Case Report 2

Juvenile Nasopharyngeal Angiofibroma

By :

Aulia Hanum

Advisor :

dr. Achmad Bayhaqi Nasir Aslam, SpRad(K)

Specialist Medical Education Program I

Radiology

Medical Faculty of Brawijaya University

Malang

2018
 Table of Content
Table of Content....................................................................................................i

Table of Figures....................................................................................................ii

CHAPTER I Preface.............................................................................................1

CHAPTER II Case Presentation...........................................................................3

2.1 Identity........................................................................................................ 3
2.2 History Taking.............................................................................................3
2.3 Physical Examination..................................................................................3
2.4 Additional Examination................................................................................5
2.4.1 Water’s (Aisyiah Hospital).................................................................5
2.4.2 Thorax Radiography..........................................................................6
2.4.3 Head CT scan (Panti Waluya Hospital).............................................7
2.4.4 Endoscopy........................................................................................9
2.4.5 Biopsy...............................................................................................9
2.4.6 CT Angiography..............................................................................10
2.4.7 Laboratory Examination..................................................................11
2.5 Preoperative Embolization.....................................................................12
2.6 Surgical Therapy....................................................................................12
CHAPTER III Discussion....................................................................................14

3.1 Introduction...............................................................................................14
3.2 Epidemiology.............................................................................................15
3.3 Clinical Presentation..................................................................................15
3.4 Radiographic Features..............................................................................19
3.5 Treatment..................................................................................................22
3.6 Prognosis..................................................................................................23
3.7 Differential Diagnosis................................................................................23
Bibliography........................................................................................................25

i
 Table of Figures
Figure 1. Waters Radiography..............................................................................5
Figure 2. Thorax Radiography..............................................................................6
Figure 3. CT scan.................................................................................................8
Figure 4. Endoscopy.............................................................................................9
Figure 5. CT Angiography...................................................................................10
Figure 6. Embolization........................................................................................12
Figure 7. Mass in the left nasal cavity.................................................................13
Figure 8. Patient condition after surgery.............................................................13
Figure 9. Photograph of JNA patient...................................................................16
Figure 10. Intranasal softtissue mass of JNA......................................................16
Figure 11. Macroscopic of JNA...........................................................................17
Figure 12. Histologic images...............................................................................18
Figure 13. Staghorn-shaped blood vessels with endhotelial cell.........................18
Figure 14. The posterior wall of the maxillary sinus ...........................................20
Figure 15. Coronal CECT scan images .............................................................21
Figure 16. Axial section T1-weighted MRI...........................................................22
Figure 17. MRI with contrast...............................................................................22

ii
 CHAPTER I
Preface

Juvenile nasopharyngeal angiofibroma is an uncommon benign tumour of

the nasopharynx1. It accounts for less than 0.5% of all head and neck tumors, but
it is the most common tumor of the nasopharynx 2. A frequency of 1:5,000-
1:60,000 in otolaryngology patients has been reported. Juvenile nasopharyngeal
angiofibroma (JNA) occurs exclusively in males. Females with juvenile
nasopharyngeal angiofibroma should undergo genetic testing. Onset is most
commonly in the second decade; the range is 7-19 years3. Hippocrates first
described this tumor in the 5thcentury B.C. In 1940, Friedberg called it juvenile
angiofibroma. The term juvenile is debatable as JNA may occur in older patients
as well4.
Juvenile nasopharyngeal angiofibroma are slow-growing and initially
expand intra-nasally into the nasopharynx and nasal cavity and later spread into
the pterygomaxillary space2. The site of origin of JNA remains controversial.
While some authors stress its origin from the superior lip of the sphenopalatine
foramen at the junction of the pterygoid process and the sphenoid process of the
palatine bone; others believe that it arises from the vidian (pterygoid) canal2. Over
time, JNAs will eventually erode bone and invade the infratemporal fossa, orbit,
and middle cranial fossa2. Although a histopathologically benign tumor of
vascular origin, JNA behaves locally invasive and exhibits an aggressive clinical
course. Erosion of the adjacent anatomical structures, particularly of the bones,
may result in intracranial extension of this tumor5.
Many theories regarding the pathogenesis of JNA have been proposed.
Given the high prevalance of JNA among adolescent boys, one theory suggests
that the tumor may be hormone dependent. Although the genetic studies have
shown the expression of estrogen-androgen receptors by JNAs, an association
with increased serum hormone levels hasn’t been proved, and the influence of
the hormones on these tumors remains debatable5.
The diagnosis of JNA is essentially based on a detailed history and
clinical examination; confirmed by multiplanar imaging studies like Computed
Tomography (CT) and Magnetic Resonance Imaging (MRI). Incisional biopsies
are not recommended because of the vascular and hemorrhagic nature of this

