International Journal of Pediatric Otorhinolaryngology 77 (2013) 1186–1189

Juvenile nasopharyngeal angiofibroma: Timisoara ENT Department’s experience

Gheorghe Iovanescu *, Steluta Ruja, Stan Cotulbea

Department of Otolaryngology, ‘‘Victor Babes’’ University of Medicine and Pharmacy Timisoara, Romania

A R T I C L E I N F O

Article history:

Received 5 December 2012

Received in revised form 24 April 2013

Accepted 27 April 2013

Available online 2 June 2013

Keywords:

Juvenile nasopharyngeal angiofibroma

Unilateral nasal obstruction

Surgical technique

A B S T R A C T

Juvenile nasopharyngeal angiofibroma is a histologically benign, but very aggressive and destructive

tumor found exclusively in young males. The management of juvenile nasopharyngeal angiofibroma has

changed in recent years, but it still continues to be a challenge for the multidisciplinary head and neck

surgical team.

Objective: The purpose of this study was to review a series of 30 patients describing the treatment

approach used and studying the outcome of juvenile nasopharyngeal angiofibroma in the ENT

Department Timisoara, Romania for a period of 30 years.

Methods: The patients were diagnosed and treated during the years 1981–2011. All patients were male.

Tumors were classified using Radkowski’s staging system. Computed tomography and magnetic

resonance imaging allowed for accurate diagnosis and staging of the tumors. Biopsies were not

performed. Surgery represented the gold standard for treatment of juvenine nasopharyngeal

angiofibroma. All patients had the tumor removed by an external approach, endoscopic surgical

approach not being employed in this series of patients.

Results: All patients were treated surgically. Surgical techniques performed were: Denker–Rouge

technique in 13 cases (43.33%), paralateronasal technique in 7 cases (23.33%), retropalatine technique in

5 cases (16.66%) and transpalatine technique in 5 cases (16.66%). No preoperative tumor embolization

was performed. The recurrence rate was 16.66%. The follow-up period ranged from 1 year to 12 years.

Conclusions: Management of juvenile nasopharyngeal angiofibroma remains a surgical challenge.

Clinical evaluation and surgical experience are very important in selecting the proper approach. A

multidisciplinary team, with an experienced surgeon and good collaboration with the anesthesiologist

are needed for successful surgical treatment.

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International Journal of Pediatric Otorhinolaryngology

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1. Introduction

Juvenile nasopharyngeal angiofibroma (JNA) is a rare, histolog-ically benign, mesenchymal, vascular and highly cellular tumor.JNA accounts for 0.5% of all neoplasms of the head and neck and isconsidered to be the most common benign neoplasm of thenasopahrynx. Despite its histologically benign nature, it is locallydestructive and can cause bone remodeling. The tumor has thepotential of life-threatening complications, such as massiveepistaxis, intracranial extension and intraoperative massivehemorrhage. The tumor occurs almost exclusively in males inthe second decade. Typical symptoms for JNA are progressiveunilateral nasal obstruction (80–90%) with rhinorrhea andrecurrent unilateral epistaxis (45–60%); thus these complaints inan adolescent male should immediately generate suspicion.

* Corresponding author at: Str. Frantz Schmits, Nr. 9, Timisoara, Romania.

Tel.: +40 744206855; fax: +40 256474747.

E-mail addresses: giovanescu@gmail.com, giovanescu@hotmail.com

(G. Iovanescu).

