Carotid body tumor pathophysiology
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sahar Memar Montazerin, M.D.[2]
Overview
Pathophysiology
Physiology
The normal physiology of [name of process] can be understood as follows:
Pathogenesis
- Carotid body tumor is a neuroendocrine neoplasm originating from carotid body.[1]
- Carotid body is a component of the autonomic system derived from neural crest cells of the third embryonic branchial arch.[2]
- The overgrowth of chemoreceptor paraganglioma cells in the carotid body leads to the formation of carotid body tumor.
- Carotid body tumors are normally located in the medial aspect adventitia of the carotid bifurcation.
- Carotid body tumor occurs sporadicly in 85% to 90% of the cases.[2][3]
- The tumor may be bilateral in fewer than 5% of the sporadic cases.
- The tumor may be familial in the rest 10% to 15%.
- In familial cases, it may be bilateral in 30% to 40% of the cases.
- It has been explained that the hyperplastic form of the tumor is associated with chronic hypoxia due to such as:[2][4]
- Chronic obstructive pulmonary disease
- Cyanotic heart disease
Genetics
- The genetic mutations associated with the development of carotid body tumor, include:
- MEN 2A
- MEN 2B
- Chromosome 3p25.5
Associated Conditions
Conditions associated with [disease name] include:
Gross Pathology
- On gross pathology, characteristic findings of carotid body tumor, include:
- Dusky color
- Highly vascular mass
Microscopic Pathology
- On microscopic histopathological analysis, carotid body tumor composed of:
- The chief or paraganglionic cells composing the predominant part of the tumor and contain eosinophilic grannular materials and oval or round nuclei.[5]
- The supporting or sustentacular cells responsible for the chemoreceptor activity of the carotid body
- The carachteristic finding of this tumor is:
- Chief cells Arranged in distinctive pattern called cell balls (zellballen)
- Separated by fibrovascular stroma and surrounded by sustentacular cells
- The tumor is highly vascular.
- Positive for chromogranin, synaptophysin, neuron-specific enolase, serotonin, neurofilament and neural cell adhesion molecule; they are S-100 protein negative.
- The sustentacular cells are S-100 positive and focally positive for glial fibrillary acidic protein.
- By histochemistry, the paraganglioma cells are argyrophilic, periodic acid Schiff negative, mucicarmine negative, and argentaffin negative.
- The images below demonstrate gross pathology and microscopic histopathological findings of the carotid body tumor.
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![Gross pathology of carotid body tumor[6]](/images/0/08/Carotid-body-tumour-gross-pathology.jpg)
Gross pathology of carotid body tumor[6]
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![Carotid body tumor higher magnification[7]](/images/9/98/800px-Carotid_body_tumour_2_intermed_mag.jpg)
Carotid body tumor higher magnification[7]
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![Carotid body tumor lower magnification[7]](/images/9/9b/218px-Carotid_body_tumour_2_low_mag.jpg)
Carotid body tumor lower magnification[7]
![Gross pathology of carotid body tumor[6]](/index.php/File:Carotid-body-tumour-gross-pathology.jpg)
![Carotid body tumor higher magnification[7]](/index.php/File:800px-Carotid_body_tumour_2_intermed_mag.jpg)
![Carotid body tumor lower magnification[7]](/index.php/File:218px-Carotid_body_tumour_2_low_mag.jpg)
References
- ↑ Boedeker CC, Ridder GJ, Schipper J (2005). "Paragangliomas of the head and neck: diagnosis and treatment". Fam. Cancer. 4 (1): 55–9. doi:10.1007/s10689-004-2154-z. PMID 15883711.
- ↑ 2.0 2.1 2.2 Burgess, Alfred; Calderon, Moises; Jafif-Cojab, Marcos; Jorge, Diego; Balanza, Ricardo (2017). "Bilateral carotid body tumor resection in a female patient". International Journal of Surgery Case Reports. 41: 387–391. doi:10.1016/j.ijscr.2017.11.019. ISSN 2210-2612.
- ↑ Ridge, Brian A.; Brewster, David C.; Darling, R. Clement; Cambria, Richard P.; LaMuraglia, Glenn M.; Abbott, William M. (1993). "Familial Carotid Body Tumors: Incidence and Implications". Annals of Vascular Surgery. 7 (2): 190–194. doi:10.1007/BF02001015. ISSN 0890-5096.
- ↑ Sajid, M.S.; Hamilton, G.; Baker, D.M. (2007). "A Multicenter Review of Carotid Body Tumour Management". European Journal of Vascular and Endovascular Surgery. 34 (2): 127–130. doi:10.1016/j.ejvs.2007.01.015. ISSN 1078-5884.
- ↑ Patetsios, Peter; Gable, Dennis R.; Garrett, Wilson V.; Lamont, Jeffrey P.; Kuhn, Joseph A.; Shutze, William P.; Kourlis, Harry; Grimsley, Bradley; Pearl, Gregory J.; Smith, Bertram L.; Talkington, C.M.; Thompson, Jesse E. (2002). "Management of Carotid Body Paragangliomas and Review of a 30-year Experience". Annals of Vascular Surgery. 16 (3): 331–338. doi:10.1007/s10016-001-0106-8. ISSN 0890-5096.
- ↑ Carotid body tumor. Dr Yuranga Weerakkody and Assoc Prof Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/carotid-body-tumour. Accessed on December 7, 2015
- ↑ 7.0 7.1 Carotid body tumor. Wikipedi 2015. Accessed on November 23, 2015. https://en.wikipedia.org/wiki/Paraganglioma#/media/File:Carotid_body_tumour_2_low_mag.jpg
