International Journal of Pediatric Otorhinolaryngology

58 (2001) 163166

Case report

Extranasopharyngeal angiofibroma arising from the nasalseptum

K.K Handa a,*, Abhinit Kumar a, M.K. Singh b, Aru H. Chhabra a

a Department of Otorhinolaryngology, All India Institute of Medical Sciences, New Delhi, 110029 Indiab Department of Pathology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, 110029 India

Received 24 August 2000; received in revised form 3 November 2000; accepted 4 November 2000

Abstract

Extra nasopharyngeal origin of angiofibroma is very rare. The nasal septum is a very rare site of extranasopharyngeal angiofibroma with only two cases reported in the medical literature. We report here a case of avascular mass arising from the nasal septum of an 8 year old boy. Histopathology confirmed it to be a case ofangiofibroma. A review is also made of the other reported cases of angiofibroma arising from the nasal cavity. Thelikely theory of origin of the tumor and the management is also discussed. 2001 Published by Elsevier ScienceIreland Ltd.

Keywords: Angiofibroma; Extra nasopharyngeal; Child; Septum

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1. Introduction

Angiofibroma arising outside the nasopharynxis very rare. Our search of the medical literaturerevealed only 46 cases to date of extra-nasopha-ryngeal angiofibroma [13]. Of these the maxil-lary sinus is the commonest site with 15 casesbeen reported so far. The nasal septum is anextremely rare site and to date only two cases ofextra-nasopharyngeal angiofibroma arising in thenasal septum have been reported [1,4]. We report

here the third case of angiofibroma arising fromthe nasal septum. This is being presented becauseof its unusual origin and clinical presentation.

2. Case report

A 8 year old boy presented to the out patientDepartment of the All India Institute of MedicalSciences, New Delhi with a history of recurrentepistaxis and obstruction of the left nostril overthe previous 6 months. Epistaxis was moderate inamount and was spontaneous. It required anteriornasal packing twice. He did not have any historyof bleeding disorder.

* Corresponding author. Fax: 91-11-6859812.E-mail address: handak@chequemail.com (K.K. Handa).

0165-5876:01:$ - see front matter 2001 Published by Elsevier Science Ireland Ltd.

PII: S0165 -5876 (00 )00460 -2

K.K. Handa et al. : Int. J. Pediatr. Otorhinolaryngol. 58 (2001) 163166164

Fig. 1. CT with contrast (axial cut) shows the contrast enhanc-ing mass arising from the nasal septum.

Fig. 3. (H&E, 240 ) Numerous vascular spaces lined byendothelium and separated by stroma.

ative diagnosis of a vascular tumor, most likelyhemangioma, was made.

Under general anesthesia. Initially an endo-scopic removal was attempted but this was aban-doned due to excessive bleeding. Next an externalapproach via left alar crease incision was madefor better access to the anterior nasal cavity. Thetumor was found firmly adherent with the nasalseptum at the junction of the quadrangular carti-lage and the bony septum. The muco-perichon-drium could not be separated from the tumor.The tumor was removed with a part of the under-lying septum. However the opposite side mu-coperichondrium was intact. Total blood loss wasabout 100 ml during the procedure. The bleedingwas controlled with electrocautery. Anterior nasalpacking was done with gauze soaked withNeosporin. The pack was removed after 48 h.Post operative recovery was uneventful.

Histopathology report showed dilated, cav-ernous vascular spaces lined by endothelial cellsand separated by fibrostroma with stromal cellnuclei. These findings were compatible with adiagnosis of angiofibroma (Fig. 3).

There has not been any recurrence in the 6months post operative follow up.

3. Discussion

Extra nasopharyngeal angiofibroma is rare. Asearch of the medical literature revealed that to

On examination a spatula test showed blockadeof the left nostril. Anterior rhinoscopy showed asmooth reddish mass present in the left nostril,between the nasal septum and the inferior tur-binate. As it appeared to be a vascular mass itwas not probed. No mass was seen on posteriorrhinoscopy. A contrast enhanced CT scan of thenose and the paranasal sinuses showed a contrastenhancing mass arising from the nasal septum andfilling the nasal cavity ( Figs. 1 and 2). There wasno extension beyond the nasal cavity into thenasopharynx or any other paranasal sinuses. Theresults of blood, urine examination and X-rayexamination of the chest were within normal lim-its. On the basis of the above findings a pre-oper-

Fig. 2. CT with contrast (coronal cut) showing the contrastenhancing mass in the nasal cavity.

