Table of Contents  
Year : 2020  |  Volume : 7  |  Issue : 1  |  Page : 34-38

Diagnosis and treatment of carotid body tumors: A retrospective analysis of a single-center experience over 12 years

1 Department of Vascular Surgery, King Hussin Medical Center at Royal Medical Services, Amman, Jordan
2 Department of Histopathology, King Hussin Medical Center at Royal Medical Services, Amman, Jordan
3 Department of Otolaryngeology, King Hussin Medical Center at Royal Medical Services, Amman, Jordan

Date of Submission07-Sep-2019
Date of Decision25-Oct-2019
Date of Acceptance27-Oct-2019
Date of Web Publication16-Mar-2020

Correspondence Address:
Dr. Mohammed A Rashaideh
Department of Vascular Surgery, King Hussin Medical Center at Royal Medical Services, Amman
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijves.ijves_61_19

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Introduction: Carotid body tumor (CBT) is a neuroendocrine tumor, located at the carotid artery bifurcation. Management of such tumors is challenging due to the silent presentation and difficulties encountered during surgical excision which is the standard treatment method. Aim: This study aimed to report our experience in the diagnosis and management of CBTs. Materials and Methods: A retrospective analysis of all CBTs treated at our center during the period from January 2005 to March 2017. The analysis was based on data collected from histopathology database, hospital records, and the follow-up notes from the clinic. Results: A total of 32 CBTs were excised in 28 patients. There were 22 females and 6 males, with a mean age of 44 years. Twenty-four patients had unilateral tumors and bilateral tumors were present in four patients (15%). The mean size of the tumors was 3.5 cm. Surgical excision was achieved under general anesthesia in all cases. Intraoperatively, vascular repair in any form was needed in 41% and permanent cranial nerve injury was detected in 16% of cases. Histopathologically, incomplete excision was found in 13% of cases, and no metastasis was detected. During the follow-up period, there was only one case of recurrence. Conclusions: CBTs are challenging pathology. Despite the lack of recommendations for treatment, early resection is performed to avoid the complications associated with the increased size and the malignant transformation. Routine follow-up for all patients is advised due to the potential recurrence of these tumors.

Keywords: Carotid body tumor, chemodectoma, cranial nerve, paraganglioma, vascular tumor

How to cite this article:
Rashaideh MA, Neshwati FM, As'ad M, Ajarmeh E, Alsharayeh E, Yasin N. Diagnosis and treatment of carotid body tumors: A retrospective analysis of a single-center experience over 12 years. Indian J Vasc Endovasc Surg 2020;7:34-8

How to cite this URL:
Rashaideh MA, Neshwati FM, As'ad M, Ajarmeh E, Alsharayeh E, Yasin N. Diagnosis and treatment of carotid body tumors: A retrospective analysis of a single-center experience over 12 years. Indian J Vasc Endovasc Surg [serial online] 2020 [cited 2020 Jul 14];7:34-8. Available from:

  Introduction Top

Carotid body tumors (CBTs) are rare, slow-growing neuroendocrine tumors, originating from the neural crest, usually located at the common carotid artery (CCA) bifurcation; it is also known as paraganglioma or chemodectoma, accounting for 70% of head-and-neck paragangliomas.[1] The carotid body which is located within the adventitial tissue at the carotid bifurcation receives its blood supply from the external carotid artery (ECA), which is composed of two types of cells arranged in nests (zellballen). The carotid body detects changes in the partial pressure of arterial oxygen, carbon dioxide, pH, and temperature, and then triggers an action potential through the nerve of Hering to the glossopharyngeal nerve, which relays the information to the central nervous system.[2],[3]

CBT is usually asymptomatic, but may present as a slowly growing neck mass compressing the adjacent structures with their related symptoms. Usually, these tumors are dormant, but on rare occasions, they are functional and associated with pheochromocytoma. Classically, on physical examination, a neck mass that moves horizontally but not in the vertical plane is characteristic of CBT. There are three types of CBT, namely, familial, sporadic, and hyperplastic forms; the latter type is usually associated with chronic hypoxic states such as chronic obstructive pulmonary disease and cyanotic heart disease. CBTs are more common in patients living in high altitudes such as New Mexico, Peru, and Colorado.[4],[5] Defective succinate dehydrogenase has been found in both sporadic and familial forms as it increases the hypoxic and growth mediators.[6]

CBT is initially diagnosed using color Doppler ultrasound of the neck, showing the characteristic features of hyperechoic mass with the high color flow that is located at the carotid artery bifurcation splaying its branches [Figure 1]. Computed tomography angiography (CTA) scan is considered the preferred method for diagnosis, in which the tumor appears as an enhancing hyperintense homogenous mass with carotid splaying [Figure 2]. Magnetic resonance imaging (MRI) scan (with or without contrast) could be another alternative modality. Angiography is usually reserved for large tumors when preoperative embolization of the feeding artery is needed to shrink the tumor size and to minimize blood loss intraoperatively.[7]
Figure 1: Color Doppler ultrasound showing hyperechoic carotid body tumor (yellow arrow) located between the internal carotid artery and external carotid artery