1
tumor. Angiography is done to assess the vascular supply in larger tumors and to
make it possible to embolize the feeder vessel to reduce intra-operative bleeding.
Surgery is the Gold Standard of treatment, but for large expansile lesions
radiotherapy, chemotherapy, and hormone therapy are also used2.
Although several treatment modalities have been used during the past
century , surgical removal is widely accepted as the primary mode of therapy.
JNAs are highly vascular lesions, and their excision is frequently accompanied by
significant intraoperative blood loss. Preoperative tumor embolization has,
therefore, been proposed as an attempt to minimize surgical blood loss6.
JNAs are highly vascular lesions that are difficult to remove, and
hemorrhagic fatalities have been reported. The preoperative embolization of
JNAs, which was first described by Roberson and colleagues in 1972, can
significantly reduce the volume of blood loss. Blood loss without embolization can
reach volumes of more than 2000 ml7.
JNAs are usually fed by distal branches of the internal maxillary artery.
Embolization should be terminated when there is no significant residual tumor
blush to prevent the delivery of embolic material to normal dental and mucosal
tissues8.

2
 CHAPTER II
Case Presentation

2.1 Identity
Name : An. M

Gender : Male

Age : 10 years old

Occupation : Student

Address : Malang

2.2 History Taking

The patient complained with the lump in the left nose since one year ago.
He also got nasal congestion in the both nostril, feel decrasing of olfaction, and
had a nosebleeds from the left nostril since he was child especially when he feel
fatigue. He also complained with watery eyes on the left side. He got history of
surgery in May 2017 because he was diagnosed with polyp. The mass only taken
± 70% because there was a lot of bleeding during surgery and treated for 2
weeks after surgery, then a lump still appeared until now. Then the patient is
referred to RSSA. Patients have been given angiofibroma artery feeding
embolization on July 30, 2018 at RSSA. There is no history of cough, snoring,
sore throat, hoarseness, ear pain, discharge from the ear, tinnitus, hearing lost.
There is no history of drug allergy, family atopy, and also no family history with
tumor / cancer.

2.3 Physical Examination

General Condition :

GCS 456

BP 100/80

Pulse rate 90 x/min

RR 22 x/min

Temperature 36.7oC

Body weight 26 kg

Head and neck :

Pupil Isokor (3mm/3mm)

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Light reflex +/+

Anemia -/-

Icteric -/-

Thorax

Cor

S1/S2 Normal

Murmur (-)

Lung:

Breath sound Vesicular:

+ +

+ +

+ +

Ronchi -

Wheezing -

Abdomen:

Flat +

Soefl +

Bowel sounds +

Extremity :

Acral Warm

CRT < 2 second

4
Ear Nose Throat

CAE D/S CN D/ Faring

Edema -/- Hyperemi - Hyperemi -

Hyperemi -/- Edema - Granul -

Seret -/- Sekret -

MT D/S CN S/ Tonsil

Intact +/+ White mass with smooth T1/T1

surface
RC +/+ Hyperemi -/-

2.4 Additional Examination

2.4.1 Water’s (Aisyiah Hospital)

Figure 1. Waters Radiography

5
 Waters radiography show the mucosal thickening of the nasal cavity and
the left maxillary sinus. The conclusion of waters radiography is left rhinosinusitis
maxilaris.

2.4.2 Thorax Radiography

Figure 2. Thorax Radiography

The conclusion of thorax radiography on posteroanterior position is cor

and pulmo are within normal limit.

6
2.4.3 Head CT scan (Panti Waluya Hospital)

7
 Figure 3. CT scan

From the head CT-Scan there are solid mass in the projection of left nasal
cavity (with intraosseous impression) with size approximately 3 cm x 2. 15 cm x
4.3 cm. The mass has strong enhanchement after contrast admission, the mass
posteriorly reaching choane, with deviation of nasal septum to the right, pushing
the medial wall of the left maxillary sinus. There are also appearance of isodens
lesion in the left maxillary sinus, left etmoidalis sinus, left frontal sinus. But there
is no pathological lesion on the brain. The conclusion of head CT scan are solid
mass on the left intraosseus nasal cavity with the impression coming from nasal
concha, left hemipansinusitis, and normal intracranial.

8
2.4.4 Endoscopy

Figure 4. Endoscopy

Nasoendoscopy examination is perform on December 6 th 2017,from the

examination appears whitish flat mass filling the left nasal cavity and deviation of
nasal septum toward the lateral wall of the nasal cavity until it attaches to the
inferior konka in the middle 1/3. The nasopharynx is difficult to evaluate.

