Egyptian Journal of Ear, Nose, Throat and Allied Sciences (2013) 14, 41–44

Egyptian Society of Ear, Nose, Throat and Allied Sciences

Egyptian Journal of Ear, Nose, Throat and Allied

Sciences

www.ejentas.com

CASE REPORT

Adenoid cystic carcinoma of external auditory canal: A

case report

Safinaz Zainor *, Hamidah Mamat, Sakina Mohd. Saad,

Mohd. Razif Mohamad Yunus

Department of Otorhinolaryngology, Hospital Sultan Abdul Halim, Sungai Petani, Kedah, MalaysiaDepartment of Otorhinolaryngology, Faculty of Medicine, Universiti Kebangsaan Malaysia Medical Centre,

Kuala Lumpur, Malaysia

Received 4 September 2012; accepted 2 November 2012Available online 12 January 2013

*

H

A

45E

Pe

Th

20

ht

KEYWORDS

Adenoid cystic carcinoma;

External auditory canal;

Cylindroma

Corresponding author at:

ospital Sultan Abdul Halim

manjaya, 08000 Sungai Peta

70344; fax: +60 04 4480106-mail address: safinaz329@y

er review under responsibili

roat and Allied Sciences.

Production an

90-0740 ª 2012 Egyptian So

tp://dx.doi.org/10.1016/j.ejen

Departm

, Jalan

ni, Keda

.ahoo.com

ty of Eg

d hostin

ciety of E

ta.2012.1

Abstract Primary malignancies of the external auditory canal (EAC) are extremely rare with more

than 80% being squamous cell carcinomas and adenoid cystic carcinoma (ACC) accounting for

approximately 5%. These tumours are associated with a high risk of recurrence and significant mor-

bidities from surgical management and adjuvant radiotherapy. Therefore, we present a case of

young female with right EAC mass diagnosed as cylindroma and confirmed postoperatively to

be ACC of the EAC which is a rare case in the literature.ª 2012 Egyptian Society of Ear, Nose, Throat and Allied Sciences.

Production and hosting by Elsevier B.V. All rights reserved.

1. Introduction

Adenoid cystic carcinoma (ACC) is a rare epithelial tumour

entity and comprises about 1% of all malignant tumours ofthe oral and maxillofacial region.1 ACC, first described as‘‘cylindroma’’ by Billroth,2 is commonly classified with the sal-

ent of Otorhinolaryngology,

Lencongan Timur, Bandar

h, Malaysia. Tel.: +60 016

(S. Zainor).

yptian Society of Ear, Nose,

g by Elsevier

ar, Nose, Throat and Allied Scien

1.001

ivary gland tumours, although it may arise in any site wheremucous glands exist. Half of these tumours occur in glandularareas other than the major salivary glands, principally in the

hard palate, but they also arise in the tongue and in other areasthat are the sites of minor salivary glands.3,4 Unusual locationsinclude the external auditory canal, nasopharynx, lacrimal

glands, breast, vulva, oesophagus, cervix, and Cowper glands.The long natural history of this tumour, its propensity for

perineural invasion, and its tendency for local recurrence are

well known.4 Although it presents a widespread age distribu-tion, peak incidence occurs predominantly among women, be-tween the 5th and 6th decades of life.5 It is a slow growing buthighly invasive cancer with a high recurrence rate. Lymphatic

spread to the local lymph nodes is rare. Haematogenousspread, however, occurs often in the course of the disease.6

Perineural spread of ACC has long been recognized. The liter-

ature revealed the region of Gasserian ganglion to be the mostcommon site of involvement (35.8%).7–9 Microscopically, it is

ces. Production and hosting by Elsevier B.V. All rights reserved.

Figure 1 The picture shows the tumour protruding out from

external auditory canal.

42 S. Zainor et al.

composed of basaloid cells with primarily myoepithelial/basalcell differentiation. Cribriform, tubular and solid are the three

recognized morphologic patterns. One of the important prog-nostic factors is the histological grade determined by the per-centage of solid component in the tumour. We present a case

of young female with right external auditory canal (EAC) massdiagnosed as cylindroma and confirmed postoperatively to beACC of the EAC which is a rare case in the literature.

2. Case report

A 28 years old Malay female attended to our otorhinolaryn-

gology clinic with a 6 month history of right ear mass that pro-gressively increased in size till it blocked the right external earcanal. It was associated with intermittent otalgia, purulent eardischarge and contact bleeding from the swelling. However, no

fever, headache, nausea, vomiting, vertigo or giddiness wereobserved. She also did not notice any facial asymmetry, neckswelling, loss of appetite or loss of weight.