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Figure 2: Computed tomography angiography scan axial (a) and sagittal (b), with three-dimensional reconstruction (c), showing carotid body tumor (yellow arrow) with splaying.of the internal carotid artery and external carotid artery

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The surgical excision of CBT involves exposure and control of the CCA and its branches, and then safe peri-adventitial removal of the tumor is achieved. Although it seems a simple procedure, the reconstruction of the internal carotid artery (ICA) or ligation of the ECA might be necessary in some cases. The complexity of the procedure depends on the size of the tumor, invasion of nearby structures, and expertise of the surgeon.[4]

The complications of surgical excision vary from local wound complications to cranial nerve injury and stroke, which were reported in up to 50% of cases.[8],[9] However, in the modern era with improved surgical techniques and anesthesia, the rate of complication has dropped to 2%–6%.[9]

  Materials and Methods Top

This study was approved by our institutions' ethical committee. Informed consent was waived as the data were analyzed retrospectively. The patients' data were extracted from the histology department, hospital admission records, and the clinic follow-up notes.

During the period from January 2005 to March 2017, our vascular surgery department was involved in the diagnosis and management of 32 CBTs in 28 patients. The initial diagnosis was made using color Doppler ultrasound. CTA or MRI scan was used as a definite diagnostic tool in all patients. The size and the Shamblin classification of the tumor were addressed by both a radiologist and a vascular surgeon in all cases. The medical records were reviewed for demographics, operative details, pathology, postoperative complications, survival, and tumor recurrence.

Tumor excision was done using the standard surgical exposure of the carotid artery with incision along the anterior border of sternocleidomastoid muscle, division of the fascia, platysma, and the carotid sheath along the incision. First, dissection and control of the CCA is done and then we dissect high up to isolate and control both ICA and ECA beyond the tumor; at this point, we give unfractionated heparin 3000–5000 IU intravenously. Bipolar diathermy is used to control oozes which are usually minor. Then, we dissect the tumor from nearby vessels and vital structures in periadventitial plane when possible; otherwise, subadventitial dissection is done. After excision, the mass is sent for histopathology; clamping is not necessary in most cases. At the end, we secure hemostasis and close the wound with drain in the field; heparin reversal with protamine sulfate is not often used.

  Results Top

The study group consisted of 28 patients with 32 CBTs. There were 22 females (79%) and 6 males (21%), with a male-to-female ratio of 1:3.7. The mean age at presentation of all patients was 44 years (range: 25–67 years). The average age of the female patients was 45.6 years ranging from 25 to 67, whereas the average age of males was 38 years (range: 27–55 years). The CBT was bilateral in four (15%) patients (two males and two females), with a mean age of 33 years. In four cases, there was a positive family history, out of those, two were bilateral. In the unilateral CBTs, two-thirds were left sided, whereas one-third were on the right side. [Table 1] summarizes the demographics and radiological findings of all patients with CBTs.
Table 1: Patient demographics and radiological characteristics of the carotid body tumor

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All patients presented to our vascular clinic complaining of slowly growing asymptomatic neck mass; most of them were referred from other specialties. Previous investigations with ultrasound and CT scan showed a solid mass nearby the carotid arteries. At our department, the initial modality for diagnosis in all patients was color Doppler ultrasound. Further imaging using a CTA scan was done in 27 patients and an MRI scan was needed in one patient because of a high creatinine level.

The mean size of CBTs was 3.5 cm, ranging from 1.5 to 7 cm. Using CTA and MRI scan, the tumors were classified according to the Shamblin classification,[10] with 18 (56%) CBTs were of Shamblin I, 12 (38%) were of Shamblin II, and only 2 (6%) were of Shamblin III. Surgical excision was done in all tumors with the standard carotid artery exposure; those with bilateral tumors had them removed within a 6-month interval. Preoperative selective embolization was done in two cases (in tumors with Shamblin III), and the tumors were excised within 48 h after embolization.

Intraoperatively, complete macroscopic excision with lymph node sampling and excision of all enlarged lymph nodes were achieved in all cases. Overall, vascular intervention in any form of repair was needed in 41% of cases, suture repair of the carotid bifurcation was needed in six (19%) cases, ligation of ECA in four (13%) patients, and ICA repair using interposition vein graft was done in three patients (9%).

Postoperatively, superior laryngeal nerve injury was detected in three cases (one was transient), hypoglossal nerve injury in two cases (both were temporary and recovered within 3 months), recurrent laryngeal nerve injury in two cases (both were unilateral and permanent), and one case had Horner's syndrome. Overall, all nerve injuries were detected in 25%. There was one case of mortality post surgery due to hemorrhagic stroke, which was attributed to hypertensive-encephalopathy following the removal of the second CBT in a case with bilateral tumors. Wound hematoma occurred in five cases, with only one requiring evacuation. In our study, the follow-up period ranged from 1 to 7 years with a yearly duplex scan. Only one recurrence was detected after 3 years (a female patient with bilateral CBTs and positive family history).

