JNEVI Journal of Neurovascular Intervention Vol 2.No.

1 January 2020

Preoperative Embolization in Juvenile Nasopharyngeal Angiofibroma

Gilbert Tangkudung1, Junita Maja Pertiwi2, Ronaldy E. C. Tumbel3, Hedy Angeline4, Herdy
Johanis4
1
Neurologist and Interventional Neurologist, Neurology Department of Sam Ratulangi
University/Prof.Dr.R.D.Kandou Hospital,Manado, North Sulawesi, Indonesia
2
Neurologist, Neurology Departement of Sam Ratulangi University/ Prof.Dr.R.D.Kandou
Hospital,Manado, North Sulawesi, Indonesia
3
Otorhinolaringology-Head and Neck Surgeon, ENT Department Prof.Dr.R.D.Kandou
Hospital,Manado, North Sulawesi, Indonesia
4
Neurology Resident, Medical Faculty Sam Ratulangi University/Prof.Dr.R.D.Kandou Hospital,
Manado, North Sulawesi, Indonesia

*Correspondence Author
Gilbert Tangkudung
Neurology Department of Sam Ratulangi University/Prof.Dr.R.D.Kandou Hospital,Manado,
Email: gilbert.tangkudung@gmail.com

Abstrak
Juvenile nasopharyngeal angiofibroma (JNA) merupakan kasus jarang (0,05%), jinak dan banyak
vaskularisasi, berasal dari pembentukan massa pada foramen sphenopalatine. Insidens terjadinya
biasa pada usia 7-19 tahun, dan jarang pada usia lebih dari 25 tahun. Embolisasi preoperatif
merupakan suatu teknik endovaskular intervensional neuroradiologi yang sudah terbukti untuk
devaskularisasi preoperatif JNA. Laki-laki, umur 17 tahun dengan keluhan epistaksis berulang pada
hidung kanan sejak 3 bulan. Pasien juga mengalami hidung tersumbat, gangguan penciuman dan
suara sengau. Riwayat trauma tidak ada. Pemeriksaan neurologi ditemukan Anosmia pada kavum
nasi dextra. Pada funduskopi tidak ditemukan kelainan. Pemeriksaan serum darah normal. CT Scan
kepala dengan kontras tampak lesi massa tumor yang hipodens di regio nasofaring dengan
permukaan reguler, batas tidak begitu tegas, dan menyengat kontras. Tampak perluasaan lesi dari
nasofaring ke rongga hidung dan orofaring. Tidak ada destruksi tulang tengkorak atau mengenai
saraf otak, menunjukkan kecurigaan suatu lesi angiofibroma nasofaring. Angiografi serebral
menunjukan tumor blush di cavum nasi yang meluas ke sinus sphenoid yang mendapat suplai tunggal
dari arteri sphenopalatina dextra. Dilakukan embolisasi tumor intra arterial dengan menggunakan
partikel polivinil alkohol (PVA) dengan ukuran 150-250µ sampai tidak tampak tumor blush. Operasi
reseksi tumor dilakukan 4 hari setelah embolisasi dengan teknik endoskopi transnasal. Tumor
berhasil diangkat secara keseluruhan dengan kehilangan darah total yg minimal selama prosedur
(±120cc).
Keywords: juvenile nasopharyngeal angiofibroma,embolization, tumor

Abstract
Juvenile nasopharyngeal angiofibroma (JNA) is a rare (0.05%), the benign tumor with numerous
vascularization, which originates from the mass formation in the sphenopalatine foramen. Incidence
was mostly within age ranging from 7-19 years old, and occurrence after 25 years of age is
considered rare. Preoperative embolization is a proven neuroradiological interventional
endovascular technique for preoperative JNA devascularization.Male, 17 years old, presented with
recurrent epistaxis in his right nose since 3 months. The patient also experienced nasal congestion,
olfactory disorders, and hoarseness. History of facial trauma was denied. Neurological examinations
revealed anosmia in the right nasal cavity. Funduscopy was revealed no abnormalities. Laboratory
examinations findings are normal. Brain CT-Scan contrast was revealed a hypodense mass lesion
within the nasopharyngeal region that has regular surface and the unclear border with contrast
enhancement. The mass extend from nasopharynx area to the nasal cavity and oropharynx. Skull
destruction nor nerve injury were not found, which increased the suspicion of nasopharyngeal
angiofibroma. Cerebral angiography revealed a tumor blush in the nasal cavity which extends to
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JNEVI Journal of Neurovascular Intervention Vol 2.No.1 January 2020

sphenoid sinus and has single vascularization from a right sphenopalatine artery. Intraarterial tumor
embolization was performed using polyvinyl alcohol (PVA) particles with a size of 150-250µ until no
tumor blush were seen. The resection surgery of the tumor was performed 4 days after embolization
with the transnasal endoscopic technique. The tumor was removed completely with minimal total
blood loss during procedur (± 120cc).
Keywords: juvenile nasopharyngeal angiofibroma,embolization, tumor