2.4.5 Biopsy

Macroscopic examination there is solid and white tissue measuring

aproximately 0.5 cm in diameter. From microscopic examination there is swollen
fibrouse connective tissue with infiltration of lymphocyte inflammatory cells and
eosinophils. There are no layered epithelium or malignant cells in biopsy
specimens. There is also seen blood vessels between the stoma. The conclusion
of biopsy examination that perform on January 8 th 2017 are suggestive part of a
nasal polyp probably a part of angiofibroma.

9
2.4.6 CT Angiography

Figure 5. CT Angiography

CT Angiography examination was performed on May 22nd 2018. From the

examination there is appearance of hypervascular mass in the nasal cavity region
and left choane extends to the inferior wall of the left nasopharynx, medially
urges the nasal septum and causing deviation of the right nasal septum as far as
1 cm.There is impression that the mass is being fed from the left internal
maxillary artery, bilateral fascial artery. The size of the mass is ± 2.7 x 5.1 x 1.3
cm. There is also appear dilatation from right fascialis artery (±2.48 mm), left
fascialis artery (± 1.94 mm), right maxillary artery (± 2.56 mm), and left maxillary
artery (± 2.64 mm). There is also isodens lesions appear on the left maxillary
sinus. And the CT Angiography examination is concluded with in accordance with
the description of hypervascular tumors in the left nasal cavity and left choane
which are fed from the left internal maxillary artery and bilateral facial artery,
dilation of bilateral fascialis artery and bilateral maxillary artery, and left maxillary
sinusitis.

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2.4.7 Laboratory Examination

Darah Lengkap Kimia Klinik

Hemoglobi 11,80 g/ 11,4- GDS 84 g/dL <200

n dL 15,1
SGOT 18 U/L 0-32
Lekosit 6.840 /µL 4700-
11,30 SGPT 11 U/L 0-33
0
Albumin 4,26 g/dL 3,5-
Hematokri 33,50 % 40-47 5,5
t
Ureum 16,70 mg/dL 16,6-
Trombosit 373.00 /µL 142- 48,5
0 424.1
03
Kreatinin 0,49 mg/dL <1,2

Diff count
Serum Elektrolit

Eosinofil 2,8 % 0-4

Natrium 143 mmol/ 136-
L 145
Basofil 0,6 % 0-1
Kalium 3,85 mmol/ 3,5-
Netrofil 48,8 % 51-67 L 5,0

Limfosit 40,2 % 25-33 Klorida 105 mmol/ 98-

L 106
Monosit 7,6 % 2-5

Faal Hemostasis

PPT 10,60 Dtk 9,3-

11,4

APTT 34,30 Dtk 24,6-

30,6

INR 1,02 <1,5

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2.5 Preoperative Embolization

Embolization is performed on July 30th 2018 with the following steps :

1. Patients are supine

2. General anesthesia by dr. Harjuna / dr. Rajid
3. Right inguinal puncture
4. Skeath 5 Fr installed
5. Vertebral cath 5Fr with hydrophilic wire 0.035 "is used for the cannulation
of bilateral carotis externa artery.
6. Mass appearance on nasal cavity-nasopharyngeal projections with
primary feeding from the bilateral internal maxilla (left dominance)
7. Super selective cannulation with nucso catheter tency and STC 2.4 Fr
with mouth through wire
8. Super selective arteriography not showing communication between
ophtalmica artery and internal carotid artery.
9. Embolization with 300-500 um of PVA until nearly stasis.
10. Appears decreasing tumor vascular supply after embolization DSA.

Figure 6. Embolization

2.6 Surgical Therapy

Extirpation of angiofibroma on the left nasal cavity with lateral Rhinotomy

approach and general anasthesia, with following step :

1. Patient's position is supine and general anesthesia are performed by

anesthetic.
2. Disinfection of surgical field demarcation.
3. Inserting the hypopharyngeal tampons.
4. Making landmarks of the area to be incised in the lateral of left nasal
cavity

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5. Infiltration of pehacain is carried out in the area to be incised
6. An incision is made with a moureour incision, incision perform layer by
layer until the bone is identified
7. Preparation of separating periosteum with bone at the side of piriformis
aperture, lateral wall of left nasal cavity perforated with pean then given
bandage, nasal retraction to contralateral, appearance of solid white
bluish mass on the nasal cavity until pushing the left hard palate and
reach choana.
8. Widening the site of incision on the lateral wall of nasal cavity. The
incision is directed to the superior with the mass and protected by raspat
and cutting inferior concha with concha scissors.
9. Mouthgag installation
10. Angifibroma is extracted with blakeslay while releasing the tumor
boundary with raspat, mass of white bluish solid with size approximately 6
x 5 cm
11. Evaluation of bleeding  bleeding is treated with cautery and tampons
12. Smoothening the surface of the piriformis bone by using a ‘kikir’
13. Belloq tampon was installed
14. Washing with normal saline on the left nasal cavity
15. Evaluate the bleeding and insert a boorzalf tape tampon, first tape
inserted in posterior to the nasal cavity, the second to the ethmoid sinus
16. Surgical sutures are performed, wound closure with sufratule and hypafix
gauze
17. Operation completed

