Rom J Morphol Embryol 2013, 54(3 Suppl):867870

CASE REPORTS

RJME

Romanian Journal of
Morphology & Embryology
http://www.rjme.ro/

Particular aspects in endoscopic surgery for

juvenile nasopharyngeal angiofibromas.
Case reports and review of literature
V. BUDU1), I. BULESCU1), CARMEN AURELIA MOGOANT2)
1)

Prof. Dr. Dorin Hociot Institute for Phono-Audiology

and Functional ENT Surgery, Bucharest
2)
ENT Department,
University of Medicine and Pharmacy of Craiova

Abstract

Juvenile nasopharyngeal angiofibromas (JNA) are rare, benign, highly vascular tumors which appear in proximity to the sphenopalatine foramen.
The tumor arises most commonly in adolescent males suggesting that in could be hormonally responsive. Although it is histologically
benign, it has a high destructive potential and a high grade of recurrence. It is a fibrous vascular tumor with vascular sources from branches of
the external or internal carotid arteries. Modern treatment of JNA includes surgery and also radiotherapy, chemotherapy and hormone therapy.
This paper presents two cases from the eight of our clinics experience, with morphological features which made their treatment challenging.
Keywords: juvenile angiofibroma, nasopharynx, endoscopic surgery, arteriography.

Introduction
Juvenile nasopharyngeal angiofibromas represent between
0.05% and 0.5% of head and neck neoplasms [13]. It is
a highly vascular tumor, which affects primarily adolescent
males [4]. Usually, JNA patients present with unilateral nasal
obstruction, recurrent epistaxis and nasopharyngeal mass
in adolescent males [1]. The histological appearance of the
tumor is benign, although some theories suggest that it could
possibly be a vascular malformation [47]. The tumor usually
arises near the sphenopalatine foramen and extends towards
nearby structures [8, 9]. Histopathologically, JNAs are nonencapsulated tumors composed of a mixture of blood vessels
and fibrous stroma [1, 3, 10]. Microscopic examination of
the tumor reveals plump fibroblasts of ovoid to spindle
shape and a high amount of connective tissue. The stroma
contains blood vessels of different sizes and shapes lined
by endothelial cells but with little or no smooth muscle or
elastic fibers (Figure 1).

Figure 1 Juvenile nasopharyngeal angiofibroma in

HE stain showing blood vessels lined by endothelial
cells but no muscle fibers in their structure (100).
ISSN (print) 12200522

This structure, lacking muscles fibers, contributes to

the capacity of JNAs to bleed excessively after minimal
manipulation [1, 11, 12], thus making its surgical treatment
a challenge.
Because of the rich vascularity of this type of tumor,
the vascular sources that supply it have been long studied
and observed. Usually, the blood supply of JNA comes
from branches of the external carotid arteries, but
sometimes also from the internal carotid system [9, 13].
According to the study of Tang IP et al. [9] the tumors
were supplied by branches from both left and right
maxillary arteries in 69.2% of cases and 30.8% were
supplied by branches from the ipsilateral maxillary
artery. These results were similar to the ones presented
by Pryor SG et al. [14], where bilateral and unilateral
contributions to the vascular supply of the tumor were
almost equal in numbers. Knowledge of the vascular
supplies of JNA is of great importance prior to any
surgical procedure for this tumor. For that reason, preoperative angiography is usually a standard procedure.
Treatment for JNA is usually surgical. Other treatment
methods have been emphasized in the past such as chemotherapy, radiotherapy or hormone therapy. These methods
are nowadays only used as occasional complementary
treatments [1, 8, 10, 15]. Surgical approaches for JNA
include classic approaches such as medial maxillotomy,
transpalatine, lateral rhinotomy, mid-facial degloving, and
more recently video-assisted nasal endoscopic surgery
[8, 14, 1618]. The type of surgical approach for JNA,
classical or endoscopic is limited by the extension of the
tumor in surrounding areas such as pterygopalatine fossa or
infratemporal fossa [19]. However, Mitskavich MT et al.
[17] concluded that the tumor may be treated exclusively
via nasal endoscopy even when it is extended to the
pterygopalatine of infratemporal fossae [8]. The endoscopic
ISSN (on-line) 20668279

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V. Budu et al.

