Mehmet Atalar, MD, Orhan Solak, MD, Suphi Müderris MD KBB-Forum

Juvenile Nasopharyngeal Angiofibroma: Radiologic evaluation and Pre-operative embolization 2006;5(1)

www.KBB-Forum.net

CASE REPORT

JUVENILE NASOPHARYNGEAL ANGIOFIBROMA: RADIOLOGIC

EVALUATION AND PRE-OPERATIVE EMBOLIZATION
Mehmet ATALAR, MD1, Orhan SOLAK, MD1, Suphi MÜDERRİS MD2
1
Cumhuriyet University Faculty of Medicine, Department of Radiology, Sivas, Turkey
2
Cumhuriyet University Faculty of Medicine, Department of Otorhinolaryngology, Sivas, Turkey

SUMMARY
Juvenile nasopharyngeal angiofibroma (JNA) constitutes less than 0.5% of all head and neck neoplasms. It is a histopathologically
benign, yet locally agressive, vascular tumor that occurs most frequently in males from 5 to 25 years. Advances in imaging and treatment
techniques are now facilitated more accurate staging of this disease. In this study, we present a case of JNA, to whom pre-operative
embolization is administered.

Keywords: Nasopharynx; angiofibroma; imaging; embolization

JÜVENİL NAZOFARENGEAL ANJİYOFİBROM: RADYOLOJİK DEĞERLENDİRME VE PRE-OPERATİF

EMBOLİZASYON

ÖZET
Jüvenil nazofarengeal anjiyofibrom (JNA), tüm baş-boyun tümörlerinin %0.5’den daha azını oluşturmaktadır. Histopatolojik olarak
benign olmasına rağmen lokal olarak agresif tümörler olup en sıklıkla 5–25 yaşları arası erkeklerde görülür. Görüntüleme ve tedavi
tekniklerinde ki ilerlemeler, daha doğru evrelendirmesine yardım etmektedir. Bu yazıda, pre-operatif embolizasyon uygulanan JNA’lı bir
olguyu sunuyoruz.

Anahtar Sözcükler: Nazofarenks; anjiyofibrom; görüntüleme; embolizasyon

INTRODUCTION Other physical and laboratory examination

JNA, as its suggests, is a combined vascular findings were normal. To elucidate the underlying
and fibrous neoplasm arising in the nasopharynx of pathologic abnormality, CT images at 2- and 4-mm
prepubertal and adolescent males. The tumor exhibits increments obtained in the axial and coronal planes
a strong tendency to bleed and, despite being before and after contrast medium injection. CT scan
microscopically benign, frequently exhibits confirmed a well-defined mass in the right post-nasal
1
destructive and agressive behaviour. In diagnostics, space, projecting into right sphenoid sinus and
besides epypharyngoscopy, computed tomography nasopharynx, with strong contrast enhancement (Fig.
(CT), magnetic resonance imaging (MRI) are the 1). Minimal bone erosion and remodeling were noted
primary diagnostic tests.2 Angiography is used for at the level of the skull base, suggesting that the
pre-operative embolization. In this case report, we lesion was a slow growing tumor. The MRI protocol
aimed to demonstrate a case of JNA, to whom pre- included axial, sagittal and coronal planes in T1-
operative embolization is administered, associated (repetition time [TR] / echo time [TE] =
with CT, MRI and angiography. 440msec/15msec) and T2- (TR/TE =
4000msec/100msec) weighted sequences. Gd-DTPA
CASE was used as a contrast agent at MRI. The lesion was
A 17-year-old boy, admitting to the hospital characterized as a contrast enhancing mass within the
with intermittent right-sided epistaxis of spontaneous nasopharynx on MRI (Fig. 2). Given these findings,
onset and vertigo, was examined with CT, MRI, and the differential diagnoses included the tumors of the
conventional catheter angiography. On examination vascular origin.
the patient had a large fleshy mass completely filling In our case, angiographic examination and
the nasopharynx. There were no associated otologic embolization were performed during the same
complaints, generalized bleeding tendency, or loss of session. Superselective angiography demonstrated the
appetite or weight. Cranial nerves were all intact. feeding vessels from the right internal maxillary
Corresponding Author: Mehmet Atalar MD; Cumhuriyet artery (IMA), and a smaller feeding vessel from the
Üniversitesi, Radyoloji anabilim dalı, Sivas, Türkiye, Telephone: +90 346 right internal carotid system. The embolization was
2191300 E-mail: mehmet5896@yahoo.com
performed under fluoroscopic control using digital
Received: 19 October 2005, revised for: 19 December 2005, substraction angiography (DSA), which gives an
accepted for publication: 20 December 2005
excellent image quality. Embolization of the feeding

