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 Table of Contents  
CASE REPORT
Year : 2019  |  Volume : 25  |  Issue : 1  |  Page : 34-36

Paraganglioma of external auditory canal: A rare entity


1 Department of ENT, Head and Neck Surgery, Institute of Naval Medicine, INHS Asvini, Mumbai, Maharashtra, India
2 Department of Pathology, Institute of Naval Medicine, INHS Asvini, Mumbai, Maharashtra, India

Date of Web Publication19-Jun-2019

Correspondence Address:
Dr. Shruti Anil Sharma
Department of ENT, Head and Neck Surgery, Institute of Naval Medicine, INHS Asvini, Colaba, Mumbai - 400 005, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/indianjotol.INDIANJOTOL_131_18

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  Abstract 


We present the description of a painful paraganglioma of the external auditory canal. Atypical presentation made the diagnosis difficult. We discuss the role of histopathology and immunohistochemistry in the diagnosis of the condition. A 43-year-old male presented with left-sided otalgia and hearing loss. Physical examination revealed a firm swelling arising from the posterosuperior portion of the left external auditory canal. Differential diagnosis of exostosis and squamous cell carcinoma of the external auditory canal was considered. The high-resolution computed tomography of the temporal bones was suggestive of neoplasia. The patient underwent excision biopsy. The hemorrhage during surgery led to suspicion of a vascular tumour. The histopathology and immunohistochemistry confirmed the diagnosis of paraganglioma. Early intervention with imaging and biopsy should be sought for unusual masses of the external ear.

Keywords: External auditory canal, paraganglioma, rare


How to cite this article:
Rajguru RS, Sharma SA, Mehta RK, Singh I. Paraganglioma of external auditory canal: A rare entity. Indian J Otol 2019;25:34-6

How to cite this URL:
Rajguru RS, Sharma SA, Mehta RK, Singh I. Paraganglioma of external auditory canal: A rare entity. Indian J Otol [serial online] 2019 [cited 2019 Jul 20];25:34-6. Available from: http://www.indianjotol.org/text.asp?2019/25/1/34/260719




  Introduction Top


Paragangliomas are rare tumors of the head and neck comprising approximately 0.6% of all neoplasms in the head-and-neck region.[1] They arise from paraganglia of neural crest cells. Paragangliomas of the temporal bone are categorized into jugular, jugulotympanicum, and tympanic paragangliomas. Tympanic paragangliomas are confined to tympanic cavity whereas jugular paragangliomas develop from adventitia of the jugular bulb. Jugulotympanic is reserved for those tumors in which the site of origin is impossible to define and those that have invaded the temporal bone.[2] There is female preponderance.[3] Although tympanic paragangliomas are common, paragangliomas confined to the external auditory canal are extremely rare with only four cases reported in literature till date.[4],[5],[6]


  Case Report Top


A 43-year-old male patient presented with a painful swelling (L) ear and decreased hearing (L) for 7 days duration. Examination revealed a tender firm mass arising from the posterosuperior wall of bony External auditory canal partially occluding the lumen [Figure 1]. The overlying skin was hyperaemic. The entire extent of the mass could not be ascertained clinically due to the narrowed lumen. There was no history of trauma, otorrhea, bleeding, tinnitus or vertigo. Pure tone audiometry revealed moderate conductive hearing loss.
Figure 1: Endoscopic image showing the mass arising from posterosuperior wall of bony external auditory canal partially occluding the lumen

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Examination of the (R) ear, nose and throat was unremarkable. No cervical lymph node/neck mass was palpable. General examination was within normal limit. Blood pressure was 130/80 mm Hg. Routine hematological and biochemical investigations were normal. High-resolution computed tomography (HRCT) temporal bones showed a soft-tissue mass measuring 1.1 cm × 0.55 cm within the canal with focal speculated periosteal reaction at the posterior wall of the external auditory canal along with an irregular hypodense lesion in the anterior part of mastoid bone causing focal cortical breach [Figure 2]. There was no intracranial extension.
Figure 2: High-resolution computed tomography temporal bones showing a soft-tissue mass measuring 1.1 cm × 0.55 cm within the canal

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The tumor was excised under general anesthesia through the postaural approach. Brisk bleeding was encountered during surgery. The tumor was found to be extremely vascular and was excised in toto from its attachment with the help of bipolar cautery [Figure 3]. The posterosuperior canal wall was found to be spongiotic, vascular with focal erosion. Tympanic membrane was intact. Hemostasis was achieved. Posterior bony canal wall was covered with temporalis fascia and ear pack was placed. Wound was closed in layers. Postoperative period was uneventful. Postaural sutures and External Auditory Canal (EAC) pack was removed on the 7th and 14th postoperative day, respectively.
Figure 3: Intraoperative image showing vascular mass arising from posterosuperior wall of the external auditory canal

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Histopathologic examination

Macroscopy revealed a lobular, reddish purple mass measuring 1 cm × 0.5 cm [Figure 4]. Microscopic examination revealed skin covered polypoidal tissue composed of lobules and nests of epithelioid cells arranged around small caliber blood vessels. Individual cells had round to ovoid nuclei with finely granular chromatin and a moderate amount of eosinophilic cytoplasm [Figure 5]. Immunohistochemistry panel showed vimentin V9 positivity, synaptophorin (GR007) positivity with polyclonal chromogranin showing multifocal positivity. Ki 67 (MIB-1) was found to be 07%.
Figure 4: Gross view of the specimen showing lobular, reddish purple mass measuring 1 cm × 0.5 cm

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Figure 5: Photomicrograph depicting lobules and nests of epithelioid cells arranged around small calibre blood vessels with cells having round-to-ovoid nuclei, fine granular chromatin, and moderate amount of eosinophilic cytoplasm

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The opinion of endocrinologist was sought to rule out paragangliomas elsewhere.