or classic approaches are chosen in relation to the tumoral

stage. Staging of JNA has been heavily disputed among
authors according to certain criteria like behavior of the
tumor [20], routes of spread, difficult areas for surgical
exposure and possible sites of recurrence [4]. According
to Andrade NA et al. [8], the current staging used for
JNA is that of Andrews JC et al., which classifies local
and regional spreading of the tumor, as well as bone
destruction [22]. Whatever the stage, or the surgical
technique used, most authors consider that preoperative
selective arterial embolization of feeding vessels of the
tumor has significantly decreased intraoperative bleeding
and facilitated resection of larger tumors [1, 23].
Patients, Methods and Results
This paper presents two cases from the eight of our
clinics experience, with morphological features which

made them challenging from a surgical point of view. Both

patients underwent preoperative computed-tomography
scans and angiography. Both cases were resolved using
an endoscopic endonasal surgical technique.
Case No. 1
The first case was an 18-year-old male, which presented
with unilateral nasal obstruction and recurrent epistaxis
following surgery for JNA with ligation of the left external
carotid artery three years before presentation. The CTscan revealed a tumoral mass, which completely blocked
the rhinopharynx and the posterior third of both nasal
fossae. Preoperative angiography revealed a vascular-type
tumor with feeding vessels from the left internal carotid
artery (Figure 2). The protocol used for this case was
endoscopic endonasal surgical excision with prior dissection
and exposure of the left common carotid artery.

Figure 2 Case No. 1: CT-scan showing the tumoral mass obstructing the rhinopharynx. Pre-operative angiography
revealing vascular sources from the internal carotid artery.

Case No. 2
The second case was a 14-year-old male, which
presented with bilateral nasal obstruction, epistaxis and
rhinophonia following previous adenoidectomy. The
CT-scan revealed a mass obstructing the rhinopharynx
extending to the left pterygopalatine fossa and the left

sphenoid sinus. Preoperative angiography showed a highly

vascular tumor with feeding vessels from both left and
right maxillary arteries (Figure 3) The protocol in this
case was preoperative angiography with selective arterial
embolization and complete endoscopic endonasal resection
of the tumor and its expansions.

Figure 3 Case No. 2: Pre-operative angiography revealing the tumor with vascular sources from both left and right
maxillary arteries.

Particular aspects in endoscopic surgery for juvenile nasopharyngeal angiofibromas. Case reports and review

For both cases, the tumors were examined in the

pathology compartment revealing areas with numerous
anastomotic blood vessels, with relatively thin walls,
teleangiectatic appearance and heavily developed fibrous
stroma. Also, immunohistochemical analysis showed
receptors for estrogen, progesterone and androgen
hormones in the stromal cells, CK7, CK8/18 positive in
surface epithelium, SMA, CD31, CD34 positive for the
blood vessels and S100 positive for the stromal cells
and nervous fibers. The histological diagnosis was nasopharyngeal angiofibroma.
Discussion
The highly vascular nature and the structure of JNA
with numerous blood vessels lacking muscular fibers
makes this particular tumor a never ending challenge as
it is for head and neck surgeons. Nowadays improvements
of endoscopic endonasal surgical techniques enable the
use of endoscopic surgery for complete removal of JNA
[1]. However, the decision to perform JNA resection in
an endoscopic approach is based not only on the extent
of the tumor but also on the experience and skill of the
surgical team [1, 4, 15, 24]. The cases presented were
especially problematic because of previous surgical
procedures and vascular abnormalities. The first patient
had previous surgery for JNA through an external
approach combined with external carotid ligation. Preoperative angiography revealed that the tumor received
vascular sources from the ipsilateral internal carotid artery.
For this reason, pre-operative selective embolization was
not possible being considered of great risk to the patient.
For safety reasons, in case of uncontrollable local bleeding,
the surgical team dissected and exposed the common
carotid artery for possible ligation, prior to endoscopic
resection of the tumor. Complete endoscopic surgical
removal of the tumor was achieved in this case, without
any complications. The second case was particularly
interesting because of multiple arterial sources from both
left and right maxillary arteries and because it extended
to the left pterygopalatine fossa and sphenoid sinus. The
patient underwent pre-operative angiography and selective
embolization of bilateral vascular sources of the tumor.
This allowed the surgical team to excise the mass and its
extensions in the pterygopalatine fossa and sphenoid
sinus using endonasal endoscopic techniques.
Endoscopic surgery for JNA has a great advantage by
improving visualization by obtaining magnified views of
the tumor from different angles and related anatomical
structures [1, 8, 18, 19]. Also, endoscopic surgery allows a
more accurate and complete dissection of the tumor,
minimizing local bleeding and the possibility of residual
tumor, as well as preserving the normal anatomy and
physiology of the nose and prevents facial growth
disturbance which occurred after classical osteotomies
[1, 8, 19, 2426]. It is the general opinion that preoperative embolization of the tumor about 48 hours
before surgery reduces blood loss and the risk of intraoperative hemorrhage [1, 8, 18]. However, Lloyd G et al.
consider that pre-operative embolization of JNA could
increase the risk of incomplete resection as a result to
reduced definition of tumor border, especially when there