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Mehmet Atalar, MD, Orhan Solak, MD, Suphi Müderris MD KBB-Forum
Juvenile Nasopharyngeal Angiofibroma: Radiologic evaluation and Pre-operative embolization 2006;5(1)
www.KBB-Forum.net

vessel from the external carotid system using

polyvinyl alcohol (PVA) particles 500–710 µm
(Contour embolization particles, Boston Scientific,
USA) in size for good tumor penetration and three
fibered platinum coils (4mm/20mm Target
Therapeutics, Boston Scientific, USA) were
performed, without complication, in order to reduce
bleeding at the time of operation. Post-embolization
angiogram revealed significant tumor blush
reduction. There was residual blood flow from the
branches of the right ophtalmic artery. Branches
arising from the internal carotid or ophthalmic
arteries were not embolized. Figure–3 shows the 3a
arteriogram before (Fig. 3a) and after (Fig. 3b)
embolization of the feeding vessels. The patient
underwent resection of tumor through a right lateral
rhinotomy and partial medial maxillectomy on the
next day. The histopathologic features were
consistent with an angiofibroma. The patient was
followed with 1-month interval for the first 6-month;
and then with 3-month interval for two years; and 1-
year interval thereafter. The patient was followed
over 2 years without recurrence or residual tumor.

3b
Figure 3. Pre-operative carotid angiogram. (a) Pre-embolization
carotid angiogram revealing a vascular blush in the right nasal
fossa. The vessel feeding the tumor is the internal maxillary
artery, (b) Post-embolization angiogram shows significant blush
reduction.

DISCUSSION
Hippocrates described the tumor in the 5th
century BC, but Friedberg first used the term
Figure 1. Contrast-enhanced axial CT image: Tumor extends angiofibroma in 1940.3 Angiofibromas are
from the right pterygopalatine fossa into nasopharynx and histopathologically benign but potentially locally
continuing into sphenoid sinus on the right side. destructive vascular tumors. They are unencapsulated
neoplasms composed of a rich vascular network
within a fibrous stroma.4 Angiofibromas originate
predominantly in the posterior-lateral wall of the
nasopharynx. Angiofibromas arise typically in the
nasopharynx, specifically at the trifurcation of the
sphenoidal process of the palatine bone, the
horizontal ala of the vomer, and the roof of the
pterygoid process. These vascular tumors expand
commonly beyond the nasopharynx into the cranium,
nose, and paranasal sinuses.4,5 JNAs are age- and sex-
linked. Classically, they are confined to boys in
adolescence and early adulthood.6 JNA accounts for
less than 0.5% of all benign lesions that originate in
the nasopharynx. JNA is an uncommon tumor, with
Figure 2. MRI scan: Gd-enhanced T1-weighted SE coronal
image demonstrates a large, intensely enhancing nasal mass reported incidence between 1 in 5000 and 1 in 60,000
extending into the nasopharynx. otolaryngology patients. Despite this low incidence,

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Mehmet Atalar, MD, Orhan Solak, MD, Suphi Müderris MD KBB-Forum
Juvenile Nasopharyngeal Angiofibroma: Radiologic evaluation and Pre-operative embolization 2006;5(1)
www.KBB-Forum.net

