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CASE REPORT
Year : 2022  |  Volume : 28  |  Issue : 3  |  Page : 246-248

Paraganglioma of external auditory canal: A rare presentation case report


Department of ENT and HNS, Command Hospital Airforce, Bengaluru, Karnataka, India

Date of Submission29-Jan-2022
Date of Decision02-Mar-2022
Date of Acceptance04-Jul-2022
Date of Web Publication21-Nov-2022

Correspondence Address:
Dr. Aakriti Sanjay Athavale
Command Hospital Airforce, Agram Post, Ulsoor, Bengaluru - 560 008, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/indianjotol.indianjotol_20_22

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  Abstract 


Paragangliomas (PGLs) are benign tumors of neuroectodermal origin. In the ear, they are commonly found in the middle ear or jugular bulb. PGL of external auditory canal (EAC) is extremely rare. To date, only a few cases of PGL of EAC have been reported. Here, we present a case of a 52-year-old female, who presented with complaints of decreased hearing and painful left ear discharge. On evaluation, she was found to have a soft-tissue mass arising from the posterosuperior wall of the left EAC, which was excised under general anesthesia and histopathologically confirmed to be EAC PGL. At 1 year of follow-up, the patient has a patent EAC and is disease-free. Although rare, PGL should be considered in the differential diagnosis of soft-tissue mass in EAC.

Keywords: External auditory canal, paraganglioma, rare


How to cite this article:
Athavale AS, Singh R, Sahu PK. Paraganglioma of external auditory canal: A rare presentation case report. Indian J Otol 2022;28:246-8

How to cite this URL:
Athavale AS, Singh R, Sahu PK. Paraganglioma of external auditory canal: A rare presentation case report. Indian J Otol [serial online] 2022 [cited 2022 Dec 6];28:246-8. Available from: https://www.indianjotol.org/text.asp?2022/28/3/246/361638




  Introduction Top


Paragangliomas (PGLs) are tumors from cells of neural crest origin. In the head and neck, paraganglia are found along the adventitia of jugular bulb, carotid body, along the course of Jacobson's nerve, and rarely Arnold's nerve. PGLs account for 0.6% of all head and neck tumors and only 2%–3% of them are secretory.[1] PGLs of external auditory canal (EAC) are extremely rare.[2] They arise from paraganglia surrounding Arnold's nerve in the mastoid segment of the facial nerve (Frisch et al.).[6] This article reports a case of a 52-year-old lady with left EAC PGL.


  Case Report Top


A 52-year-old female reported with a complaint of left ear decreased hearing and blood-stained painful discharge of 10 days duration. There was no history of tinnitus, vertigo, or trauma to the ear. The patient gave a history of palpitation but no flushes.

Ear examination revealed purulent discharge in left EAC with firm, tender polypoidal mass arising from posterosuperior EAC wall [Figure 1]. The tympanic membrane was minimally visible only in the anteroinferior quadrant. Pure-tone audiometry revealed a moderate conductive hearing loss in the left ear. Examination of the right ear was normal. The nose and throat had no significant findings. There was no cervical lymphadenopathy.
Figure 1: Preoperative endoscopic finding of mass in the left EAC with attachment to posterosuperior EAC wall (arrow). EAC: External auditory canal

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Imaging correlation of high-resolution computed tomography of temporal bones and contrast-enhanced magnetic resonance imaging study revealed an enhancing soft-tissue mass measuring 4.6 mm × 11.2 mm × 4.6 mm in the left EAC, impinging onto the tympanic membrane [Figure 2]. There was no ossicular/scutal/bony EAC erosion seen without any extension into aditus and no intracranial extension. Preoperative whole-body Ga-68 DOTANOC positron-emission computed tomography was done in view of palpitations which revealed no abnormal somatostatin receptor uptake systemically or in the left EAC. 24 h urine metanephrines and normetanephrines were also found within normal range. However, during preoperative evaluation, the patient was diagnosed with primary hypertension which was managed medically. The tumor was noted to be arising from posterosuperior EAC wall which was excised under general anesthesia by postaural approach. The mastoid and middle ear was also inspected with no special inference. A perforation noted in the posterosuperior quadrant of pars tensa of tympanic membrane was repaired using temporalis fascia graft by underlay technique. Histopathological examination of the excised mass was conclusive of PGL [Figure 3]. Immunohistochemical examination was found positive for S100, synaptophysin, chromogranin, and CD56. The postoperative period was uneventful and the patient is on regular follow-up. The operated site has no tumor recurrence and EAC is patent 1-year postsurgery.
Figure 2: HRCT temporal bones showing a soft- tissue mass in the left EAC (White arrow). HRCT: High-resolution computed tomography, EAC: External auditory canal

