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Year : 2019  |  Volume : 52  |  Issue : 4  |  Page : 143-146

Multiple paragangliomas involving carotid body and vagal region

Department of Otolaryngology and Head Neck Surgery (ENT), Post Graduate Institute of Medical Education and Research, Chandigarh, India

Date of Submission16-Jan-2019
Date of Decision25-Feb-2019
Date of Acceptance09-Apr-2019
Date of Web Publication27-Aug-2019

Correspondence Address:
Dr. Jaimanti Bakshi
Department of Otolaryngology and Head Neck Surgery (ENT), Post Graduate Institute of Medical Education and Research, Chandigarh - 160 012
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/fjs.fjs_8_19

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Paragangliomas are rare neuroendocrine tumors also known to occur in the head-and-neck regions and generally involve the carotid body or vagal region. Till date, only a single study has reported the occurrence of paragangliomas involving both the carotid body and vagal region. The present study, to the best of our knowledge, is the second study in the world which reported the multiple paragangliomas involving the carotid body and vagal region. The patient in the present case study was a 45-year-old male with a single swelling in the left upper part of the neck. In the preoperative clinical examination, the swelling was identified as carotid body tumor, and the patient was then planned for surgery. The intra-operative findings revealed that it involved both the vagal and carotid body and was subsequently operated successfully. Postoperatively, the swelling was described as bilateral carotid paragangliomas with left vagal paraganglioma.

Keywords: Carotid, paragangliomas, radiotherapy, surgery, tumor, vagal

How to cite this article:
Bakshi J, Goyal AK, Vir D, Panda NK. Multiple paragangliomas involving carotid body and vagal region. Formos J Surg 2019;52:143-6

How to cite this URL:
Bakshi J, Goyal AK, Vir D, Panda NK. Multiple paragangliomas involving carotid body and vagal region. Formos J Surg [serial online] 2019 [cited 2021 Feb 28];52:143-6. Available from: https://www.e-fjs.org/text.asp?2019/52/4/143/265493

  Introduction Top

Paragangliomas are neuroendocrine tumors that represent 0.012% of all tumors.[1] Paragangliomas arise from extra-adrenal paraganglia, microscopic islands of cells derived embryonically from the neural crest cells of the autonomic nervous system.[1],[2] Paragangliomas may occur anywhere from the skull base to the pelvic floor along with the distribution of paraganglia.[3] The common sites of occurrence include the abdomen, retroperitoneum, chest and mediastinum, and various head-and-neck locations.[4],[5] In the head-and-neck region, paragangliomas are very rare and represent 0.6% of all tumors[1] and found to occur in the jugulotympanic membrane, orbit, nasopharynx, larynx, vagal body, and carotid body regions.[5],[6]

Carotid body tumors are the most common paraganglioma often present as slow-growing, nontender neck masses located just anterior to the sternocleidomastoid muscle at the level of the hyoid.[4] Carotid body paragangliomas arise at the bifurcation of the internal and external carotid arteries.[5] Vagal paraganglioma tumors arise from nests of paraganglionic tissue within the perineurium at various sites of the vagal nerve.[7],[8] As opposed to carotid body tumors, vagal paragangliomas are located more cephalad in the neck, between the jugular vein and the internal carotid artery, sometimes extending to the base of the skull through the jugular foramen or posteriorly to the mastoid tip.[7]

All types of paragangliomas are generally recommended be removed earliest possible because their development will involve contiguous structures, with difficult excision and postoperative cranial nerve damage.[6] Surgical excision of the tumor includes proximal and distal control of the carotid arteries with ligation of feeding branches from the external carotid arteries and dissection of the mass starting at the carotid bifurcation and continuing in a caudal-cranial fashion.[6] Elective external carotid arteries ligation can be helpful for tumor mobilization.[6] Alternatively, the dissection of the tumor can be started cranially which can facilitate early proximal control over the majority of adjacent nerves, reducing the risk of postoperative morbidity.[6]

The present case report represents the sporadic case of multiple paragangliomas, involving the carotid body and vagal region. Till date, an only a single study has reported the occurrence of paragangliomas involving both the regions.[6] The present study, to the best of our knowledge, is the second study in the world which reported the multiple paragangliomas involving the carotid body and vagal region.

  Methodology Top

Recruitment of the patient

The patient in the present case report was recruited in the Otolaryngology and Head-and-Neck Surgery Outpatient Department (ENT) of the Postgraduate Institute of Medical Education and Research, Chandigarh. Written consent was obtained from the patient and patient was also addressed about the numerous potential complications associated with the paraganglioma surgery. The patient was informed about the possible neural dysfunction with associated hoarseness, vocal change, aspiration, dysphagia, dysarthria, facial asymmetry, and shoulder weakness in addition to the possibility of a cerebrovascular accident.