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is deep invasion of the sphenoid bone [8, 27]. In case of

tumors that cannot be embolized, arterial ligations and
other haemostatic techniques could be used for control
of intra-operative or post-operative bleeding. The choice
for endoscopic surgery in removal of JNA should be
made after complete investigation and staging of the
tumor and according to the skill and experience of the
surgical team.
Conclusions
JNA is a highly vascular tumor with aggressive local
behavior that affects primarily adolescent males. It has a
high persistence and recurrence rate and represents a
challenge to head and neck surgeons. Various treatment
options have been emphasized for its treatment (surgery,
radiotherapy, chemotherapy, hormone therapy). The gold
standard treatment method in selected cases is endoscopic
surgery combined with pre-operative selective embolization
of the main blood vessels feeding the tumor, although
special cases such as recurrences and vascular variations
should be considered separately. Endoscopic endonasal
surgery in JNA is only limited by the extension of the
tumor in surrounding anatomical regions, and the experience
of the surgical team.
References
[1] Gara MF, Yuca SA, Yuca K, Juvenile nasopharyngeal
angiofibroma, Eur J Gen Med, 2010, 7(4):419425.
[2] Shahid G, Danyal R, Bashir A, Common presenting symptoms
diagnosis and management of angiofibroma a study of
twenty cases, Pak J Med Sci, 2004, 20(4):377380.
[3] de Andrade NA, de Andrade SC, Silva PDM, de Oliveira VB,
Andrade BB, Nasopharyngeal angiofibroma: review of the
genetic and molecular aspects, Int Arch Otorhinolaryngol
(Sao Paulo), 2008, 12(3):442449.
[4] Onerci M, Oretmenolu O, Ycel T, Juvenile nasopharyngeal
angiofibroma: a revised staging system, Rhinology, 2006,
44(1):3945.
[5] Beham A, Regauer S, Beham-Schmid C, Kainz J, Stammberger H,
Expression of CD34-antigen in nasopharyngeal angiofibromas,
Int J Pediatr Otorhinolaryngol, 1998, 44(3):245250.
[6] Beham A, Beham-Schmid C, Regauer S, Aubck L,
Stammberger H, Nasopharyngeal angiofibroma: true neoplasm
or vascular malformation? Adv Anat Pathol, 2007, 7(1):3646.
[7] Schick B, Plinkert PK, Prescher A, Aetiology of angiofibromas:
reflection on their specific vascular component, Laryngorhinootologie, 2002, 81(4):280284.
[8] Andrade NA, Pinto JA, Nbrega Mde O, Aguiar JE, Aguiar TF,
Vinhaes ES, Exclusively endoscopic surgery for juvenile
nasopharyngeal angiofibroma, Otolaryngol Head Neck Surg,
2007, 137(3):492496.
[9] Tang IP, Shashinder S, Gopala Krishnan G, Narayanan P,
Juvenile nasopharyngeal angiofibroma in a tertiary centre:
ten-year experience, Singapore Med J, 2009, 50(3):261264.
[10] Mendenhall WM, Werning JW, Hinerman RW, Amdur RJ,
Villaret DB, Juvenile nasopharyngeal angiofibroma, J HK
Coll Radiol, 2003, 6(1):1519.
[11] Gleeson M, Juvenile angiofibroma. In: Gleeson MJ,
Browning GG, Burton MJ, Clarke RC, Hibbert J, Jones NS,
Lund VJ, Luxon LM, Watkinson JC (eds), ScottBrowns
th
otorhinolaryngology, head and neck surgery, 7 edition, vol. 2,
Hodder Arnold, London, 2008, 24372444.
[12] Wenig BM, Angiofibroma (juvenile nasopharyngeal angiofibroma). In: Wenig BM (ed): Atlas of head and neck
nd
pathology, 2 edition, Elsevier, 2008.
[13] Twu CW, Hsu CY, Jiang RS, Surgical treatment of nasopharyngeal angiofibroma, Mid Taiwan J Med, 2002, 7(2):7175.
[14] Pryor SG, Moore EJ, Kasperbauer JL, Endoscopic versus
traditional approaches for excision of juvenile nasopharyngeal
angiofibroma, Laryngoscope, 2005, 115(7):12011207.

