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] Maria Fernanda Villarreal, M.D. [3]


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.




ventilation rate.



Associated Conditions

Conditions associated with carotid body tumor include:[6]

Gross Pathology

|Characteristic findings of carotid body tumor, include:[6]

    • Well-circumscribed with psudocapsule
    • The size of the tumor varies greatly and it may be as large as 10 cm
    • The cutting surface is solid with a smooth, rubbery texture
Contributed by Paweł Kuźniar in wikimedia.commons

Microscopic Pathology

  • 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 granules.[13]
  • The nuclei are round to spindle shape.
Contributed by Sarahkayb in wikimedia.commons



  1. 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.
  2. Robertson, David (2012). Primer on the autonomic nervous system. London: Elsevier. ISBN 978-0-12-386525-0.
  3. 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.
  4. 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.
  5. 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.
  6. 6.0 6.1 6.2 6.3 6.4 6.5 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.
  7. 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.
  8. 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.
  9. Young AL, Baysal BE, Deb A, Young WF (September 2002). "Familial malignant catecholamine-secreting paraganglioma with prolonged survival associated with mutation in the succinate dehydrogenase B gene". J. Clin. Endocrinol. Metab. 87 (9): 4101–5. doi:10.1210/jc.2002-020312. PMID 12213855.
  10. Davila, Victor J.; Chang, James M.; Stone, William M.; Fowl, Richard J.; Bower, Thomas C.; Hinni, Michael L.; Money, Samuel R. (2016). "Current surgical management of carotid body tumors". Journal of Vascular Surgery. 64 (6): 1703–1710. doi:10.1016/j.jvs.2016.05.076. ISSN 0741-5214.
  11. Carney JA, Sheps SG, Go VL, Gordon H (June 1977). "The triad of gastric leiomyosarcoma, functioning extra-adrenal paraganglioma and pulmonary chondroma". N. Engl. J. Med. 296 (26): 1517–8. doi:10.1056/NEJM197706302962609. PMID 865533.
  12. 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.
  13. Bibbo, Marluce (2008). Comprehensive cytopathology. Philadelphia, PA: Saunders/Elsevier. ISBN 978-1-4160-4208-2.
  14. 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.