  Discussion Top


The 2004 World Health Organization classification of endocrine tumors defined head and neck paragangliomas as:

“[…] tumours arising from the paraganglia distributed along the parasympathetic nerves in the head, neck and mediastinum. These tumours are histologically identical and are named by anatomical site of origin.”[7]

The term is derived from the site of origin of the tumor. Temporal bone paraganglia are present in adventitia of the jugular bulb, along the IX and X cranial nerves and in the middle ear in association with Jacobsen's plexus.[2] Paragangliomas of the temporal bone are categorized into jugular, jugulotympanicum, and tympanic paragangliomas. Tympanic paragangliomas are confined to tympanic cavity, whereas jugular paragangliomas develop from adventitia of the jugular bulb. Jugulotympanic is reserved for those tumors in which the site of origin is impossible to define and those that have invaded the temporal bone.[2] There is female preponderance.[3] A mutation in succinate dehydrogenase gene seen in 40% of patients of the head-and-neck paragangliomas makes them vulnerable to developing multiple paragangliomas. They are considered to belong to amine precursor uptake and decarboxylation tumors.[2]

The presenting features of paraganglioma in the ear (commonly glomus jugulare or glomus tympanicum) are pulsatile tinnitus, conductive deafness, a red mass (the rising sun behind the drum) in the middle ear, cranial nerve palsy including otalgia and aural bleeding in some cases. The tumors are generally benign or low-grade malignancy, but they can be locally invasive with the destruction of bones and adjacent structures. Biologically indolent,[2] these tumors have a median growth rate of 1 mm per year and a median doubling time of 4.2 years.[1] Progressive cranial nerve palsies may be evident at presentation even without a substantial increase in the volume of tumor.[2] Rarely, the tumor may present with both local and distant metastases. Imaging with contrast aids in the diagnosis of paragangliomas. HRCT is a useful tool to delineate the adjacent bony detail. Magnetic resonance imaging has been used in such cases is to detect synchronous tumors in familial cases.[5] Most synchronous tumors are detected incidentally in patients with a heredofamilial pattern (40%) and nonfamilial cases (7%).[8]

In our case, the classic presenting features of a paraganglioma arising from middle ear were absent as it originated from the postsuperior bony meatal wall and was limited to the external auditory canal. The patient's external auditory canal tumour has been postulated to arise from paraganglionic tissue associated with Arnold's nerve.[9] The pain could be attributed to the auricular branch of the Vagus nerve, which relays sensory input from the posterosuperior part of the external auditory canal which conducted the nociceptive stimuli.[10]

Vanillylmandelic acid estimation could not be performed before the receipt of histopathological diagnosis due to the unusual presentation and rarity of the entity.

Imaging was useful to delineate the extent of tumor with limited diagnostic utility. Histopathology and Immunohistochemistry were of immense importance in resolving the diagnostic dilemma.

The patient is under regular follow-up and remains symptom-free.

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.

Acknowledgment

We would like to acknowledge the technical support from the Department of Anaesthesia during surgery and Department of Pathology for sample processing.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Suárez C, Sevilla MA, Llorente JL. Temporal paragangliomas. Eur Arch Otorhinolaryngol 2007;264:719-31.  Back to cited text no. 1
    
2.
Obholzer R. Scott Brown's Otolaryngology Jugular Foramen Lesions and Their Management. 8th ed., Vol. 2. Ch. 107. Milton: Chapman and Hall/CRC;2018.  Back to cited text no. 2
    
3.
Hashimoto N, Rabo CS, Okita Y, Kinoshita M, Kagawa N, Fujimoto Y, et al. Slower growth of skull base meningiomas compared with non-skull base meningiomas based on volumetric and biological studies. J Neurosurg 2012;116:574-80.  Back to cited text no. 3
    
4.
Singh KB, Hanna GS, Dinnen JS. Paraganglioma of the external auditory canal. J Laryngol Otol 1993;107:228-9.  Back to cited text no. 4
    
5.
Skinner LJ, Curran AJ, Barnes C, Timon CI. Paraganglioma of the external auditory canal: An unusual case. J Laryngol Otol 2000;114:370-2.  Back to cited text no. 5
    
6.
Hyams VJ, Batsakis JG, Michaels L. Tumours of the Upper Respiratory Tract and Ear. Washington DC: Armed Forces Institute of Pathology; 1988. p. 306.  Back to cited text no. 6
    
7.
World Health Organization. Pathology and Genetics: Tumours of Endocrine Organs. Lyon, France: International Agency for Research on Cancer; 2004.  Back to cited text no. 7
    
8.
Hodge KM, Byers RM, Peters LJ. Paragangliomas of the head and neck. Arch Otolaryngol Head Neck Surg 1988;114:872-7.  Back to cited text no. 8
    
9.
Kundu I, Goswami S, Barman D, Biswas S. Paraganglioma of external auditory canal. Indian J Otolaryngol Head Neck Surg 2001;53:304-6.  Back to cited text no. 9
    
10.
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. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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