870

V. Budu et al.

[15] Ferreira LMB, Gomes EF, Azevedo JF, Souza JR, Arajo RP,
Rios AS, Endoscopic surgery of nasopharyngeal angiofibroma,
Rev Bras Otorrinolaringol, 2006, 72(4):475480.
[16] Lim IR, Pang YT, Soh K, Juvenile angiofibroma: case report
and the role of endoscopic resection, Singapore Med J, 2002,
43(4):208210.
[17] Mitskavich MT, Carrau RL, Snyderman CH, Weissman JL,
Fagan JJ, Intranasal endoscopic excision of a juvenile angiofibroma, Auris Nasus Larynx, 1998, 25(1):3944.
[18] Nicolai P, Berlucchi M, Tomenzoli D, Cappiello J, Trimarchi M,
Maroldi R, Battaglia G, Antonelli AR, Endoscopic surgery
for juvenile angiofibroma: when and how, Laryngoscope,
2003, 113(5):775782.
[19] Roger G, Tran Ba Huy P, Froehlich P, Van Den Abbeele T,
Klossek JM, Serrano E, Garabedian EN, Herman P,
Exclusively endoscopic removal of juvenile nasopharyngeal
angiofibroma: trends and limits, Arch Otolaryngol Head Neck
Surg, 2002, 128(8):928935. Erratum in Arch Otolaryngol
Head Neck Surg, 2003, 129(1):88.
[20] Sessions RB, Bryan RN, Naclerio RM, Alford BR, Radiographic
staging of juvenile angiofibroma, Head Neck Surg, 1981,
3(4):279283.

[21] Chandler JR, Goulding R, Moskowitz L, Quencer RM,

Nasopharyngeal angiofibromas: staging and management,
Ann Otol Rhinol Laryngol, 1984, 93(4 Pt 1):322329.
[22] Andrews JC, Fisch U, Valavanis A, Aeppli U, Makek MS,
The surgical management of extensive nasopharyngeal
angiofibromas with the infratemporal fossa approach,
Laryngoscope, 1989, 99(4):429437.
[23] Mann WJ, Jecker P, Amedee RG, Juvenile angiofibromas:
changing surgical concept over the last 20 years, Laryngoscope, 2004, 114(2):291293.
[24] Ahmed Z, Mutiullah S, Ur-Rahim D, Marfani MS, Juvenile nasopharyngeal angiofibroma: stage and surgical approach,
JLUMHS, 2009, 8(1):3740.
[25] Carrau RL, Snyderman CH, Kassam AB, Jungreis CA,
Endoscopic and endoscopic-assisted surgery for juvenile
angiofibroma, Laryngoscope, 2001, 111(3):483487.
[26] Wormald PJ, Van Hasselt A, Endoscoic removal of juvenile
angiofibromas, Otolaryngol Head Neck Surg, 2003, 129(6):
684691.
[27] Lloyd G, Howard D, Phelps P, Cheesman A, Juvenile angiofibroma: the lessons of 20 years of modern imaging, J Laryngol
Otol, 1999, 113(2):127134.

Corresponding author
Vlad Budu, MD, PhD, Department of ENT Microsurgery, Prof. Dr. Dorin Hociot Institute for Phono-Audiology
and Functional ENT Surgery, 21 Mihai Cioranu Street, 050751 Bucharest, Romania; Phone +4021410 21 70,
e-mail: vladbudu@yahoo.com

Received: March 20, 2013

Accepted: October 22, 2013