Introduction found, which increased the suspicion of

Juvenile nasopharyngeal angiofibroma nasopharyngeal angiofibroma.
(JNA) is a rare, benign well-vascularized
tumor. Incidence ranged from 0.05% from
tumors of head and neck. Affected age
ranged from 7-19 years.1 The etiology of
this tumor remained unknown. JNA is a
very well-vascularized tumor that
originates from ascending pharyngeal
artery or internal maxillary artery.2
Preoperative embolization is a
neuroradiological endovascular
interventional technique that has been
proven for preoperative devascularization
JNA.3,4 Purpose of this preoperative
embolization is to occlude or block one or
more blood vessels or vascular supplies
that are abnormal or malformed, which
will result in tumor devascularization.
Bleeding that occurred without
preoperative embolization were reported
about 2000cc. Since preoperative
embolization is recommended for standard
procedure, blood loss during surgery
decreased to less than 1000cc.5
Figure 1. Brain CT Scan non-contrast (A) and
Case Presentation
with contrast (B) pre embolization. Mass
Male, 17 years old, presented with tumor was visible on nasopharynx region with
recurrent epistaxis in his right nose since 3 circular shape, hypodense structure, regular
months. The patient also experienced nasal surface, and unclear border, with enhancement
congestion, olfactory disorders, and after contrast. Expansion of nasopharynx
hoarseness. History of facial trauma was lesion to nasal and oropharynx area were also
denied. Neurological examinations obvious. No bone nor nerve destruction were
revealed anosmia in the right nasal cavity. observed. Lympathic nodes were not clear
Funduscopy was revealed no wheter there is any regional enlargement.
abnormalities. Laboratory examinations
findings are normal. Brain CT-Scan
contrast was revealed a hypodense mass
lesion within the nasopharyngeal region
that has regular surface and the unclear
border with contrast enhancement (figure
1). The mass extend from nasopharynx
area to the nasal cavity and oropharynx.
Skull destruction nor nerve injury were not

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JNEVI Journal of Neurovascular Intervention Vol 2.No.1 January 2020

Figure2. Cerebral angiography by injection

from right internal carotid artery with
anteroposterior projection (A), lateral
projection (B), and right external carotid artery
with anteroposterior projection (C), lateral
projection (D). On figurre 2A and B, normal
circulation were observed without any
vascular supply towards the tumor. On figure
2C and D, tumor blush were seen (white circle
with dotted line) which had artery supply from
right sphenopalatine artery, branch from right
distal internal maxillary artery (arrow).

Figure 3. Intraarterial embolization procedure with Polyvynl Alcohol Particle (PVA) with size of
150-250µ.On figure 5A and B, microcatheter tip were seen proximally on right sphenopalatine artery.
On angiography evaluation, there is no collateral circulation extra nor intracranially, which increase
the possibility of embolization procedure to proceed. After the first PVA were administered, first
angiography were done (figure 5C), and tumor blush visibility was decreasing (~50%). Figure 5D and
E, tumor blush was not seen after embolization procedure. Retrograde flow from contrat was seen
which indicates right sphenopalatine artery occlusion. Figure 5F angiography on right internal carotid
artery, normal intracranial circulation were observed.

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JNEVI Journal of Neurovascular Intervention Vol 2.No.1 January 2020

Figure 4. Check angiography on right internal carotid artery (above) (4A) and right external carotid
artery (4B). On figure 4A, normal intracranial circulation were observed. On figure 4B, branches from
external carotid artery were seen intact. However, severe vasospasm were observed on 1/3 medial of
external carotid artery. CT scan with contrast post embolization (below) with saggital view (A),
coronal (B), and axial (C) 7 days after embolization. Heterogeneous contrast enhancement were seen
on tumor lesion that decreased >80% compared with previous imaging before embolization.

Figure 5. Tumor tissue of 4,5 x 3 x 1 cm in size, with white solid consistency, and total bleeding
volume of 120cc (above). Anatomical pathology (below) with 10x magnification (A), and 40x
magnification (B). Tissue were seen covered by epithelial cells, and vascular proliferation were seen
subepithelially between microfibromatous stroma.

