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Carotid Body Tumors

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Carotid body tumors arise in the internal or external carotid arteries. Read the article to learn about them in detail.

Medically reviewed by

Dr. Sapkal Ganeshrao Patilba

Published At February 10, 2022
Reviewed AtMay 10, 2024

What Is Carotid Artery Function?

Most paraganglia within the head and neck region are associated with the normal functions of the parasympathetic nervous system. The most common site of extra-adrenal paragangliomas usually arises within the bifurcation of the external and internal carotid arteries. Besides being the major blood suppliers to the brain, neck, and face, these carotid arteries are also bilaterally in the neck region. Like most major arteries, the carotid artery comprises three tissue layers, namely,

  • The smooth inner layer (intima).

  • The muscular middle layer (media).

  • The outer layer (adventitia).

The carotid sinus is a significant branch point where this artery undergoes the most widening and is referred to as the carotid bulb. The bulb or sinus has receptors or sensor regulators for blood pressure. The same pulse of this arterial supply is felt in the neck region for physicians to check the pulse (with physicians' fingertips usually pressed against the trachea region of the neck).

Von Hippel Lindau syndrome and neurofibromatosis type 1 are also identified as posing a genetic or hereditary inheritance in patients suffering from carotid body tumors (CBTs). Most paragangliomas, apart from CBTs, are usually traced to a congenital or hereditary form of familial tracing, though more evidence is needed to prove this in the literature.

What Is the Clinical Significance of Carotid Body Tumors?

A weak spot, area, or an inflamed or narrowed region in the carotid artery can lead to life-threatening medical conditions like carotid artery vasculitis, embolism, stenosis, stroke, and hypersensitivity. As the carotid bulb receptors function to regulate blood pressure, which is a vital sign of human consciousness, the conditions arising in the carotid artery can be life-threatening and severe.

Also, CBTs can be due to various causative factors, from autoimmune diseases to atherosclerosis or blood pressure-related conditions. The vascular tumors arising within the carotid artery can be very challenging for both the physicians and the patient because of the significant and extensive vascularity of the artery. Although it is estimated that less than ten percent of carotid body tumors may be malignant, studies show the rate of cancerous potential or malignancy may be up to 50 percent in the affected patients. Most cases are evidence of negligence by the patient as the clinical symptoms of these tumors may be ignored for years together, given the slow growth and painless clinical presentation of the tumors.

On clinical examination, the physician mainly recognizes this tumor by the apparent "Fontaine" sign—wherein the tumor mass appears vertically fixed over the bifurcation of the common carotid artery. CBTs are commonly associated with vagal body tumors and cranial nerve palsy. Ten percent of patients suffering from CBTs may only realize they suffer from it once they are diagnosed with the paralysis of spinal accessory, hypoglossal, glossopharyngeal, or recurrent laryngeal nerves.

Though they may go unnoticed for a long time, the clinical features of these tumors are mainly pain, hoarse voice, dysphagia (difficulty swallowing food), and shoulder drop. The compression of the artery as the tumor enlarges may create further clinical symptoms like paresis of the tongue (impairment of tongue muscles or movements).

What Is the Differential Diagnosis of Carotid Body Tumors?

Before confirming CBTs, these other conditions should be cross-checked by the physician:

Though histologic differentiation of these lesions is challenging, immunohistochemistry and microscopic analysis by the pathologist will help in a precise diagnosis of these lesions, which show distinctive histologic patterns. The differential diagnosis for these lesions can be established by examining the lesion mainly based on its location or occurrence point in the neck. However, the gold standard for diagnosis remains the MRI (magnetic resonance imaging) scan of the carotid artery, which thus reflects the tumor upon radiographic imaging.

What Are the Histological and Radiographic Features of Carotid Body Tumors?

Radiologically, on an MRI accompanied by contrast imaging (as contrast enhances the vascular component of these lesions), the tumors display a characteristic salt-and-pepper appearance with low signal and high signal foci of hemorrhage within the internal or external carotid artery. Some physicians also recommend nuclear imaging scans to differentiate the tumor using the somatostatin analog indium 111-octreotide, mainly for detecting more than 1. 5 centimeters seen as hypervascular masses.

