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 Table of Contents  
Year : 2017  |  Volume : 5  |  Issue : 2  |  Page : 86-89

Bilateral carotid body tumor in a 13-year-old child: Our experience

1 Department of Otolaryngology and Head and Neck Surgery, Postgraduate Institute of Medical Education and Research, Chandigarh, India
2 Department of Cardiovascular and Thoracic Surgery, Postgraduate Institute of Medical Education and Research, Chandigarh, India

Date of Web Publication22-Jan-2018

Correspondence Address:
Prof. Naresh Kumar Panda
Department of Otolaryngology and HNS, Postgraduate Institute of Medical Education and Research, Chandigarh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jhnps.jhnps_11_17

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Carotid body tumors are rare neuroendocrine tumors derived from the extra-adrenal paraganglioma of the autonomic nervous system. Very few cases have been reported in literature in the pediatric age group <14 years of age. We present our experience in managing a Shamblin III carotid body tumor in a 13-year-old boy who underwent an unsuccessful attempt at excision at another hospital. Surgical excision of the tumor along with ligation of the internal carotid artery was done without any postoperative neurological sequel. The tumor on the contralateral side was being managed conservatively with serial follow-up.

Keywords: Carotid body tumor, glomus caroticum, internal carotid artery ligation, pediatric paraganglioma

How to cite this article:
Singh A, Subash A, Panda NK, Mishra AK. Bilateral carotid body tumor in a 13-year-old child: Our experience. J Head Neck Physicians Surg 2017;5:86-9

How to cite this URL:
Singh A, Subash A, Panda NK, Mishra AK. Bilateral carotid body tumor in a 13-year-old child: Our experience. J Head Neck Physicians Surg [serial online] 2017 [cited 2021 Dec 5];5:86-9. Available from: https://www.jhnps.org/text.asp?2017/5/2/86/223757

  Introduction Top

Alfred Kohn in 1903, described paraganglioma (PG) as a group of neuroendocrine cells arranged like ganglions that are innervated by nerves without being a true ganglion.[1] PGs are tumors of neural crest origin, arising from chromaffin cells. These cell secret catecholamines. Carotid body tumors (CBT) are nonchromaffin neoplasms arising from paraganglion (glomus bodies) located at carotid bifurcation. They are composed of chemoreceptor cells of neuroectodermal origin that migrate from neural crest along with autonomic nervous system.[2],[3],[4]

Carotid body tumor accounts for almost 60% of head and neck PG and is the most common head and neck PG [5],[6] in children; PGs are predominantly extra-adrenal and can occur anywhere along the autonomic nervous system.[7] PGs appear to have male predilection before adolescence.[8]

It usually present as accidentally detected painless slow-growing upper cervical mass.[9],[10] Surgery remains the mainstay of treatment in almost all cases as enlarging mass may involve glossopharyngeal, hypoglossal, vagus or recurrent laryngeal nerve,[11] or may invade carotid vessel with resultant stenosis, occlusion, or even rupture.[12]

According to of tumor size and involvement of the carotid vessels, three types of CBT has been classified by Shamblin where type I and II are amenable to subadventitial resection while type III includes tumors encircling the carotid vessels, invading the media and thus excluding surgical resection possibility.[5],[13]

Only few cases of CBT in children have been reported in literature till now, most of them sporadic in type. We report our experience in managing the case of a 13-year-old boy with bilateral CBT.