Language, grammar, and plagiarism

The references were inserted using EndNote software Version 7.4 (Thomson Reuters, Toronto, Canada), language and grammar was checked by Grammarly software version 6.6 (Grammarly, Inc., San Francisco, California, United States), plagiarism was checked with the help of Turnitin plagiarism detection service (Webster St., California), proofread by doc navigator©, Chandigarh.

  Case Report Top

Sociodemographic analysis

In the present case study, the patient was a 45-year-old male [Figure 1]. The patient belonged to Sirmaur, Himachal Pradesh, and was watchman by profession. The patient came with the complaint of swelling in the upper left region of the neck. The patient revealed that swelling is present from the last 3 years and the swelling was insidious at onset and progressed gradually.
Figure 1: Clinical examination showing swelling on the upper left region of the neck

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Clinical examination

The patient was a rare case of multiple paragangliomas having a single 6 cm × 5 cm, well-defined ovoid, firm swelling in the left upper part of the neck. The swelling is located at mastoid tip superiorly, at the level of hyoid inferiorly, at an angle of mandible anteriorly, and 5 cm fromthe angle of the mandible. The swelling was nontender, nonpulsatile, and mobile only in horizontal direction. Overlying skin was healthy, and no complaint of pain and other symptoms was reported. On physical examination, no other such swelling was observed in the patient body. No family history of similar disease was reported. The clinical examination was cross confirmed with the histopathological findings.

Radiological assessment

Contrast-enhanced computed tomography (CECT) of the neck [Figure 2] revealed enhancing lesion at the level of bilateral carotid bifurcations causing splaying of bilateral carotid bifurcations. Digital Substraction Angiography (DSA) shows tumor blush at the level of bilateral carotid bifurcations more on the left than the right side showing characteristic Lyre sign as described in the literature [Figure 3] and [Figure 4].
Figure 2: Contrast-enhanced computed tomography scan showing the enhancing mass lesions in the left and right carotid bifurcations

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Figure 3: Left common carotid bifurcate (a) and right common carotid bifurcate (b) showing the Lyre's sign (*)

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Figure 4: Intraoperation (a) picture showing vagal paragangliomas (*) and carotid paragangliomas (#) which was removed successfully postoperatively as showed in picture (b)

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Surgical findings

In a preoperative diagnosis, the swelling was identified as a carotid body tumor. Intraoperative findings revealed that it involved both the vagal and carotid body. Both the tumors were present on the left side and were excised during the surgery successfully [Figure 4]. Postoperatively, the swelling was described as bilateral carotid paragangliomas with left vagal paragangliomas. The patient remained admitted after surgery and was discharged after removal of sutures on the 7th day.

Follow-up analysis

Follow-up of the patient revealed healthy woun, and indirect laryngoscopy showed left vocal cord palsy with no aspiration. Speech therapy then was prescribed, and the patient was followed up for 2 years for the possibility of recurrence.

  Discussion Top

The existence of multiple paragangliomas, involving carotid and vagal region, is extremely rare,[6] and to the best of our knowledge, the present study represents the second case of the same in the world. An earlier study has reported that the incidence of paragangliomas is more in individual living at high altitudes and who are affected by chronic obstructive pulmonary disease.[6] The patient in the present study was also living in high altitude. Earlier, the paragangliomas were reported to occur five times more frequently in female compared to the males[6] and more frequently in middle-age patients[7] to which contradictory with the present case report, in which a 45-year-old male was reported with paragangliomas. The family history of the disease is considered as a potential risk factor in case of paragangliomas, and around 50% of cases in earlier studies were reported to have a family history of the disease.[6] However, in the present study, the patient did not have any family history of the disease. The most frequent symptoms associated with the paragangliomas in the neck region reported were palpable neck mass (55%), tinnitus (18%), and cranial nerve palsies (16%).[9] In the present study, we also reported the neck mass, but tinnitus and palsies were absent. The patient in the present study reported having no complaints of pain which is consistent with the previous studies.[6],[10]

Accurate diagnosis of paraganglioma is thought to be difficult, and therefore, both clinical findings and radiographic studies are recommended in such cases.[7] Noninvasive techniques such as CT, magnetic resonance imaging, or positron emission tomography can be combined with clinical examination for accurate characterization of the tumor.[9] In the present study also, we used the CECT scan to see the extent of paragangliomas in the neck region. In some cases, the conventional angiography or digital subtraction angiography was also done to demonstrate the vascular supply of paraganglioma[6] which was also done for the present case.