Examination revealed a mass protruding from the rightexternal auditory canal (Fig. 1). It was non tender, non ery-thematous and non fluctuating. No discharge noted coming

out from the right external auditory canal. The right tympanicmembrane could not be visualized as it was obscured by themass. Left ear, nose and throat examination was unremark-able. No cervical lymph node palpable.

Initially, the patient presented in November 2008 andbiopsy was taken and the histopathological examination wassuggestive of a skin adnexal tumour, possibly of a cylindroma.

High Resolution Computed Tomography (HRCT) petrousbone showed an ill-defined heterogeneously enhancing massoccupying predominantly the external acoustic canal of the

right ear. It measures 1.7 cm · 2.9 cm and extends medially tillthe right middle ear cavity. Laterally, it was protruding outsidethe right ear pinna. Superiorly, it invades into the right mas-

toid air cell with partial destruction of the septa. There is abreak of the floor of the right temporal bone. The impressionat that time is highly suggestive of right cholesteatoma withdestruction of the right mastoid air cell and break of the floor

of the right temporal bone.

The patient then defaulted on the treatment till June 2011when she claimed that the swelling increased in size with inter-mittent pus discharge, but with no postauricular swelling or

pain. No nausea or vomiting, however headache was predom-inant. On examination there was protruding mass from theright external auditory canal that increased in size as compared

to before. Repeated HRCT temporal bone (Fig. 2) showed ill-defined enhancing mass arising from the right pinna was nowlarger measuring 3.7 cm · 1.9 cm that occupies the entire right

EAC into the right middle ear cavity. Superiorly, it invadesinto the right mastoid air cell with more destruction of the sep-ta. There was more break of the floor of the right temporalbone. The right incudomalleus complex eroded and had no evi-

dence of intracranial extension.The right modified radical mastoidectomy was performed

on 15/8/2011. Intraoperative findings were large EAC granu-

loma with stalks arising from anteroinferior and part of theposterior wall of EAC. The ossicles were surrounded by thegranulation tissue which also filled the middle ear. As there

was no facial nerve monitoring in our centre, the facial nervewas not properly traced and identified. Grossly, facial canalseems to be intact. No pus or keratin noted. Mastoid cavity

looks sclerosed.Postoperatively, the patient has immediate facial nerve

palsy (House Brackman Grade III) due to intraoperative in-jury. Histopathological examination confirmed it as adenoid

cystic carcinoma. Magnetic resonance imaging (MRI) of theinternal auditory meatus and brain (Fig. 3) was performedand showed features that represent tumour residual with local

extension, however no obvious cerebellopontine angle or brainparenchymal lesion. Then, the patient was referred to KualaLumpur General Hospital (tertiary hospital) for further man-

agement. Tumour debulking surgery was performed. Intraop-erative findings were tumour mass occupying mastoid antrumand middle ear adhering to the tympanic membrane and also

extending into the Eustachian tube. The patient was then re-ferred to oncology for their expert opinion and managementand completed her radiotherapy session there.

3. Discussion

Malignant tumours of the EAC are rare and most are squa-mous cell carcinomas. ACC arising in the EAC is exceedingly

rare. Although ACC is a rare EAC tumour, it is relatively com-mon in the salivary glands of the head and neck. ACC growthrate is slow and the nature of this carcinoma shows a slow

malignant course. The true origin of ACC in the EAC is con-troversial. It has been proposed that these tumours arise fromthe ceruminous glands. Microscopic studies of these tumours

and ceruminous glands demonstrate similar histologic features.Some authors have suggested that these tumours arise from theectopic salivary glands of the EAC, although this opinion hasnot been proved. In general, ceruminous gland tumours can be

classified as ceruminous adenoma, pleomorphic adenoma,ceruminous adenocarcinoma and adenoid cystic carcinoma.ACC has 3 main histological patterns: tubular, cribriform

and solid.10,11 In the salivary glands, the prognosis of ACCcorrelates with the predominant histological pattern. TubularACC has the best prognosis, whereas solid ACC has the worst

prognosis.11,12 However, in the EAC there was no significantcorrelation between these histological patterns.

Figure 2 The HRCT temporal bone for this patient shows the tumour mass that invades the right mastoid air cell right incudomalleus

complex eroded.

Figure 3 The axial and coronal view of MRI postoperative period showing the tumour residual.