JNA is the most common benign tumor originating in most patients. In our case, the lesion was mainly fed
the nasopharynx of young males. The etiology of by a branch of the IMA. Surgical resection of JNAs is
JNA is unknown but it is postulated that there may be difficult because of abundant tumor vasculature;
a relation to sex hormones. There is evidence of moreover, incomplete resection not infrequently
increased androgen receptors of tumors and results in tumor recurrence.10 Preoperatively tumor
successful tumor regression after anti-androgen embolization can greatly facilitate surgery. In JNA,
therapy.7 superselective embolization of feeding vessels arising
from the external carotid artery is highly effective
Diagnostic imaging should be performed
and safe. Anastomosis between branches of the
prior to an invasive procedure since it may suggest
external and internal carotid artery and vascular
the diagnosis, thereby reducing the risk of
spasm have to be considered when planning
catastrophic hemorrhage associated with biopsy.
superselective embolization. Several materials have
In a study of 72 patients, Lloyd et al.7 been tried over the years, including non-absorbable
reported three findings on CT and MR imaging that silastic spheres, Gelfoam, dura mater, and polyvinyl
should suggest a diagnosis of JNA: alcohol particles.11 Recently, non-absorbable
1. a soft tissue mass in the nasopharynx or nasal polyvinyl alcohol particles are preferred.12 Migration
cavity, of embolization particles to the ophthalmic artery, the
cerebral or vertebral arteries via anastomosis or
2. a mass in the pterygopalatine fossa, reflux of particles applied to the external carotid
3. erosion of posterior osseous margin of the artery may cause severe ischemic deficits. In our
sphenopalatine foramen extending to the base of case, the IMA was embolized with 500–710 µm PVA
the medial pterygoid plate. In our case, the particles followed by three platinum coils proximally
lesion was located in the nasopharynx, and to prevent recanalization.
projected into the right sphenoid sinus. In JNA cases, spontaneous regression is rare.
Angiofibroma can be diagnosed using CT, Recurrence rates ranged from 6% to 60%.
MR imaging and angiography. CT is a most Recurrence can occur as early as 3–4 months after
important pre-operative test because it is useful for surgery.13
showing the destruction of bony structures and The differential diagnosis of JNA includes
widening of foramen and fissures at the skull base antrochoanal polyp, inflammatory sinonasal polyp,
due to spread of tumor. CT can also help recognize neurofibroma, adenoidal hypertrophy, naso-
invasion of sphenoid and thus determine the pharyngeal cyst, pyogenic granuloma, chordoma, and
aggressiveness of surgery. On CT, bone involvement malignant neoplasms, such as nasopharyngeal
and tumoral spread can be seen best on thin-section carcinoma, lymphoma, or rhabdomyosarcoma.3,13 CT
axial or coronal images. MRI is useful to show and MR imaging of nasopharyngeal carcinoma show
presence of intracranial extension of the tumor. MRI an inhomogeneous mass arising from the
also helps discern between sinus invasion, nasopharyngeal mucosa or submucosal space with
obstruction and retention of secretions. On MR skull-base erosion or intracranial extension.
imaging, JNA appears as a heterogenous mass with Lymphoma may arise from adenoidal tissues of the
signal voids (representing hypertrophic tumor nasopharynx or Waldeyer’s ring and can be
vessels) that are consistent with the highly vascular associated with lymphadenopathy. Imaging of
tumor; intense enhancement with gadopentetate rhabdomyosarcoma reveals a soft-tissue mass with
contrast material is typical.3,4 In this case, bone frequent bone destruction and mild enhancement on
involvement is demonstrated best on CT. Selective CT, but marked enhancement on MR imaging.14
angiography identifies the feeding vessels and allows
the option of pre-operative embolization for vascular In conclusion, JNA should be suspected in a
control.8 This imaging method shows the size and site boy or adolescent male with a history of chronic
of the lesion as well as size and location of the nasal obstruction or recurrent epistaxis without
feeding vessel. The arterial supply to JNAs is history of trauma and a soft tissue mass in the nose or
primarly from distal IMA branches, commonly the nasopharynx. Preoperative angiography is mandatory
sphenopalatine, descending palatine, and posterior for identification of the feeding vessels in view of
superior alveolar branches. The ascending pharyngeal preoperative embolization before surgery in all cases.
artery not infrequently supplies JNAs.9 As it grows, REFERENCES
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Juvenile Nasopharyngeal Angiofibroma: Radiologic evaluation and Pre-operative embolization 2006;5(1)
www.KBB-Forum.net

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