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Figure 3: Histopathological findings (H and E) showing the zellballen pattern of cells

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  Discussion Top


Embryologically PGLs are benign tumors of neural crest origin, most common in adrenal glands. These tumors account for about 0.6% of tumors of the head and neck and are found along the adventitia of jugular bulb, carotid body, Jacobson's nerve, and Arnold's nerve (auricular branch of vagus).[2],[3],[7] Based on his study of temporal bone sections, Guild reported the presence of paraganglionic tissue along the course of Arnold's nerve, which has a variable course entering the facial canal or traversing separately and entering the stylomastoid foramen.[7] PGLs of EAC are extremely rare.[2] The common presentation of Jugulotympanic PGLs is pulsatile tinnitus, conductive deafness, and aural fullness. In our case, the mass was arising from the posterosuperior wall of EAC and hence presented only with conductive hearing loss. Frisch CD et al. have suggested that these tumors arise from paraganglia surrounding Arnold's nerve which, may arise from the facial nerve in the mastoid segment and supply the posterosuperior canal wall.[6],[7] In our case, the pain in EAC may be related to the path of Arnold's nerve which supplies the superior aspect of EAC.[3],[4]

Unlike PGLs of other sites, only 1%–3% PGLs of the head and neck region are secretory and produce catecholamines. Secretory PGL presents with symptoms such as palpitations, sweating, and flushing.[1],[6] Imaging is advantageous in detecting familial cases.[5] Our patient reported symptoms of palpitations. This warranted her to be evaluated for secretory PGL and Pheochromocytoma. She underwent a preoperative WB-DOTANOC scan, urinary metanephrines, and normetanephrines which were found to be normal. However, during her hospital stay preoperatively, she was found to be hypertensive which explained the symptom of palpitation. Surgery provided complete tumor clearance with a definitive histopathological and immunohistochemical diagnosis while also giving an opportunity to inspect the middle ear and mastoid region. Hence, this gave our patient a tumor-free and comfortable postoperative period to date.


  Conclusion Top


The presentation of PGLs of the head and neck is highly variable. The otoscopic findings and location of the tumors are also highly variable. Thus, as compared to jugulotympanic PGLs, PGLs in EAC may not be easily suspected on otoscopic examination and imaging. Due to the rarity of EAC PGLs, they may be misdiagnosed as polyp, missing proper evaluation. Our case suggests that EAC PGL should be considered in the differential diagnosis of an EAC mass, despite its rarity, to avoid misdiagnosis and inadequate management.

Ethical clearance

Written and informed consent of the patient and institutional ethics committee clearance was obtained for scientific publishing of the patient data and findings.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Hayward N, Cousins V. Head and neck paraganglioma: Medical assessment, management, and literature update. J Otorhinolaryngol Hear Balance 2017;1:1-12.  Back to cited text no. 1
    
2.
Seo HS. Paraganglioma of the external auditory canal: A case report. J Clin Otolaryngol 2017;28:234-7.  Back to cited text no. 2
    
3.
Rajguru RS, Sharma SA, Mehta1 RK, Singh I. Paraganglioma of external auditory canal: A rare entity. Indian J Otology 2019;25:35-6.  Back to cited text no. 3
    
4.
Grover N, Amen F, Gallimore A, Brookes G. External auditory canal paraganglioma: An atypical presentation. J Laryngol Otol 2012;126:1278-80.  Back to cited text no. 4
    
5.
Sharma P, Thakar A, Suman KC, Dhull VS, Singh H, Naswa N, et al. 68Ga-DOTANOC PET/CT for baseline evaluation of patients with head and neck paraganglioma. J Nucl Med 2013;54:841-7.  Back to cited text no. 5
    
6.
Frisch CD, Driscoll CL, Neff BA. Paraganglioma of the facial canal: A report of 2 cases and literature review, Am J Otolaryngol 2014;35:800-5.  Back to cited text no. 6
    
7.
Goyal S, Singh SK, Singh R, Roy R, Pati S, Arumugam SV. Primary secretory paraganglioma of facial nerve canal: A case report. J Int Adv Otol 2021;17:175-8.  Back to cited text no. 7
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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