0165-5876/$ – see front matter � 2013 Elsevier Ireland Ltd. All rights reserved.

http://dx.doi.org/10.1016/j.ijporl.2013.04.035

Headache (25%) and facial pain may arise secondarily to theblockage of paranasal sinuses, or impairment of Eustachian tubefunction with unilateral middle ear effusion, respectively. Tumorextension into the sinonasal cavity can cause chronic rhinosinusitis[1]. Proptosis and alteration of vision clearly indicate an involve-ment of the orbit. Swelling of the cheek, neurologic deficits,alteration in olfaction, rhinolalia clausa, and otalgia also may occur[2]. After intracranial invasion the tumor may involve cranialnerves II, III, IV and VI. Although several nonsurgical methods havebeen proposed, surgery is considered the treatment of choice forJNA. Radiation therapy is reserved for unresectable, life-threaten-ing tumors, chemotherapy is reserved for recurrent tumors withprevious surgery and radiation with hormone therapy – estrogensand antiandrogens used to decrease tumor size and vascularity.

2. Methods

Retrospective demographical, clinicopathological data, clinicalpresentation signs and symptoms, investigations as well astreatment of these patients who were diagnosed with a histologically

Table 1Age distribution.

Age Number of patients Percentage

11–12 years old 8 patients 26.66

13–14 years old 5 patients 16.66

15–16 years old 10 patients 33.3

16–17 years old 7 patients 23.3

Table 2Classification according to Radkowski.

Stage Patients Percentage

IA 14 cases 46.66

IB 9 cases 30.00

IIA 5 cases 16.66

IIB 1 case 3.33

IIC 1 case 3.33

2

2

1

Stage IIA Stage IIB Stage IIIA

Fig. 1. Recurrence distribution.

G. Iovanescu et al. / International Journal of Pediatric Otorhinolaryngology 77 (2013) 1186–1189 1187

confirmed JNA during 1981–2011 in ENT Department in Timisoarawere reviewed and collected from the medical records.

The hospital records were reviewed and collected regarding thepatient’s age, sex, signs and symptoms, date of diagnosis, tumorextension and stage, imaging, histopathological examination,treatment modality, surgical approach, rate of recurrences, dateand status at last follow-up visit.

All cases were operated in our department. A total of 30patients, all males, aged 11–18 years, were included. All patientswere treated surgically as a single modality of treatment. None ofour patients had undergone preoperative embolization. Allpatients had a minimum of 1 year follow up, up to 12 years.

Primary staging of all cases was based on CT scan or MRI.All patients were staged according to the Radkowski et al.classification.

IA: involvement limited to the nose and/or nasopharynx.IB: extension into one or more sinuses.IIA: minimal extension into the pterygopalatine fossa.IIB: tumor occupying the entire pterygopalatine fossa with orwithout erosion of the orbital apex.IIC: involvement of the infratemporal fossa with or withoutextension to the cheek or posterior to the pterygoid plates.IIIA: erosion of the skull base (the middle cranial fossa/base ofthe pterygoids); minimal intracranial extension.IIIB: erosion of the skull base; extensive intracranial extensionwith or without invasion of the cavernous sinus.

3. Results

All the patients were males. All the cases were referred fromprivate or government general practitioners. The average age atdiagnosis was 15 years, the age distribution of the 30 patientsdiagnosed with juvenile angiofibroma was: 8 patients (26.66%)were between 11 and 12 years of age, 5 patients (16.66%) between13 and 14 years old, 10 patients (33.3%) between 15 and 16 yearsold, and 7 patients (23.3%) between 17 and 18 years old (Table 1).

The most common presenting symptoms were progressivenasal obstruction in all cases (100%), rhinolalia in 15 cases (50%),massive and recurrent epistaxis in 22 cases (73.33%), combinednasal obstruction and epistaxis in 15 patients (50%), associated

Table 3Surgical techniques.

Surgical technique performed Number of patients

Denker–Rouge technique 13

Paralateronasal technique 7

Retropalatine technique 5

Transpalatine technique 5

symptoms including conductive hearing loss and middle eareffusion in 18 patients (60%), headache and rhinorrhea in 3 cases(10%) and facial swelling in 1 case (3.33%). 10 patients (33.3%)noted symptoms for less than 1 year.