Figure 7. Mass in the left nasal cavity

Figure 8. Patient condition after surgery

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 CHAPTER III
Discussion

3.1 Introduction
Juvenile nasopharyngeal angiofibroma (JNA) is a combined vascular and
fibrous stroma arising in the nasopharynx. Hippocrates described the tumor in the
5th century BC, but Friedberg first used the term angiofibroma in 1940.
Angiofibromas are histopathologically benign but potentially locally destructive
vascular tumors. They are unencapsulated neoplasms composed of a rich
vascular network within a fibrous stroma9. The blood vessels are
characteristically large and stellate, often with a “staghorn” appearance. Others
range from smaller capillaries to sinusoids. The blood vessels are typically devoid
of smooth muscle, although JNA have vessels with an uneven layer of smooth
muscle. The stroma is dense and fibrous. The stromal cells are predominantly
fibroblasts and rarely myofibroblasts. Cellularity can vary. The stromal cells are
strongly positive for vimentin and negative for CD 34. A few cells may be focally
positive for smooth muscle actin. Beta-catenin has also been observed in the
nuclei of fibroblasts but not in endothelial cells of most JNA10.

Histopathologically, the tumor is comprised of haphazardly arranged

vascular channels surrounded by dense paucicellular fibroblastic stroma, with the
myofibroblast being the principal cell. The bleeding propensity of these tumors
is due to the findings that the smaller vessels in the center of
the lesion tend to lack muscular elastic lamina, predisposing these
vessels without a muscular surrounding layer that may otherwise
assist in vasoconstriction to uncontrolled bleeding. The blood supply to these
lesions is derived from the internal maxillary artery, a branch off the external
carotid artery11.

JNA is thought to originate from the posterior-superior part of the

sphenoplatine foramen, which is located on the posterolateral wall of the nasal
cavity. The tumor is located in close relationship with the pterygoplatine fossa,
and although growing slowly, it may extend into the infratemporal fossa, the
maxillary sinus, nasal cavity, orbita, sphenoid sinus, skull base, and into the
cranial cavity5.

Many theories regarding the pathogenesis of JNA have been proposed.

Given the high prevalance of JNA among adolescent boys, one theory suggests
that the tumor may be hormone dependent. Both androgen and estrogen
receptors have been demonstrated in JNA. Recently, estrogen beta-adrenergic
receptors have been found in a high percentage of JNA. Schlauder and
associates have postulated that the presence of aromatase in tumor cells
converts endogenous androgens to estrogens, causing tumor growth via an
autocrine-like mechanism. Most tumors stain for vascular endothelial growth
factor12.

14
 Although the genetic studies have shown the expression of estrogen-
androgen receptors by JNA, an association with increased serum hormone levels
hasn’t been proved, and the influence of the hormones on these tumors remains
debatable5.

From this case, the patient age is on the first decade of life. He is a male
and complaining of the lump since a year ago, when he is 9 years old. This is
relevant with the theory that JNA is almost exclusively
affects males, usually around the age of adolescence with a range
involving boys from ages 7 to 29 years old11.

3.2 Epidemiology
Juvenile nasopharyngeal angiofibroma is the commonest benign tumour
of the nasopharynx but accounting for 0.5% of all head and neck tumours 1. This
tumor has a predilection for males, and some believe that it occurs exclusively in
males. Nasopharyngeal angiofibroma is most common in the second decade of
life and is uncommon after age 25 years13. 

Incidence of JNA is 3.7 per 1,000,000 males.14 A review of the literature

reveals rare cases of female and older male patients with JNA 5. If a female is
affected, testicular feminization has to be excluded by chromosome analysis.
Although white patients tend to be fair-skinned and red-haired, this is not the
case in other endemic populations (Central and South Americans, Africans, or
Asians)14.

3.3 Clinical Presentation

JNA classically presents as a painless, progressive unilateral nasal
obstruction4. The percentage of typical symptoms of JNA are unilateral
progressive nasal obstruction (80%–90%) and recurrent epistaxis (45%–60%) 5.
The other symptomps like rhinorrhea, pain, and facial swelling may also be
seen3,4. If the tumor already extended into softtissues of the face and orbit, the
patients may present with facial deformities, proptosis, rhinolalia, deafness,
sinusitis, otitis, and a bulging palate resulting from extension of the tumor into soft
tissues of the face and orbit14.

