform and Vaseline gauze dressing over the surgical cavity palate (Fig. 6). The patient also had OSAHS due to airway
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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 middle cranial fossa could not be fully removed, and residual
tumor had a calabash-like lobe extending through the tumor tissue was left behind. Other complicating factors
superior orbital fissure into the middle cranial fossa, and included a concurrent epidural hematoma following the
another lobe extending deeply into the infratemporal fossa craniotomy as well as an epidural abscess. The patient was
and cheek region. The pterygoid venous plexus was enlarged transferred for radiotherapy at 39.6 Gy. Two years later, CTA
and tortuous. DSA showed feeding branches of the tumor showed significant reduction of the residual intracranial
from: 1) the bilateral internal maxillary arteries, 2) the left tumor. The patient's left eye returned to the normal position.
middle cerebral artery, 3) transmitting branches from the He is currently a factory worker.
cavernous portion of the bilateral ICAs, and 4) the left
ophthalmic artery. 2.4. Complications
In November 2007, the extracranial part of the tumor was
removed via a transantralinfratemporal fossanasal cavity 1. One case of epidural hematoma occurred secondary to
combined approach and the intracranial portion was excised pyogenic epidural abscess.
via a pterional approach. The operation lasted 10.5 hours, and 2. One case of excessive bleeding led to disseminated
blood loss was 5200 ml. However, the calabash-like lobe in the 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.

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


3. Discussion removed using a transantral and nasal cavity combined
approach [6,8]. From a neurosurgical perspective and
The previous classification or staging of JNAs is rather according to gross anatomy and clinical and feasible
complicated (Table 1), and the preferred treatment methods research, Philip et al [11]. stated that the transantral and
also vary among experts. Bremer et al [9]. reported clinical nasal cavity approach can be utilized for lesions in the
data from 150 cases collected over more than 40 years: cases temporal fossa and in cases of exposure of the mandibular
from 1945 to 1955 were treated with radiotherapy, those nerve, middle meningeal artery, and even parts of the ICA
from 1955 to 1971 were treated mainly using lateral near the skull base. This report explains the important
rhinotomy, and JNAs in all cases from 1971 to 1983 were significance for clinical practice.
removed by surgical resection. Fisch [2] proposed the According to our experience in treating 51 cases of JNA, we
classification of JNAs into four types. Tumors of type 1 and have found that these tumors can be broadly divided into
II were removed through a transpalatine approach or lateral three types. Type I tumors should be resected using an
rhinotomy, whereas tumors of type III and IV were removed intranasal endoscopic approach. Type II tumors can be
via the infratemporal fossa approach. Radkowski et al [4]. removed by a transantralinfratemporal fossanasal cavity
proposed that stage IIIa and IIIb tumors be removed through combined approach via an extended CaldwellLuc incision.
midfacial degloving. Onerci et al [5]. reported 36 cases of The advantages of this approach are obvious and reliable. For
JNAs treated by resection via an external or endonasal example, selective removal of the medial, external (facial),
approach. Yi et al [10]. reported the use of a transantral superior (orbital), or posterior (zygomatic or infratemporal)
and nasal cavity combined approach to remove tumors surface of the antrum provides ideal access to the maxillary
involving the pterygopalatine fossa, infratemporal fossa, and sphenoidal sinuses, infratemporal fossa, orbit, and cheek.
cheek, sinus, or other intracranial regions. Fisch [2], This approach avoids the piecemeal and blind dissection of
Radkowski et al [4],. and Onerci et al [5]. have reported the tumor. Moreover, an extended CaldwellLuc incision can
cases in which the tumor involved the cavernous sinus, optic avoid facial scarring and provides a wider surgical field. Type
chiasm, and pituitary fossa region. Our previous study III tumors require the use of an intracranial and extracranial
showed JNAs to be a vascular hamartoma that does not combined approach, and radiotherapy should be initiated if
infiltrate surrounding tissue, and if it causes deviation of residual tumor is present in the middle cranial fossa.
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3.1. Blood loss and tumor recurrence Gy radiotherapy [12]. Regardless of tumor size, after DSA-
guided embolization of ECA blood-supplying branches, the
According to our imaging findings, most feeding arteries operation should be performed under direct visualization, the
originate from the internal maxillary artery and the ascending tumor should be carefully resected, and bleeding of the ICA
pharyngeal artery, and occasionally from the middle menin- and the pedicle vein plays a crucial role in determining
geal artery of the ipsilateral ECA. A large JNA is often supplied surgical success and avoiding recurrence.
by the contralateral ECA. Tumors that extend widely into the
middle cranial fossa are often supplied by branches of the
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