Endoscopic examination of the nasal cavity and nasopharynxrevealed a tumor arising from the nasopharynx in all the patients.No biopsies were taken in any of these patients as JNA was theprimary provisional diagnosis.

All patients underwent contrast-enhanced CT, MRI or angio-MRI for the initial assessment, and were classified according toRadkowski system as stage IA 14 patients (46.66%), IB 9 patients(30%), IIA 5 patients (16.66%). IIB 1 patient (3.33%) and IIC 1 patient(3.33), respectively (Table 2).

In our study we found 5 recurrences (16.66%), 2 patients (6.66%)were in stage IIA, 2 patients (6.66%) in stage IIB and 1 patient(3.33%) (Fig. 1). Angiography performed in 12 cases revealed that 6tumors (50%) were supplied by both internal maxillary arteries, 5tumors (41.67%) received blood supply from the ipsilateral internalmaxillary artery and one tumor (8.33%) also received blood supplyfrom the internal carotid artery.

All patients were treated surgically. Surgical techniquesperformed were: Denker–Rouge technique in 13 cases (43.33%),paralateronasal technique in 7 cases (23.33%), retrovelopalatinetechnique in 5 cases (16.66%) and transpalatin technique in 5 cases(16.66%) (Table 3) (Fig. 2).

Percentage Stages Percentage

43.33 IA – 6 cases 20.00

IB – 2 cases 6.66

IIA – 5 cases 16.66

23.33 IA – 1 case 3.33

IB – 4 cases 13.33

IIB – 1 case 3.33

IIC – 1 case 3.33

16.66 IA – 3 cases 10.00

IB – 2 cases 6.66

16.66 IA – 4 cases 13.33

IB – 1 case 3.33

Fig. 2. F.N., 15 years old, stage IIC, paralateronasal technique; recurrence at 16 months postoperative.

G. Iovanescu et al. / International Journal of Pediatric Otorhinolaryngology 77 (2013) 1186–11891188

All 30 patients underwent primary surgical resection. Nopreoperative embolization was performed. There was no surgery-related mortality and no major complications.

The average duration of surgery was 120 min (ranging from 100to 360 min). The average blood loss was 1200 ml (range 500–2500 ml). The patients were transfused with a minimum of twounits and a maximum of five units, and an average of three units.Postoperatively, all patients were monitored in the intensive careunit for at least 2 days. Nasal packing remained in place for anaverage of 90 h (range 72–170 h).

Four out of 5 patients with recurrent tumors (80%) benefited bysurgical treatment, using the paralateronasal technique (Table 4).None of the recurrences was treated endoscopically. In the 1 caseout of 5 recurrences, JNA presented with intracranial extension orlesions incompletely resected and was effectively cured withexternal beam radiotherapy. Average time to recurrence was 12months (ranging from 6 to 12 months).

Table 4JNA recurrences.

Initial stage Patients Percentage

IB 1 case 3.33

IIA 2 cases 6.66

IIB 1 case 3.33

IIC 1 case 3.33

4. Discussion

The typical case presentation is usually of an adolescent malewith an average age of 17 years presenting with spontaneousrecurrent epistaxis, nasal obstruction and discharge. Other clinicalpresentations depend on the extent of the tumor [3,4].

JNA diagnosis is based on a precise clinical history andexamination of the patient including endoscopy and imaging(CT or MRI). The dominant symptom is persistent nasal obstructionand recurrent epistaxis. Biopsy should be avoided due to thepotential for massive bleeding.

Choosing the surgical approach is still debatable, but surgeryremains the treatment of choice, aiming for complete resection ofthe tumor. Transpalatal, transpharyngeal, Denker approach,medial maxillectomy, transfacial through lateral rhinotomy,midfacial degloving, and Le Fort I osteotomy, other thaninfratemporal and subtemporal lateral approaches are the

Recurrence stage Period of recurrence (months)

IA – paralateronasal technique 16

IB – paralateronasal technique 6

IIB – paralateronasal technique 12

IIIA – radiotherapy 12

IB – paralateronasal technique 16

G. Iovanescu et al. / International Journal of Pediatric Otorhinolaryngology 77 (2013) 1186–1189 1189

traditional surgical methods commonly used to remove JNA. In ourseries Denker–Rouge technique was the most commonly usedtechnique for primary surgery. Recurrences were operated throughparalateronasal technique.