  Case Report Top

A 13-year-old boy (weight 45 kg: height 150 cm), hailing from the plains of Northern India presented to a general surgeon with complaints of a painless slowly growing left-sided neck swelling for 4 months. An attempt at excision was made there and procedure was abandoned after biopsy due to torrential bleeding. The child was subsequently referred to our center for further management. Initial evaluation revealed that the child did not have any other symptom apart from painless neck swelling. The child did not have any other associated comorbities or addiction. There was no family history of PG and child had never resided in hilly areas and was not exposed to chronic hypoxic conditions. Examination revealed a 3 cm × 2 cm firm, pulsatile, nontender swelling in the left upper part of neck with a transverse scar in the overlying skin [Figure 1]. Oral cavity examination did not show any bulge in the lateral pharyngeal wall. There was no cranial nerve involvement. An ultrasonography of neck with Doppler scan revealed a tumor in the left carotid space. A contrast-enhanced computed tomography with angiography of neck vessels revealed an enhancing lesion in the left carotid space, encasing the left internal carotid artery (ICA) (Shamblin III) with thrombosis of left ICA system [Figure 2]. An incidental finding noted was an enhancing lesion occupying the opposite carotid bifurcation (Shamblin I). The findings were suggestive of carotid body tumor, and hence, to ascertain the nonfunctioning nature of the tumor, an ultrasound abdomen and urine vanillylmandelic acid levels were done which were essentially normal. A digital subtraction angiography was done with access obtained from right femoral artery which revealed abnormal tumor blush from a branch of left external carotid artery (ECA). Selective cannulation of the feeding vessels was done and gel foam injected under fluoroscopic guidance. Postprocedure run showed around 90% decrease in tumor blush [Figure 3]. After 48 h, the child was then taken up for left carotid body tumor excision. Intraoperatively, the tumor was seen completely encasing the left ICA with resultant thrombosis and cord-like vessel, which was ligated and resected. There was an inadvertent injury to left ECA that was repaired with 6-0 prolene suture and its ascending pharyngeal and posterior auricular branches had to be ligated. Postoperatively, the child had no focal neurological deficit, made early recovery, and was discharged on 3rd postoperative day. The histopathological examination confirmed features of carotid body PG. Since on the right side was a Shamblin I tumor, it was kept on close regular follow-up as any surgical injury to contralateral side would have caused devastating neurological consequences [Figure 4].
Figure 1: A 3 cm × 2 cm swelling in the upper part of the neck on the left side, with an overlying scar

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Figure 2: Computed tomography angiography scan of neck vessels

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Figure 3: Digital subtraction angiography – pre- and post-embolization

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Figure 4: Postoperative – day 3

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  Discussion Top

Head and neck PGs constitute 3% of all PG in the body. They most commonly occur in the fourth to sixth decades of life.[14],[15] PGs are usually benign and slow growing. Only 20% of head and neck PGs have subsequent malignant behavior. Malignant character is justified only by metastatic disease predominantly to cervical nodes but infrequently to distant organs.[16]

CBTs are sporadic in form but rarely can have familial inheritance.[17] They are often bilateral. Splaying of internal and external carotid arteries (Lyre sign) on angiography is the most consistent feature.

Considering the vascular nature of CBTs, incisional biopsy is not recommended as it results in profuse bleeding and can cause cranial nerve injury. Complete CBT resection combined with vascular reconstruction is more frequently needed in Shamblin type III tumor. In our case, an attempt at excision was made at another institute which was abandoned midway due to torrential bleed. This iatrogenic injury to left ICA system could probably explain the resultant slow thrombosis and cord-like appearance of ICA intraoperatively. Increased blood flow through left ECA resulted in engorgement of posterior auricular artery, ascending pharyngeal artery, and other branches, which were injured during surgery and have to be ligated. These changes and the fibrosis due to the previous surgical attempt made the dissection difficult, and inadvertently, there was injury to the ECA requiring repair. Harmonic scalpel and magnifying loupes came in handy. As the thrombosis of ICA slowly progressed to complete occlusion, there would have been adequate time for the opposite carotid system to compensate which probably explains why the child did not have any neurological deficit despite total thrombosis of the internal carotid system on the left side. These findings were confirmed on angiography. The repair of ICA hence was not warranted in this child. Preoperative embolization of the feeder vessels of the tumor resulted in decreased blood loss, which is a key factor in children as they slip into shock rather rapidly.

Although surgical excision is the treatment of choice,[18] radiation therapy has been widely explored in the treatment of PGs, its principal indications being the postoperative recurrences and advanced nonresectable diseases. Only one case of radiation therapy in pediatric population has been presented so far in the literature. Paragangliomas in children being small in size can be resected completely. Preoperative selective angiographic embolization leading to shrinkage of tumor vascularity and size has been proposed to facilitate surgery and to diminish blood requirements [19] or even prepare the lesion for palliative radiotherapy.

  Conclusion Top

Early and proper diagnosis of CBTs is fundamental in children under 14 years of age, for the purpose of subadventitial surgical removal of carotid body tumor.