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Discussion
Specific clinical manifestations of JNA is
a progressive unilateral nasal obstruction
(80-90%) with rhinorrhea and recurrent
unilateral epistaxis (45-60%). Other
clinical manifestations are a headache
(25%), facial pain, unilateral otitis media,
chronic rhinosinusitis, proptosis, and
visual disturbance. A Headache and
facial pain were resulted due to paranasal
sinus obstruction. Unilateral otitis media
were manifested due to disruption of
eustachius tube. Expansion of tumor to
sinonasal space can give a manifestation
of chronic rhinosinusitis. Proptosis and
visual disturbance indicate abnormalities
of orbital, and swollen cheek,
neurological deficit, olfactory
disturbance, and otalgia can also be
observed.6
In this patient, based on
DSA/embolization and angiography,
tumor in the nasal cavity was seen
expanded to the sphenoid sinus that
received single vascular supply from a
right sphenopalatine artery.
Anatomically, a sphenopalatine artery is
a terminal branch of a maxillary artery
that goes through the sphenopalatine
foramen to nasal cavity, posterior part of
superior meatus. Several branch and
anastomosis that is related with maxillary
artery like anterior temporal profunda
artery, medial temporal profunda artery,
foramen rotundum arteries that are
related with inferolateral trunk,
descending palatine artery that gives
vascularization to palatum mole,
descending palatine artery,
sphenopalatine artery, and infraorbital
artery. Temporal profunda artery
sometimes was visualized as
“pseudomeningeal appearance”. Foramen
rotundum artery has twisted arching
shape that is always going towards sellar
wall, and this artery has a very important
anastomosis in internal maxillary artery
embolization.7
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Management that could be done is as
follows; surgery, radiation, cryotherapy,
electrocoagulation, hormonal therapy,
embolization, and sclerosing agent
injection. Surgery is the golden standard,
but huge bleeding risks are due to a
highly vascularized tumor, mostly
beyond 2000cc.8
Preoperative embolization is
recommended as standard procedure to
decrease blood loss during operation,
which enables the possibility of total
excision, decreasing complication, and
minimalizing residual tumor.9 The
purpose is to decrease vascular supply for
the tumor, which in this case will be
efficient if the embolic agent can be
administered inside the tumor, and is very
effective if reached by small particle like
polyvinyl alcohol. Choosing particle size
is to balance between safety and
efficiency, as well as determining
whether catheter position can be reached
by direct injection of the embolic agent
towards the tumor. Embolization can
decrease 60-70% intraoperative bleeding.
Surgery resection can be done 2-5 days
after embolization. Injections with speed
exceeding arterial flow can result in
reflux towards the proximal arterial trunk
and intracranial embolization can occur.
Radiation as therapy is still debatable due
to the risk of sarcomatoid transformation.
Embolization in this patient was done by
using polyvinyl alcohol particle with the
size of 150-250 µ. According to
literature, using the particle of 150-250 µ
(ContourTM; Boston Scientific, USA)in
size can embolize small arteriole inside
the tumor bed, expected to necrotize
about 30-95%. Three main factor that
determines the success rate of
intratumoral deposition from the embolic
material, which are: (a) selectivity of
choosing catherization, (b) embolic
material choice, and (c) no arterial spasm
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on embolization. To avoid vasospasm
during ECA territory, a guiding catheter
was positioned only proximal from the
origin of ECA or towards proximal ECA,
and both (microcatheter and inserting
micro guidewire) inside the ECA branch
which was done in a slow manner.9
Complications that might occur during
JNA embolization which were reported in
the literature that can be considered
serious include stroke, blindness, and
paresis of cranial nerves. Stroke can
occur if there is the embolic reflex of
material from ECA to ICA, or when
embolic materials moved from ILT
branch (inferolateral trunk) and ICA
towards main blood vessels. Reflex could
be avoided by injecting embolic material
very carefully and along with heart rate,
and during systolic heart rate from
arterial blood flow. Paralysis of a cranial
nerve can occur when a branch of ICA or
ECA gives vascularization to cranial
nerves, such as blocked ILT.12JNA is a
tumor with a high recurrence rate, about
32% until as high as 40-50% in cases
with basis cranii invasion.8
Conclusion
The preoperative embolization is
effective to devascularization the tumor
and reduce the amount of bleeding during
surgery which contributes to reducing
risks and complications during procedure.
The prognosis of this patient, which was
already mentioned above is dubia et
bonam, considering that there was no
involvement of the tumor towards basis
cranii.
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