Clinical examination reveals a firm and friable mass in the nasopharynx

and nose. As this tumor is aggressive and expansile, it invades adjacent
structures causing further symptoms. Impaired Eustachian tube function and
changes in visual acuity may be seen. Invasion of the intracranial region may
lead to cranial nerve palsy. Angiofibromas originating outside the nasopharynx
may appear as an intraoral mass in the retromolar or buccal space area4.

15
 Figure 9. Photograph of JNA patient showing diffuse sweling on the right side face15

Figure 10. Intranasal softtissue mass of JNA15

Our patient was a 10-year-old boy with a chief complaint of lump in the left
nose. He also got recurrent left-sided epistaxis for four year and watery on the left
eye. This case presenting the symptoms that the mass is already extended into
softtissue of the orbit15.

Endoscopic examination of JNA usually shows a mass involving the

posterior nasal wall. Various staging systems have been proposed, with size and
location determining the outcome14.

Macroscopic finding of JNA are round or nodular, nonencapsulated

masses with a sessile or pedunculated base. The tumors may be large (up to
22 cm), although the mean size is 4 cm. The surface of the tumors is largely
covered by intact mucosa, frequently taking the shape of surrounding structures.
The cut surface is variable and shows dilated vascular channels, which give the
tumors a spongy appearance. In less vascular areas the tumors appear solid and
fibrotic14.

16
 Figure 11. Macroscopic of JNA

After performing extirpation, we found solid white bluish mass on the

nasal cavity until pushing the left hard palate and reach choana. The
characteristic of the mass is similar with the theory of JNA. On gross
examination, the mass of JNA is usually sessile, firm, rubbery, lobulated, and red-
pink to tan-grey in appearance16.

The microscopic appearance of JNA rests on the variable presence of

three elements: an abnormal vascular network, a connective tissue stroma, and
stromal cells. The vascular network consists of mostly thin-walled, slit-like
(“staghorn”) or dilated vessels, with calibers ranging from capillary to large,
patulous vessels. The muscular layer can be absent, focal, and pad-like, or
circumferential. Elastic fibers are typically absent, one of the reasons for the
profuse bleeding (vessels cannot contract down). The vascular spaces and lining
endothelium appear to be resting directly on the connective tissue stroma. The
endothelial cells may be plump but are usually attenuated. The fibrous connective
tissue stroma consists of plump spindle, angular, or stellate-shaped cells, and a
varying amount of fine and coarse collagen fibers. Myxoid changes are seen
especially in embolized specimens. The nuclei of the stromal cells are generally
cytologically bland, but they may be multinucleated or show some degree of
pleomorphism in the more cellular areas, thought to represent senescent or
degenerative changes. The chromatin is finely and evenly dispersed. Large
stromal cells with abundant cytoplasm resembling ganglion cells can be
identified. Mitotic activity and nuclear atypia are not findings of typical NPA. Mast
cells may be seen, but other inflammatory elements are usually absent (except if
there is surface ulceration). Long-standing lesions show increased fibrosis and
diminished vasculature. Treatment with androgen receptor blockers (such as
flutamide) results in increased collagenization of the stroma with fewer, but
thicker-walled vessels. In specimens excised after embolization treatment, the
tumor often shows areas of infarction, and embolic material can be seen in some
blood vessels. Sarcomatous transformation is an exceedingly uncommon event,
usually following massive doses of radiation14,17.

17
 Figure 12. Histologic images showing increased peripheral vascularity.

Figure 13. Staghorn-shaped blood vessels with endhotelial cell

We found swollen fibrouse connective tissue with infiltration of lymphocyte

inflammatory cells and eosinophils from microscopic examination on our patient.
There are no layered epithelium or malignant cells in biopsy specimens. There is
also seen blood vessels between the stoma. It is suitable with the theory that
microscopic wise the tissue of JNA are composed of two components: blood
vessels and a fibrous stroma. The blood vessels are characteristically large and
stellate. Others range from smaller capillaries to sinusoids. The blood vessels are
typically devoid of smooth muscle, although JNA have vessels with an uneven
layer of smooth muscle. The stroma is dense and fibrous. The stromal cells are
predominantly fibroblasts and rarely myofibroblasts10.

