Carotid body tumor overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:
Overview
Historical Perspective
Carotid body was first described by Von Haller in 1743. From 1930, it has been accepted that monitoring of arterial blood oxygen is its main function. Carotid body tumor was first described by Bungeler in 1952. The familial form of the carotid body tumor was first described by Chase in 1993. In 1889, The first surgical excision of the tumor without complication was done by Dr. Albert.
Classification
Carotid body tumor is classified into sporadic, familial and hyper-plastic form. According to Shamblin surgical classification, carotid body tumor divided into three subtypes which includes: class I that localized with minimal vascular attachment, class II that partially surrounds carotids and class III which encases carotids as well.
Pathophysiology
The carotid body is an ellipsoid-like structure located in the adventitia or periadventitial tissue of the bifurcation of the common carotid artery. This organ is a chemo-receptor and its function is the monitoring of arterial blood oxygen and carbon dioxide levels. The exact pathogenesis of carotid body tumor is not fully understood. however, the overgrowth of chemoreceptor paraganglioma cells in the carotid body leads to the formation of carotid body tumor. Recent litreture has suggested the role of several germline mutations in the pathogenesis of this tumor. Also, the hyperplastic form of the tumor has been associated with conditions that causes chronic hypoxia.
Causes
Carotid body tumor may be caused by familial syndromes, germline mutation of several genes, or chronic hypoxia.
Differentiating Xyz from Other Diseases
Carotid body tumor must be differentiated from other diseases that cause neck mass including schwannomas and lymphadenopathy.
Epidemiology and Demographics
The incidence of carotid body tumor is less than 3 in 100,000 individuals. It is the most common paragnaglioma of the head and neck and comprises approximately 65% of paragangliomas. The prevalence of head and neck paraganglioma is 3% of all paraganglioma. This tumor is more commonly observed in the adults and particularly in their fifth decade of life. It affects both gender equally.