Declaration of patient consent

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

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Kohn A. Die paraganglien. Arch Mikrosk Anat 1903;52:262e5.  Back to cited text no. 1
Shamblin WR, ReMine WH, Sheps SG, Harrison EG Jr. Carotid body tumor (chemodectoma). Clinicopathologic analysis of ninety cases. Am J Surg 1971;122:732-9.  Back to cited text no. 2
LaMuraglia GM, Fabian RL, Brewster DC, Pile-Spellman J, Darling RC, Cambria RP, et al. The current surgical management of carotid body paragangliomas. J Vasc Surg 1992;15:1038-44.  Back to cited text no. 3
Lustrin ES, Palestro C, Vaheesan K. Radiographic evaluation and assessment of paragangliomas. Otolaryngol Clin North Am 2001;34:881-906, vi.  Back to cited text no. 4
Knight TT Jr., Gonzalez JA, Rary JM, Rush DS. Current concepts for the surgical management of carotid body tumor. Am J Surg 2006;191:104-10.  Back to cited text no. 5
Liapis CD, Evangelidakis EL, Papavassiliou VG, Kakisis JD, Gougoulakis AG, Polyzos AK, et al. Role of malignancy and preoperative embolization in the management of carotid body tumors. World J Surg 2000;24:1526-30.  Back to cited text no. 6
Deal JE, Sever PS, Barratt TM, Dillon MJ. Phaeochromocytoma – Investigation and management of 10 cases. Arch Dis Child 1990;65:269-74.  Back to cited text no. 7
Stackpole RH, Melicow MM, Uson AC. Pheochromocytoma in children. Report of 9 case and review of the first 100 published cases with follow-up studies. J Pediatr 1963;63:314-30.  Back to cited text no. 8
Patetsios P, Gable DR, Garrett WV, Lamont JP, Kuhn JA, Shutze WP, et al. Management of carotid body paragangliomas and review of a 30-year experience. Ann Vasc Surg 2002;16:331-8.  Back to cited text no. 9
van der Mey AG, Jansen JC, van Baalen JM. Management of carotid body tumors. Otolaryngol Clin North Am 2001;34:907-24, vi.  Back to cited text no. 10
van der Mey AG, Frijns JH, Cornelisse CJ, Brons EN, van Dulken H, Terpstra HL, et al. Does intervention improve the natural course of glomus tumors? A series of 108 patients seen in a 32-year period. Ann Otol Rhinol Laryngol 1992;101:635-42.  Back to cited text no. 11
Plukker JT, Brongers EP, Vermey A, Krikke A, van den Dungen JJ. Outcome of surgical treatment for carotid body paraganglioma. Br J Surg 2001;88:1382-6.  Back to cited text no. 12
Iafrati MD, O'Donnell TF Jr. Adjuvant techniques for the management of large carotid body tumors. A case report and review. Cardiovasc Surg 1999;7:139-45.  Back to cited text no. 13
Lees CD, Levine HL, Beven EG, Tucker HM. Tumors of the carotid body. Experience with 41 operative cases. Am J Surg 1981;142:362-5.  Back to cited text no. 14
Tekautz TM, Pratt CB, Jenkins JJ, Spunt SL. Pediatric extraadrenal paraganglioma. J Pediatr Surg 2003;38:1317-21.  Back to cited text no. 15
Dias Da Silva A, O'Donnell S, Gillespie D, Goff J, Shriver C, Rich N, et al. Malignant carotid body tumor: A case report. J Vasc Surg 2000;32:821-3.  Back to cited text no. 16
Ophir D. Familiar multicentric paragangliomas in a child. Familial multicentric paragangliomas in a child. J Laryngol Otol 1991;105:376-80.  Back to cited text no. 17
Bastounis E, Maltezos C, Pikoulis E, Leppäniemi AK, Klonaris C, Papalambros E, et al. Surgical treatment of carotid body tumours. Eur J Surg 1999;165:198-202.  Back to cited text no. 18
Wang SJ, Wang MB, Barauskas TM, Calcaterra TC. Surgical management of carotid body tumors. Otolaryngol Head Neck Surg 2000;123:202-6.  Back to cited text no. 19


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

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