Adenoid cystic carcinoma of external auditory canal: A case report 43

Some factors including tumour positivity at surgical mar-

gins, parotid gland and adjacent bone involvement, perineuralinvasion and local recurrences are associated with aggressive-ness and high mortality rate. When relapse develops, it gener-

ally occurs within two years. Late relapses were rarely reportedas much as 14 years. In some of the cases, pulmonary metasta-sis was detected 20 years after treatment; this is the longest per-

iod for metastases in the literature for adenoid cysticcarcinoma.13 Metastases into the lung are more common thanregional lymph node metastases. Rarely widespread visceral

involvement may be seen.The performance status of the patient and metastasis are

important factors for selecting the treatment modality. Thesurvival with surgery and postoperative radiotherapy is better

than that with surgery alone. The presence of tumour free sur-gical margins can be correlated with a better local control andalso with longer survival.14

Treatment consisted of radical excision of the EAC via a

modified temporal bone resection.15 In tumours which werethought to be aggressive (invaded to the adjacent tissue, highergrade or impossible to get negative surgical margins) elective

neck lymph node dissection could be added to surgery. Radio-therapy and chemotherapy are not curative but can help in pal-liation and as adjuvant therapy.16

References

1. Kokemuller H, Eckardt A, Brachvogel P, Hauseman JE. Adenoid

cystic carcinoma of the head and neck – a 20 years experience. Int

J Oral Maxillofac Surg. 2004;33:25–31.

2. Billroth T. Beobachtungen Uber Geschwulste der Speicheldrusen.

Arch Path Anat. 1859;17:357–375.

3. Berdal P. Cylindroma of salivary glands: a report of 80 cases. Acta

Otolaryngol. 1970;263:170–173.

44 S. Zainor et al.

4. Spiro RH. Salivary neoplasms: overview of a 35 year experience

with 2 807 patients. Head Neck Surg. 1986;8:177–184.

5. Waldron CA, El-Mofty SK, Gnepp DR. Tumours of the intraoral

minor salivary glands: a demographic and histologic study of 426

cases. Oral Surg Oral Med Oral Pathol. 1988;66:323–333.

6. Huang MX, Ma D, Sun K, Yu G, Guo C, Gao F. Factors

influencing survival rate in adenoid cystic carcinoma of the

salivary glands. Int J Oral Maxillofac Surg. 1988;26:435–439.

7. Alleyne CH, Bakay RA, Costigan D, Thomas B, Joseph GJ.

Intracranial adenoid cystic carcinoma. Case report and review of

the literature. Surg Neurol. 1996;44:265–271.

8. Dolan EJ, Schwartz ML, Lewis AJ, Kassel EE, Cooper PW.

Adenoid cystic carcinoma, an unusual neurosurgery entity. Can J

Neurol Sci. 1985;12:65–68.

9. Wakisaka S, Nonaka A, Morita Y, Fukui M, Kinoshita K.

Adenoid cystic carcinoma with intracranial extension: a report of

three cases. Neurosurgery. 1990;26:1060–1065.

10. Fei Dong, Paul W Gidley, Tang Ho, Mario A Luna, Lawrence E

Sturgis, Erich M Sturgis. Adenoid cystic carcinoma of the external

auditory canal. Laryngoscope. 2008;118:1591–1596.

11. Shailesh M Gondivkar, Amol R Gadbail, Revant Chole, Rima V

Parikh. Adenoid cystic carcinoma: A rare clinical entity and

literature review. Oral Oncol. 2011;47:231–236.

12. Dai Takagi, Satoshi Fukuda, Yasushi Furuta, et al. Clinical study

of adenoid cystic carcinoma of the head and neck. Auris Nasus

Larynx. 2001;28:99–102.

13. Komurcu Seref, Ozet Ahmet, Safali Mukerrem, Turken Orhan,

Ozturk Bekir, Arpaci Fikret, Yalcin Bulent. Delayed pulmonary

metastasis of adenoid cystic carcinoma originated from external

earway: Case report. Turkish Journal of Cancer. 2005;35:93–95.

14. Chia-Hao Chang, Min-Tsan Shu, Jehn-Chuan Lee, Yi-Shing Leu,

Yu-Chun Chen, Kuo-Sheng Lee. Treatments and outcomes of

malignant tumors of external auditory canal. Am J Otolaryngol

Head Neck Med Surg. 2009;30:44–48.

15. Fliss DM, Kraus M, Tovi F. Adenoid cystic carcinoma of the

external auditory canal. Ear Nose Throat J. 1990;69:638–639.

16. Silverman DA, Carlson TP, Khunntia D, Bergstrom RT, Saxton J,

Esclamado RM. Role of postoperative radiation therapy in

adenoid cystic carcinoma of the head and neck. Laryngoscope.

2004;114:1194–1199.