According to the histopathological reports, microscopic involvement of the surgical margin (incomplete excision) was found in four (13%) cases, the neural invasion was seen in four (13%), vascular invasion in four (13%), and capsular invasion in three (9%) cases. Prominent pleomorphism and mitosis were seen in only 8 (25%) cases, all less than Ki67 index of 10%. Special stains were performed in 24 cases and all were positive for chromogranin, neuron-specific enolase, synaptophysin, and S-100 protein. All lymph nodes were reactive with no evidence of metastasis. [Table 2] summarizes the intraoperative and postoperative findings in all patients.
Table 2: Intraoperative and postoperative complications and the histopathology results of carotid body tumor

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  Discussion Top

CBTs (chemodectomas, paragangliomas) are rare, originating from the neural crest and located at the CCA bifurcation; they were mostly described in the literature as case reports or case series.[11] These tumors were reported most commonly to be unilateral. In the present study, 28 patients presented with unilateral tumors and four (15%) had bilateral tumors. Grotemeyer et al. reported 30 patients with unilateral and 6 (17%) with bilateral tumors, and Fruhmann et al. reported 37 patients with unilateral and 10 with bilateral tumors, whereas Gad et al. studied 42 paraganglioma patients including only two bilateral cases.[12],[13],[14] In this study, the average age was 44 years, which is comparable to the average age of the patients in the studies by Dorobisz et al. and Gad et al., which was 45 years.[11],[14] While in the study by Fruhmann et al., the average age was 55 years.[13] CBTs are more common in females; in the current study, the female-to-male ratio was 3.7:1, Kruger et al. and Makeieff et al. reported a ratio of 2:1.[15],[16] On the other hand, according to Luna-Ortiz et al., the ratio was 31:1.[17] While in the study of 29 patients by O'Neill et al., there was no gender difference in the incidence of CBT.[18] Positive family history of CBT was reported in the literature from 0% to 28%.[19] In our study, only four (15%) cases had a positive family history.

There is a lack of rigid guidelines in the literature for the diagnosis and treatment of CBTs. Diagnoses are based on imaging modalities such as color Doppler ultrasound, CTA, and MRI.[14] Although the clinical value of angiography in CBT is controversial,[19],[20] it is usually used to assess the vessels supplying the tumor and for preoperative embolization.[4],[17] According to Makeieff et al., embolization might increase the risk of complications during surgery.[16] In the present study, all patients were diagnosed initially using ultrasound, whereas CTA or MRI was used to address the tumor size and Shamblin type. Angiography with embolization was done only for the Shamblin III CBTs. Higher Shamblin class tumors were associated with greater blood loss, longer operative times, higher incidence of nerve injury, and the need for vascular sacrifice and reconstruction.[17],[21] The average size of CBTs in our study was 3.5 cm; the number of patients in Shamblin groups I/II/III, respectively, was 18/12/2. Fruhmann et al. and Makeieff et al. addressed in their study that most of the CBTs were in the groups of Shamblin I and II,[13],[16] whereas in the study by Lim et al., most of the tumors were Shamblin III, in which, the rate of persistent cranial nerves injury was relatively higher.[21]

The current recommendation for CBT treatment is surgical excision unless the patient is not a candidate for safe surgical exploration where observation is the only possible approach.[21],[22],[23] In this series, all patients were operated using the standard surgical approach for carotid endarterectomy. The rate of all cranial nerve injuries was 25%: three of them were reversible; accordingly, the rate of permanent cranial nerve injuries was 15%. In comparison to other studies, Makeieff et al. reported a rate of 14% and Lim et al. reported a rate of 23% of permanent nerve paralysis. In our series, one patient had a hemorrhagic stroke which was attributed to hypertensive encephalopathy following the removal of the second CBT in a case with bilateral tumors, with no reported ischemic strokes. In some reports, the rate of perioperative stroke was as high as 11%.[17]

Postoperatively, all our patients were on regular follow-ups using color Doppler ultrasound examination; only one patient had a recurrence after 3 years of surgery. Fruhmann et al. recommended regular checkups and ultrasound scan for all patients, whereas whole-body MR angiography was added for patients with genetic mutations.[13]

  Conclusions Top

CBTs are considered a challenge for the surgeons regarding diagnosis and management. Despite the lack of recommendations for treatment in the literature, early surgical resection is performed to avoid the complications associated with the increased size and to prevent malignant transformation. Preoperative embolization is reserved for large and Shamblin type III tumors to facilitate complete and safe excision. Routine follow-up for all patients is advised due to the potential recurrence of these tumors.

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  References Top

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  [Table 1], [Table 2]


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