Although the surgical resection is believed to be the definitive treatment for paragangliomas, difficulties may arise in cases where tumor are inoperable.[9] Furthermore, the surgery generally requires sacrificing the vagus nerve and is associated with other nervous lesions. Therefore, vagal, hypoglossal, and glossopharyngeal paralysis are common postoperative complications.[7] In the present case, unilateral vocal palsy due to the vagus nerve injury occurs, but fortunately, no aspiration during swallowing postoperatively was found. Even after successful surgery, there is always the chance of recurrence[9] which was also not seen in the present case in the follow-up of 2 years. Alternative to surgery, radiation therapy is another choice of treatment for arresting the growth of tumor in cases where the patients were inoperable[7] or at high risk for surgery due to tumor size, patient age, comorbidity conditions, preoperative cranial nerve involvement, anticipated postoperative cranial nerve paralysis, patency of the circle of Willis to provide for cross-cerebral circulation, multiplicity of paragangliomas, and the involvement of local organs (esophagus, soft palate, and larynx) and symptoms.[11],[12] Furthermore, it has been reported that the radiation can lead to neurologic sequelae and rarely can destroy the tumor[7] because tumors arising from the vagus and carotid body were thought to be radioresistant.[11] However, monolateral radiotherapy can be indicated after contralateral surgery to avoid bilateral vagus nerve damage.[7] Furthermore, the gamma knife radiosurgery and CyberKnife stereotactic radiotherapy are thought to provide a higher degree of accuracy and precision than conventional radiotherapy.[6]

  Conclusion Top

The present study represents the sporadic case of paragangliomas involving both the carotid body and vagal region. The clinical examination and radiological studies were done before surgery. The patient was treated successfully with the surgery and recurrence was not reported in 2 years to follow-up. Our study will help the clinicians in designing the treatment strategies for such complicated cases of multiple paragangliomas.

Compliance with ethical standards

Written informed consent was obtained from the patient mentioning the permission to use clinical data and photographs for research publication purpose.

Declaration of patient consent

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


The authors highly acknowledged the participation of the patient and to all of the colleagues who reviewed the present work.

Financial support and sponsorship

PGIMER has provided the facilities for performing surgery and other diagnostic facilities. The work in this research is also supported by the Junior Research Fellowship provided by ICMR, New Delhi, wide file no. 3/13/JRF -2015/HRD.

Conflicts of interest

There are no conflicts of interest.

  References Top

Xiao Z, She D, Cao D. Multiple paragangliomas of head and neck associated with hepatic paraganglioma: A case report. BMC Med Imaging 2015;15:38.  Back to cited text no. 1
Seo MC, Lee KS, Kim CJ, Kim SY. Multiple paragangliomas: Three cases. Korean J Otorhinolaryngol Head Neck Surg 2000;43:442-6.  Back to cited text no. 2
Ertz-Archambault NM, Van Gompel JJ, Neff BA, Kasperbauer JL, Shamoun FE. What happens in vagus: A case of recurrent paraganglioma with malignant transformation and an updated treatment algorithm†. J Surg Case Rep 2016;2016. pii: rjw012.  Back to cited text no. 3
Davidovic LB, Djukic VB, Vasic DM, Sindjelic RP, Duvnjak SN. Diagnosis and treatment of carotid body paraganglioma: 21 years of experience at a clinical center of serbia. World J Surg Oncol 2005;3:10.  Back to cited text no. 4
Wieneke JA, Smith A. Paraganglioma: Carotid body tumor. Head Neck Pathol 2009;3:303-6.  Back to cited text no. 5
Del Guercio L, Narese D, Ferrara D, Butrico L, Padricelli A, Porcellini M, et al. Carotid and vagal body paragangliomas. Transl Med UniSa 2013;6:11-5.  Back to cited text no. 6
Caldarelli C, Iacconi C, Della Giovampaola C, Iacconi P, Beatrice F. Vagal paragangliomas: Two case reports. Acta Otorhinolaryngol Ital 2007;27:139-43.  Back to cited text no. 7
Bakshi J, Mohammed AW, Lele S, Nada R. Ganglioneuromas involving the hypoglossal nerve and the vagus nerve in a child: Surgical difficulties. Ear Nose Throat J 2016;95:E22-4.  Back to cited text no. 8
Tonyukuk V, Emral R, Temizkan S, Sertçelik A, Erden I, Corapçioǧlu D, et al. Case report: Patient with multiple paragangliomas treated with long-acting somatostatin analogue. Endocr J 2003;50:507-13.  Back to cited text no. 9
Langerman A, Athavale SM, Rangarajan SV, Sinard RJ, Netterville JL. Natural history of cervical paragangliomas: Outcomes of observation of 43 patients. Arch Otolaryngol Head Neck Surg 2012;138:341-5.  Back to cited text no. 10
Pandey M, Chandramohan K, Sebastian P, Ramachandran K. An unusual bilateral cervical paraganglioma: A case report. Int J Oral Maxillofac Surg 2002;31:334-6.  Back to cited text no. 11
Myssiorek D, Persky M. Treatment of carotid paraganglioma. Oper Tech Otolaryngol Head Neck Surg 2016;27:30-5.  Back to cited text no. 12


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

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