CT (computed tomography) and catheter angiography may also be recommended to determine the same. However, even post-surgery in these individuals, octreotide nuclear detection is proper to check for remnant tumors and scar detection after surgery. Histologically, the appearance of the "Zellballen" cell nest pattern in these well-differentiated tumors, with basophilic cytoplasm and hyperchromatic nuclei with scattered chromatin, is diagnostic of CBTs. Pathologically, the cells undergo extensive necrosis, increased mitotic activity, and S-100 protein loss in the later stages of this tumor.

How Are Carotid Body Tumors Diagnosed?

Medical professionals use imaging tests to diagnose carotid body cancers, such as:

How Are Carotid Body Tumors Managed?

The first preventive measure for the affected individual is to recognize clinical features like pain, pressure, and compression in the neck region, along with shoulder drop or blood pressure changes. This often helps in the early diagnosis and detection of carotid body tumors by the physician (who mainly checks for the painless, often undetected mass in the carotid artery region, even at an early stage).

Pre-operative adrenergic blockade and surgical resection of these paragangliomas are the primary gold standard treatments, though it would prove a challenging surgery in severe malignant widespread tumors. Though CBTs have a five to ten-year survival rate after resection of the lesion, the post-operative prognosis usually depends on early detection, prevention of recurrence (which is common) by frequent follow-up, and radiographic imaging of the carotid region. These patients do not usually require chemotherapy or radiation therapy as they are not significantly impactful in preventing the recurrence of the lesions.

Does Treating a Carotid Body Tumor Have Any Side Effects?

Treatment for a carotid body tumor often results in no side effects. Treatment for carotid body tumors, however, may include numerous blood arteries. Following therapy, some patients might have:

  • Dysphagia, or difficulty swallowing.

  • Damage to their brain nerves.

  • Issues with the surgical site's ability to heal.

  • Stroke.

How to Lower the Chance of Developing a Carotid Body Tumor?

The risk of developing a carotid body tumor cannot be avoided or decreased. Consult a physician about the probability that there is a genetic family record of carotid body tumors.

What to Expect if One Has a Carotid Body Tumor?

After discussing the signs of the tumor with patients, the physician will advise a course of action based on the size of the tumor. If the physician advises watching the tumor, notify them as soon as any new symptoms arise. Usually, no more care is required after surgery to remove a tumor from the carotid body.


To conclude, carotid body tumors should be detected early, and physician guidance with proper diagnostic imaging and histochemical studies will ensure long-term survival rates for patients suffering from this severe neoplasm.

Frequently Asked Questions


What Is the Treatment for Carotid Body Tumors?

The treatment for carotid body tumors includes surgical management of tumors. This involves transcatheter embolization and bypass graft (if needed). Transcatheter embolization is a process by which blood supply to the tumor is reduced. It is usually done two to three days before surgery. Then the surgical removal of the tumor is done. Surgical removal may or may not involve the removal of a small part of the carotid artery.


What Is the Incidence of Carotid Body Tumors in Humans?

Carotid body tumors are a rare cause that contributes to lumps in the neck. Its incidence is less than 1 in 300000. It is more common in females, and chances of occurrence of carotid body tumors in age less than 20 is rare.


What Is the Size of a Carotid Tumor?

The size of the carotid tumor can vary. There are cases in which the tumor size was more than 10 cm. When the tumor enlarges, it may compress the surrounding nerves and can cause pain, hoarseness, and dysphagia.


How Serious Is a Carotid Body Tumor?

Carotid body tumors are generally not life-threatening. But when they increase in size, they can compress the nerves and blood vessels and can turn symptomatic.


Are Carotid Tumors Benign or Malignant?

Carotid tumors are rare tumors. They are often benign in nature. However, in some cases, it may turn malignant. There is no relationship between clinical behavior and histologic findings in those cases.
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Dr. Achanta Krishna Swaroop
Dr. Achanta Krishna Swaroop



vascular tumorscarotid body tumor
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