K.K. Handa et al. : Int. J. Pediatr. Otorhinolaryngol. 58 (2001) 163166 165

date only 46 cases have been reported of whichonly two cases have been reported as arising fromthe nasal septum (Table 1), [1,4]. The other com-mon sites of extra nasopharyngeal angiofibromawere ethmoid sinus (five cases), pterygo maxillaryfissure, infratemporal fossa and cheek (four cases)and laryngo-tracheal tree (three cases). The com-monest age of incidence was the second decade(46%) in comparison to 80% of patients withnasopharyngeal angiofibroma [5]. Extra nasopha-ryngeal angiofibromas have a higher mean age,i.e. 22 years as compared to the nasopharyngealangiofibromas which have a mean age of 17 years[6]. The male to female ratio was 2.75:1. There isa higher incidence of extra-nasopharyngeal an-giofibroma in females [5]. This is much higherthan the rare incidence of nasopharyngeal angiofi-bromas in females reported in any series [7,8]. Thecharacteristic age and sex distribution of extranasopharyngeal angiofibromas is not likenasopharyngeal angiofibromas. This fact has pro-voked doubt as to whether extra nasopharyngealangiofibromas, though structurally similar, can bethought of as being different from nasopharyn-geal angiofibromas [9].

Many theories have been given as to the originof the nasopharyngeal angiofibroma. Tillaux [10]described the tumor originating in the tissue (latercalled fibrocartilago basilaris) extending to thelower surface of the sphenoid bone from theanterior margin of the atlas. Brunner [11] used theterm fascia for the fibrocartilago fascia as therewas no cartilage in it. According to him this fasciaextended from the posterior pharyngeal wall overthe roof of the nasopharynx to the vomer, thepalate bone, the posterior ethmoid and to themedial plate of the pterygoid process. There is no

fascia basalis in the nasal septum except in theposterior portion of the vomer and the ethmoidbone. In our case like the case reported by Sarpaand Novelly [1] the tumor arose from the junctionof the bony and the cartilaginous septum. Herethe origin of the angiofibroma is probably fromthe periosteum of the perpendicular plate of theethmoid which is likely to represent an abnormalanterior extension of the fascia basalis. Anotherreason for this angiofibroma arising in the septummay be related to developmental anomalies. It ispossible that turbinate like vascular tissue is re-tained as an ectopic nidus in developing perios-teum during the growth of the nasal septum [12].

The first case arising from the nasal septum [1]was reported in a 9 year old boy. When thepatient was taken up for surgery simple manipula-tion resulted in brisk bleeding from the polyp.Surgery was abandoned and post operative CTscan and angiography was done. CT showed themass to be arising from the cartilaginous septum.The patient was again taken up for surgery undergeneral anesthesia. A frozen section indicated themass to be juvenile angiofibroma. The mass wasexcised at the base of the stalk by electrocoagula-tion. The second case was reported in a 9 year oldJapanese male [4]. Pre-operatively the mass ap-peared to be vascular. A biopsy was taken underlocal anesthesia. Microscopical examination re-vealed features of angiofibroma. The tumour wasremoved under general anesthesia per nasally.

Extra nasopharyngeal angiofibroma arising inthe nasal cavity may by accessed endonasally, i.eendoscopically or otherwise, lateral rhinotomy orby an alar crease incision as in our case. Weinitially attempted an endoscopic removal but hadto abandon it due to excessive bleeding. As our

Table 1Reported cases of extranasopharyngeal angiofibromas arising in the asal cavity

Author Sex Site of originAgeYear

12 Nasal vaultObiako and Jacobs [14] M1983Hiraide and Matsubara [4] 1984 M Nasal septum13

9 Nasal septumMSarpa and Novelly [1] 1995F691995 Nasal vaultSheldon et al. [13]

1997 Inferior turbinateGaffney et al. [15] M9Nasal septumM81999Present case

K.K. Handa et al. : Int. J. Pediatr. Otorhinolaryngol. 58 (2001) 163166166

tumor was located anteriorly we had good accessvia the alar crease incision.

On histopathology an angiofibroma classicallyshows vascular spaces lined by endothelial cellsand separated by dense fibrous stroma. Hersh etal. [13] reported a case of vascular tumor arisingin the nasal vault of a 69 year old female. Thistumor, apart from the classical features of angiofi-broma, also had a number of vessels encircled bythickened layers of smooth muscle. Discrete bun-dles of smooth muscle were noted to be present inthe fibrous stroma. They labelled it angiomy-ofibroma. Hence an extra nasopharyngeal angiofi-broma has to be kept in mind in the differentialdiagnosis of a vascular mass arising from thenasal septum.

Acknowledgements

The authors wish to thank Professor R.C.Deka, Head of ENT Department, for providingencouragement and giving permission to write thispaper.

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