Several staging systems have been proposed. Most are designed to guide
decisions regarding the resectability and optimal surgical approach to the tumor
rather than to predict prognosis12. The most commonly used are those of
Sessions, Radowski, and Fisch. Classification according to Sessions, are :

 Stage IA - Tumor limited to posterior nares and/or nasopharyngeal vault

 Stage IB - Tumor involving posterior nares and/or nasopharyngeal vault
with involvement of at least 1 paranasal sinus
 Stage IIA - Minimal lateral extension into pterygomaxillary fossa
 Stage IIB - Full occupation of pterygomaxillary fossa with or without
superior erosion of orbital bones
 Stage IIIA - Erosion of skull base (ie, middle cranial fossa/pterygoid base);
minimal intracranial extension
 Stage IIIB - Extensive intracranial extension with or without extension into
cavernous sinus13

18
 Classification according to Radowski are :

 Stage IA - Tumor limited to posterior nares and/or nasopharyngeal vault

 Stage IB - Tumor involving posterior nares and/or nasopharyngeal vault
with involvement of at least 1 paranasal sinus
 Stage IIA - Minimal lateral extension into pterygomaxillary fossa
 Stage IIB - Full occupation of pterygomaxillary fossa with or without
superior erosion of orbital bones
 Stage IIC - Extension into the infratemporal fossa or extension posterior to
the pterygoid plates
 Stage IIIA - Erosion of skull base (ie, middle cranial fossa/pterygoid base);
minimal intracranial extension
 Stage IIIB - Extensive intracranial extension with or without extension into
cavernous sinus18.

Classification according to Fisch are :

 Stage I - Tumors limited to nasal cavity, nasopharynx with no bony

destruction
 Stage II - Tumors invading pterygomaxillary fossa, paranasal sinuses with
bony destruction
 Stage III - Tumors invading infratemporal fossa, orbit and/or parasellar
region remaining lateral to cavernous sinus
 Stage IV - Tumors invading cavernous sinus, optic chiasmal region,
and/or pituitary fossa3.

Based on Fisch classification, our patient is categorized as stage II JNA.

Based on the theory, most of the tumors were in stage II. Accurate staging, and
adequate treatment are essential in the management of this lesion4. The
transpatal approach was used only in stage I tumors while the endonasal
approach was used in stage I and limited stage II tumors. Lateral rhinotomy was
used in stage I, II, and III tumors. The transfacial maxillary swing approach was
used in stages II, III, and IV tumors16. And our patient is got extirpation of
angiofibroma on the left nasal cavity with lateral rhinotomy approach.

3.4 Radiographic Features

Diagnosis of JNA may originally be made on computed tomo-graphy (CT)
examination or even suspected on the basis of plainradiographs. CT has the
benefit of better detailing osseous involvement of tumor. Definitive diagnosis,
however, is made on magneticresonance imaging (MRI) examination. The
optimal sequence to identify the anatomy and extent of intracranial extension of
JNA involves T1 weighted imaging (T1WI) with gadolinium contrast,utilizing fat-
suppression. An MR-angiogram can additionally bevaluable in defining the
arterial supply to the tumor, of primary importance for pre-embolization
planning11.

Plain films of the paranasal sinuses are the first line of investigation for
sinonasal pathology, the radiographic findings may include demonstration of the
nasopharyngeal mass which may extend into the pterygopalatine fossa resulting

19
in widening of the pterygopalatine fossa and anterior bowing of the posterior wall
of the ipsilateral maxillary antral wall. The lateral view of the plain radiograph of
the skull may demonstrate opacification of the sphenoid sinus which may spread
to also include the maxillary and ethmoid sinuses. Another plain film finding is
widening of the inferior and superior orbital fissures which is an indication of
spread into the orbit and intracranial extension1.

Computed Tomographic findings similar to those described for plain film

features are clearly demonstrable with better resolution. On CT images, a JNA
appears as a heterodense mass that is centered within the sphenopalatine
foramen. On contrast-enhanced CT images, the mass shows avid enhancement.
At the time of diagnosis, the mass classically involves the pterygopalatine fossa;
in thislocation, it produces a bowing of the posterior wall of the maxillary antrum,
widening of the pterygopalatine fossa, and inferior orbital and pterygomaxillary
fissures. Bony erosion of the nasal cavity, hard palate, and pterygoid plates are
also common. Anterior bowing of the posterior maxillary wall, due to tumor in the
pterygomaxillary space on axial CT, known as the Holman-Miller sign, is one of
the characteristic findings of JNA. CT provides good hard tissue imaging to show
invasion of the cancellous bone of the sphenoid. The deeper the extension, the
greater is the chance of tumor remnants being left behind after surgery, resulting
in recurrence. Coronal CT images are used to evaluate the staging of the tumor,
showing the relationship between the tumor and surrounding structures, so that
the choice of operative approach, prognosis, and other treatment options can be
planned accordingly1,2.

CT Angiography with contrast examination show the appearance of

hypervascular mass in the left nasal cavity region and choane extends to the
inferior wall of the left nasopharynx, medially urges the nasal septum and causing
deviation of the right nasal septum on our patient. It is suitable with the theory
that from CT scan presence of a soft tissue mass in the posterior nasal cavity
combined with enlargement of the sphenopalatine foramen and erosion of its
posterior bony margin19.