The transpalatal approach is performed using a U shapedincision in the hard palate. This approach is associated with asignificant risk of recurrence due to inadequate exposure for largetumors and the most common complication is palatal fistula. Oneof our patients operated through a transpalatal approachpresented with recurrence at 16 months postoperatively. Thelateral rhinotomy approach offers exposure for removal ofextensive JNA. Midfacial degloving avoids facial scars whilemaintaining a wide surgical exposure. The transoral Le Fort Iosteotomy is the best approach for tumors invading the skull baseand orbit.

In recent times, selected cases of tumors expanding to thepterygopalatal fossa, infratemporal fossa and even limitedintracranial involvement have been managed endoscopically [5–8]. In general, the endoscopic approach is limited and is notrecommended for tumors with large intracranial extensions orextensions laterally to the cavernous sinus [9–12]. The endoscopicapproach has a low morbidity, low rate of complications, and shorthospitalization [13].

Many authors have described the importance of exploring thebasisphenoid in order to remove the tumor extension at the skullbase [14–17] which can be better achieved with endoscopicresection. The combination of an open technique with theendoscopic approach allows accurate inspection of the nasalcavity and may reduce tumor recurrence [18]. When using theendoscopic approach, preoperative embolization is essential forhemostasis and good visualization [10–12,19]. Some centers useimaging guided navigation for safety reasons. Another usefuldissection system allowing effective endoscopic tumor removal isthe Cavitron ultrasonic surgical aspirator. Preoperative use ofintratumoral injections of cyanoacrylate glue has been suggestedto help dissection of JNA [20]. In our center the preoperativeembolization is not available, the external surgical approach beingthe treatment of choice.

Our recurrences were found in 5 patients (16.66%). Radkowskiet al. reported a 22% recurrence rate among 23 cases. Mostrecurrences occurred in stage IIB and were subjected to reopera-tion and in 3 cases to external beam radiotherapy. Understaging isone possible explanation for this result. JNA recurrence has to beconsidered, when clinical and radiological tumor suspicioncorrelates with clinical symptoms during follow up. In our series,recurrent tumor was encountered on average 12 monthspostoperatively.

Recurrence is a conspicuous feature of the natural history ofJNA. Recurrence rates as high as 30–50% have been reported byMcCombe et al. [21] and Enepekides [22]. Harma reported a 46%recurrence in a cohort of 49 patients, 28% of which were multiplerecurrences [23]. In 1992, Gullane et al. reported a 36% recurrenceafter surgery in a series of 14 patients [24].

5. Conclusions

Management of JNA is still challenging. JNA is a rare, vascular,benign tumor with high exclusivity, persistence and recurrence,and typically affects adolescent boys. The management of JNApresents a challenge to ENT surgeons. The most frequentrhinopharyngeal benign tumor, JNA presents an importantcapacity for local invasion with a bad prognosis in case ofinadequate treatment. Clinical evaluation and surgical experience

are very important in the selection of the proper surgical approach.An external surgical approach offers excellent tumor control a lowrecurrent rate. A multidisciplinary team, with an experiencedsurgeon and a good collaboration with the anesthesiologist areneeded for the treatment of JNA. Radiation therapy should bereserved for inoperable or incompletely resected tumors.

Authors’ contributions

Gheorghe Iovanescu and Stan Cotulbeais are responsible for theconception and design of the study, or acquisition of data, analysisand interpretation of data, drafting the article, revising it criticallyfor important intellectual content, and final approval of the versionto be submitted.

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