Carotid body tumor pathophysiology: Difference between revisions
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:*Chief cells Arranged in distinctive pattern called cell balls (zellballen) | :*Chief cells Arranged in distinctive pattern called cell balls (zellballen) | ||
:*Separated by fibrovascular stroma and surrounded by sustentacular cells | :*Separated by fibrovascular stroma and surrounded by sustentacular cells | ||
:*The cytoplasm is pale and diffuse with occasional presence of the eosinophilic grannules.<ref>{{cite book | last = Bibbo | first = Marluce | title = Comprehensive cytopathology | publisher = Saunders/Elsevier | location = Philadelphia, PA | year = 2008 | isbn = 978-1-4160-4208-2 }}</ref> | |||
:*The nuclei are round to spindle shape. | |||
*The tumor is highly vascular. | *The tumor is highly vascular. | ||
*Although there is no well-accepted histologic criteria for the diagnosis of malignant tumors, worrisome histologic features include:<ref name="WienekeSmith2009">{{cite journal|last1=Wieneke|first1=Jacqueline A.|last2=Smith|first2=Alice|title=Paraganglioma: Carotid Body Tumor|journal=Head and Neck Pathology|volume=3|issue=4|year=2009|pages=303–306|issn=1936-055X|doi=10.1007/s12105-009-0130-5}}</ref> | *Although there is no well-accepted histologic criteria for the diagnosis of malignant tumors, worrisome histologic features include:<ref name="WienekeSmith2009">{{cite journal|last1=Wieneke|first1=Jacqueline A.|last2=Smith|first2=Alice|title=Paraganglioma: Carotid Body Tumor|journal=Head and Neck Pathology|volume=3|issue=4|year=2009|pages=303–306|issn=1936-055X|doi=10.1007/s12105-009-0130-5}}</ref> | ||
Revision as of 14:35, 4 April 2019
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Carotid body tumor Microchapters |
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Carotid body tumor pathophysiology On the Web |
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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
Anatomy
- The carotid body is an ellipsoid-like structure located in the adventitia or periadventitial tissue of the bifurcation of the common carotid artery.[1]
- The normal carotid body weighs less than 15 mg and measures between 3 to 5 mm.
- This structure innervates by the hearing branch of the glossopharyngeal nerve, however, the carotid body is in close proximity of a majority of important nerves.
- The blood supply to the carotid body is provided by ascending pharyngeal artery, which is the branch of the external carotid artery.
Physiology
- The carotid body is a chemoreceptor and its function is the monitoring of arterial blood oxygen and carbon dioxide levels.[2][3]
- This organ is sensitive to the reduction of oxygen partial pressure in the blood which results in reflexive activation of the autonomic nervous system and the resultant increase in the
ventilation rate.
Pathogenesis
- Carotid body tumor is a neuroendocrine neoplasm originating from carotid body.[4]
- Carotid body is a component of the autonomic system derived from neural crest cells of the third embryonic branchial arch.[1]
- 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.[1][5]
- 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:[1][6]
- Chronic obstructive pulmonary disease
- Cyanotic heart disease
Genetics
- The familial form of the tumor is associated with a mutation in a gene located on 11q23.[7]
- The disease inheritance is through autosomal dominance.
- 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:[8]
- Well-circumscribed with psudocapsule
- The size of the tumor vaeirs greatly and it may be as large as 10 cm
- The cutting surface is solid with a smooth, rubbery texture
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.[9]
- 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 cytoplasm is pale and diffuse with occasional presence of the eosinophilic grannules.[10]
- The nuclei are round to spindle shape.
- The tumor is highly vascular.
- Although there is no well-accepted histologic criteria for the diagnosis of malignant tumors, worrisome histologic features include:[8]
- Necrosis
- Extensive vascular or capsular invasion
- Increased mitotic activity
- Atypical mitotic figures
- The images below demonstrate gross pathology and microscopic histopathological findings of the carotid body tumor.
-
![Gross pathology of carotid body tumor[11]](/images/0/08/Carotid-body-tumour-gross-pathology.jpg)
Gross pathology of carotid body tumor[11] -
![Carotid body tumor higher magnification[12]](/images/9/98/800px-Carotid_body_tumour_2_intermed_mag.jpg)
Carotid body tumor higher magnification[12] -
![Carotid body tumor lower magnification[12]](/images/9/9b/218px-Carotid_body_tumour_2_low_mag.jpg)
Carotid body tumor lower magnification[12]
![Gross pathology of carotid body tumor[11]](/index.php/File:Carotid-body-tumour-gross-pathology.jpg)
![Carotid body tumor higher magnification[12]](/index.php/File:800px-Carotid_body_tumour_2_intermed_mag.jpg)
![Carotid body tumor lower magnification[12]](/index.php/File:218px-Carotid_body_tumour_2_low_mag.jpg)
Immunohistochemistry
- Type 1 cells, sustentacular cells, are stained by:[13]
- S-100
- Type 2 cells, cheif cell, are stained by:
- Chromogranins
- Synaptophysisn
- The tumor cells may also be positive for other markers of neuroendocrine differentiation such as:[8]
- CD56
- Neuron specific enolase
References
- ↑ 1.0 1.1 1.2 1.3 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.
- ↑ Robertson, David (2012). Primer on the autonomic nervous system. London: Elsevier. ISBN 978-0-12-386525-0.
- ↑ Zufall, Frank (2016). Chemosensory transduction : the detection of odors, tastes, and other chemostimuli. London, UK: Academic Press is an imprint of Elsevier. ISBN 978-0-12-801694-7.
- ↑ 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.
- ↑ 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.
- ↑ Fennessy, B. G.; Kozakewich, H. P. W.; Silvera, M.; Frerichs, K.; Lillhei, C. W.; Poe, D.; Rahbar, R. (2009). "The presentation and management of multiple paraganglioma in head and neck". Irish Journal of Medical Science. 180 (3): 757–760. doi:10.1007/s11845-009-0338-0. ISSN 0021-1265.
- ↑ 8.0 8.1 8.2 Wieneke, Jacqueline A.; Smith, Alice (2009). "Paraganglioma: Carotid Body Tumor". Head and Neck Pathology. 3 (4): 303–306. doi:10.1007/s12105-009-0130-5. ISSN 1936-055X.
- ↑ 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.
- ↑ Bibbo, Marluce (2008). Comprehensive cytopathology. Philadelphia, PA: Saunders/Elsevier. ISBN 978-1-4160-4208-2.
- ↑ 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
- ↑ 12.0 12.1 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
- ↑ Schmid KW, Schröder S, Dockhorn-Dworniczak B, Kirchmair R, Tötsch M, Böcker W, Fischer-Colbrie R (April 1994). "Immunohistochemical demonstration of chromogranin A, chromogranin B, and secretogranin II in extra-adrenal paragangliomas". Mod. Pathol. 7 (3): 347–53. PMID 8058707.