20
 Figure 14. The posterior wall of the maxillary sinus has been bowed anteriorly

Computed tomography Scan with contrast enables accurate diagnosis

without resorting to the dangers of biopsy. Contrast Enhanced Computed
Tomography (CECT) is reliable for mapping tumor extensions into skull base and
bony erosion. All staging systems for JNA rely upon tumor extensions on
preoperative imaging for its classification. This emphasizes the importance of
imaging in staging and thereby determining prognosis 20. JNA is demonstrable as
an avidly enhancing lobulated non-encapsulated soft tissue nasopharyngeal
mass on CT scan following intravenous administration of contrast medium
reflecting its characteristic vascularity. This was observed on the scan of the
index patient. Intraorbital and intracranial extension is also better demonstrable.
Bone window setting highlights the important advantage of CT imaging in its
superiority in evaluating bony details compared to plain films and magnetic
resonance imaging (MRI)1.

Figure 15. Coronal CECT scan images showing the contrast enhancing mass seen widening the
pterygoid wedge compared to the uninvolved side (RAM HARAN sign)20

MRI is superior to CT for detecting the intracranial extension of the tumor into
soft tissues of the skull base. The lesion characteristically shows low signal
intensity on T1-weighted images. On T2-weighted images, heterogeneous
intermediate signal intensity is seen in the tumor mass and in contrast-enhanced
MR images, avid enhancement with flow voids are observed. These MR imaging
features, together with the patient’s age, can help in differentiating a JNA from
other nasopharyngeal lesions. MR imaging is even more important
post-operatively to show any residual or recurrent tumor and monitor the effects
of radiotherapy2.

MRI because of its better soft issue characterization in comparison to CT is

able to delineate mucosal inflammation versus sinus fluid. It is also valuable at
evaluating tumour extension into the orbit and intracranial compartments and is
accurate in tumour staging. JNA is of intermediate signal intensity and
heterogenous signal intensity on T1 and T2 weighted spin echo sequences
respectively. Multiple flow voids will also be seen within the tumour on both

21
sequences. Just as with CT scanning, following intravenous administration of
Gadolinium, JNA shows prominent enhancement, again demonstrating the
vascularity of this benign tumour. The presence of prominent flow-voids seen on
MRI scans represents enlarged tumour vessels. MR imaging also better
delineates spread in the region of the cavernous sinus and into the middle cranial
fossa1.

Figure 16. Axial section, T1-weighted MRI shows a large, well-defined mass2

Figure 17. MRI with contrast show large tumor in the infratemporal fossa and cheek shows
intensive, inhomogenous contrast enhancement21

3.5 Treatment
The gold-standard treatment of JNA is the surgical excision of the tumor
after a preoperative embolization procedure. Due to the complex anatomy of the
skull base, excision of the advanced tumors may be challenging, and
chemotherapy, radiotherapy, or hormone therapy may be added to the
treatment5.

22
 Surgical approaches used to remove angiofibroma, depending on its
origin and extensions, are listed below22 :

Location Approach
A. Nose and nasopharynx Transpalatal or endoscopic
B. Nose, nasopharynx maxillary Lateral rhinotomy with medial
antrum and pterygopalatine fossa maxillectomy OR Endoscopic
OR Le Fort I
C. As in B + Infratemporal fossa Extended lateral rhinotomy
OR Infratemporal fossa approach OR
Maxillary swing approach
D. As in C + Cheek extension Extended lateral rhinotomy
E. As in B + C + Intracranial Combined intracranial and
extracranial approach (craniotomy +
one of the extracranial approaches)
OR Radiation if intracranial part is
inaccessible
F. Residual or recurrent disease Observation OR repeat surgery or
(extracranial) radiation if inaccessible
G. Intracranial residual or recurrent Stereotactic radiation (X or gamma
knife)
Bilateral angiography of the carotid system and preoperative embolisation
are recommended to reduce intraoperative bleeding and facilitate resection,
decreasing operative time, risk of recurrence and transfusion requirements. The
benefit of embolisation should be discussed and weighed against its morbidity.
This varies widely and should be adjusted according to the patient, institution and
interventional radiology team23.

