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An Official Publication of the Indian Association of Oral and Maxillofacial Pathologists


 
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CASE REPORT  
Year : 2023  |  Volume : 27  |  Issue : 5  |  Page : 38-40
 

Extra adrenal paragnglioma of oral cavity: A rare case report


1 Department of Oral Pathology and Microbiology, NIMS Dental College, Jaipur, Rajasthan, India
2 Department of Oral Pathology and Microbiology, KGMU, Lucknow, Uttar Pradesh, India

Date of Submission09-Dec-2021
Date of Acceptance11-Jan-2023
Date of Web Publication04-Feb-2023

Correspondence Address:
Pradkhshana Vijay
Pool Officer, Department of Oral Pathology and Microbiology, KGMU, Lucknow-03, Uttar Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jomfp.jomfp_432_21

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   Abstract 


Paragangliomas are neuroendocrine tumors similar to pheochromocytomas but arising from extra adrenal site. It is a very rare tumor in an intraoral site, we found a single case of intraoral paraganglioma in a literature, and hence as per our best knowledge this is only the second case of paraganglioma presented in an intraoral location. We present here a case report of paraganglioma on the ventral surface of the tongue. A 37 years old lady presented with a large asymptomatic swelling on the ventral surface of the tongue from one year. The past medical history and familial history were non relevant to the swelling. Surgical excision was performed and histopathological examination of the specimen revealed the diagnosis of extra adrenal paraganglioma. The case is being presented here for its rarity in an intraoral location.


Keywords: Asymptomatic swelling, embolization, polygonal chief cells, radiotherapy, Zellballen appearance


How to cite this article:
Bajpai M, Vijay P, Pardhe N. Extra adrenal paragnglioma of oral cavity: A rare case report. J Oral Maxillofac Pathol 2023;27, Suppl S1:38-40

How to cite this URL:
Bajpai M, Vijay P, Pardhe N. Extra adrenal paragnglioma of oral cavity: A rare case report. J Oral Maxillofac Pathol [serial online] 2023 [cited 2023 Mar 24];27, Suppl S1:38-40. Available from: https://www.jomfp.in/text.asp?2023/27/5/38/369181





   Introduction Top


Paraganglioma is a generic term used for the tumors arising from paraganglia regardless of their location.[1] Paragangliomas, when found outside the adrenal gland are designated as extra adrenal Paragangliomas.[2] Head and neck paraganglia occur in association with the carotid body, the ganglion nodosum of the vagus nerve, the middle ear (jugulotympanic paraganglia), and in other rarer sites where paraganglia are known to exist.[1] An exhaustive review of the literature could reveal a single case of paraganglioma on an intraoral site.[3] Histologically, tumor shows a characteristic zellballen appearance.[3] Paragangliomas are considered as prognostically intermediate group of neoplasm.[4] Paragangliomas are not considered as a differential diagnosis of the swelling of the ventral surface of the tongue because of its rarity in an intraoral site.


   Case Report Top


A 37 years old female patient presented with a swelling on the ventral surface of the tongue. The swelling was present since one year with a gradual progression. The swelling was asymptomatic and gradually progressive in size from one year. The past medical and family history was non contributory. Intraoral examination revealed non tender , oval swelling measuring 2×2 cm on ventral surface of tongue with normal overlying mucosa. [Figure 1]. Initially, an FNAC of the lesion was performed but it was inconclusive. Following this lesion was surgically excised under local anesthesia and tissue was sent for histopathological examination [Figure 2]. Histopathological examination revealed, round nests of uniform polygonal chief cells with granular cytoplasm arranged in a characteristic Zellballen appearance [Figure 3]. Nest of polygonal cells were separated by the cytoplasmic processes of sustentacular cells [Figure 4]. The stroma was dense and fibrovascular. Clinical investigation of urine was negative for catecholamines. The final diagnosis of extra adrenal paraganglioma was given. Patient was on a follow up for one year and no further complications were noted.
Figure 1: Clinically lesion showing swelling on the ventral surface of the tongue

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Figure 2: Gross specimen of excised tissue

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Figure 3: Photomicrograph showing characteristic zellballen appearance (Hematoxylin and eosin Stain 20 X)

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Figure 4: Photomicrograph showing nest of polygonal cells were separated by the cytoplasmic processes of sustentacular cells. (Hematoxylin and eosin Stain 20 X)

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   Discussion Top


A paraganglioma is a rare neuroendocrine neoplasm developing at various body sites (including the head and neck thorax and abdomen).[5] Paragangliomas can present in unusual anatomic locations where normal paraganglioma are not well documented.[1] Paraganglioma in head and neck region mainly arise from paraganglionic tissue within the perineurium of the vagus nerve at its ganglion nodosum, i.e., just below skull base.[1] In large tumors, there may be small extension through the foramen jugulare. Anatomic locations of non-adrenal paragangliomas are not always well known and locating a paraganglioma on the ventral surface of the tongue implies the paraganglia related to the glossopharyngeal nerve.[6] Clinically, the paragangliomas are usually asymptomatic although all paragangliomas contain neurosecretory granules, only 1-3% of cases show a significant secretion of hormones like catecholamines.[2] In present case, the tumor was presented as an asymptomatic mass and the clinical investigation of urine was negative for catecholamines. On histopathological examination, the tumor cells are readily recognized. Individual tumor cells are polygonal to oval and are arranged in distinctive cell balls, called Zellballen.[7] The main treatment modalities are surgery, embolization, and radiotherapy.[3] The present case was treated by surgical excision and one year follow up period was uneventful.

Paragangliomas of oral cavity are unusual and there is a paucity of literature available regarding these neoplasm's in oral cavity. As per our knowledge, this is only the second case of paraganglioma reported in oral cavity.

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.
Lack EE. Pathology of Adrenal and Extra-Adrenal Paraganglia. Philadelphia, Pa: WB Saunders; 1994.  Back to cited text no. 1
    
2.
LaGuette J, Matias-Guiu X, Rosai J. Thyroid paraganglioma: A clinicopathologic and immunohistochemical study of three cases. Am J Surg Pathol 1997;21:748-53.  Back to cited text no. 2
    
3.
Nielsen TO, Séjean G, Onerheim RM. Paraganglioma of the tongue. Arch Pathol Lab Med 2000;124:877-9.  Back to cited text no. 3
    
4.
Schuller DE, Lucas JG. Nasopharyngeal paraganglioma report of a case and review of literature. Arch Otolaryngol 1982;108:667-70.  Back to cited text no. 4
    
5.
Rao A, Koeller K, Adair C. Paragangliomas of the head and neck: Radiologic-pathologic correlation. RadioGraphics 1999;19:1605-32.  Back to cited text no. 5
    
6.
Zak FG, Lawson W. Glomic (paragangliomic) tissue in the larynx and capsule of the thyroid gland. Mt Sinai J Med 1972;39:82-90.  Back to cited text no. 6
    
7.
Kairi-Vassilatou E, Argeitis J, Nika H, Grapsa D, Smyrniotis V, Kondi-Pafiti A. Malignant paraganglioma of the urinary bladder in a 44-year-old female: Clinicopathological and immunohistochemical study of a rare entity and literature review. Eur J Gynaecol Oncol 2007;28:149-51.  Back to cited text no. 7
    


    Figures

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



 

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    Abstract
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