In 1972, Roberson et al. were the first to advocate the preoperative

embolization of JNA using barium impregnated silastic spheres. Tumor
embolizations were subsequently performed using numerous materials such as
silk, gelfoam, fibrin glue, and gelatin spheres. As the endovascular field has
advanced, these materials have given way to particulate and liquid embolics.
Particulate embolics include polyvinyl alcohol and Embospheres (Guerbet
Biomedical; Louvres, France). Although they are cheaper and may achieve better
penetration within the tumor capillary bed, particulate embolics have several
distinct disadvantages compared with liquid agents. First, the particles are
radiolucent and require a contrast solution to indirectly determine the extent of
tumor embolization. This adds to the overall contrast load of the procedure and
should be a consideration in the young age group in which these tumors present.
Second, the particles tend to coalesce and occlude the microcatheters,
necessitating recatheterization of feeding arteries. Third, the particles dissipate
over time allowing revascularization of the tumor6.

23
 In JNA, superselective embolization of feeding vessels arising from the
external carotid artery is highly effective and safe. Anastomosis between
branches of the external and internal carotid artery and vascular spasm have to
be considered when planning superselective embolization. Several materials
have been tried over the years, including non-absorbable silastic spheres,
Gelfoam, dura mater, and polyvinyl alcohol particles. Recently, non-absorbable
polyvinyl alcohol particles are preferred19.

Embolization is perform 2 weeks before our patient got his surgery.

Embolization is using 300-500 um of PVA until nearly stasis. After got
embolization, we observed the patient and there is no complication after the
procedure. The most significant complication of embolization procedure would be
migration of an embolus into the intracranial circulation, but this is considered
rare. Other complications associated with embolisation include systemic reaction
to contrast, infection at the puncture site, femoral hematoma and thrombosis,
blindness, facial paralysis, skin necrosis, oronasal fistula, seizures, fever and
facial pain (1,13-17). Complications of arterial embolisation occur in 17% to 25%
of cases and are classified into major or minor. Minor complications include pain
or paresthesia, headache and facial edema, which usually resolve within a week.
Major complications include stroke, blindness, facial paralysis, septal perforation,
and skin or palate ulcers or necrosis, all of which have an incidence of less than
2%23.

Radiotherapy has been used as a primary mode of treatment. A dose of

3000 to 3500 cGy in 15–18 fractions is delivered in 3–3.5 weeks. Response is not
immediate. Tumour regresses slowly in about a year, sometimes even up to 3
years. Radiotherapy is also used for intracranial extension of disease when
tumour derives its blood supply from the internal carotid system22.

Treatment with radiotherapy is controversial. Some believe that all large

tumours with intracranial extension should be treated with radiation while others
reserve it for recurrent inoperable tumours22. Complications from radiation have
included well-documented secondary malignancies, cataracts, CNS
complications as well as panhypopituitarism. The risks of radiation should be
carefully weighed against other available modalities especially in these young
patients18.

Since the tumour occurs in young males at puberty, probably activated by

testosterone, hormonal herapy as the primary or adjunctive treatment has been
used. Diethylstilbestrol and flutamide (an androgen blocker) have been used in
the past to arrest the growth but no signifcant regression has been observed in
practice.

Chemotherapy has been used for the very aggressive recurrent tumours
and residual lesions. Doxorubicin, vincristine and dacarbazine have been used in
combination. Chemotherapy and radiotherapy can arrest the growth and cause
some tumour regression but not total tumour eradication.

24
3.6 Prognosis
This benign tumor is characterized by local aggressive growth, with
recurrences in up to 25% of patients, most commonly intracranial, and usually
within the first 2 years after diagnosis. Mortality has ranged up to 9% due to
hemorrhage and intracranial extension, but this figure has dropped with improved
radiographic and surgical techniques. Patients may be managed with selective
angiographic embolization or hormonal therapy prior to definitive surgical
resection (usually via a lateral rhinotomy). However, most clinicians do not wish
to use hormone manipulation in pubertal males. Spontaneous regression post
puberty is reported. Radiation therapy has been successfully implemented to
manage large, intracranial, or recurrent tumors, but surgery is still the therapy of
choice. The rare case of malignant transformation represents postradiation
sarcoma14.

3.7 Differential Diagnosis

The differential diagnosis of JNA includes antrochoanal polyp,
inflammatory sinonasal polyp, neurofibroma, adenoidal hypertrophy,
nasopharyngeal cyst, pyogenic granuloma, chordoma, and malignant neoplasms,
such as nasopharyngeal carcinoma, lymphoma, or rhabdomyosarcoma. CT and
MR imaging of nasopharyngeal carcinoma show an inhomogeneous mass arising
from the nasopharyngeal mucosa or submucosal space with skull-base erosion
or intracranial extension. Lymphoma may arise from adenoidal tissues of the
nasopharynx or Waldeyer’s ring and can be associated with lymphadenopathy.
Imaging of rhabdomyosarcoma reveals a soft-tissue mass with frequent bone
destruction and mild enhancement on CT, but marked enhancement on MR. DWI
and their mean ADC values can be used for distinguishing between benign and
malignant tumors imaging5,19.

25
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