Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Carotid Body Tumors Treatment & Management

  • Author: Mohamad R Chaaban, MD, MBA, MSCR, FACS, FAAOA; Chief Editor: Arlen D Meyers, MD, MBA  more...
 
Updated: May 09, 2016
 

Surgical Therapy

Carotid body tumors (CBTs) are treated with either surgery or radiotherapy. When choosing treatment, consider the following factors: presence of other paragangliomas, bilateral carotid body tumors, the age and the health of the patient, and the patient's preference.[26]

Surgical treatment of carotid body tumors can be associated with significant morbidities, especially with large Shamblin III tumors (greater than 5 cm).[27] For this reason, patients who have poor medical conditions, elderly patients, or patients with recurrent tumors are usually treated with radiation therapy.

Preoperative evaluation is extremely important to avoid major surgical complications. The use of MRI and genetic testing in susceptible families have allowed earlier detection of multicentric tumors in many patients. Operative risk is directly related to the size and extent of the tumor. Preoperative embolization is still controversial, although it has been used to decrease the risk of intraoperative bleeding, particularly in larger tumors.[28]

Radiotherapy as a primary modality of treatment for carotid body tumors has also been heavily debated because some investigators have found that these tumors are not radiosensitive and have reported regrowth after suppression. The tumors that are treated primarily with radiation are also difficult to resect afterwards because of radiation-induced fibrosis. Other investigators have reported no cure rates for patients who received radiation for their carotid body tumors. The primary goal of radiotherapy is to slow or halt progression.

For these reasons, surgery is usually the treatment modality of choice for younger, healthier patients with carotid body tumors (CBTs), and radiotherapy is reserved for the elderly, patients who are poor surgical candidates, and selected individuals with multiple paragangliomas in whom resection may be highly morbid.

Next

Preoperative Details

Carotid body tumors are rarely associated with pheochromocytomalike symptoms, but checking urinary catecholamines, vanillylmandelic acid (VMA), and metanephrines is helpful and is routine in many centers.

Preoperative imaging is necessary to evaluate the extent of the disease and its multiplicity. If catacholamine levels are elevated, an evaluation for adrenal pheochromocytomas should be performed. If detected these tumors should be removed prior to the carotid surgery.

Carotid balloon test occlusion is performed in patients who are at a high risk of carotid resection due to the tumor’s involvement. In such cases, consultation with a vascular surgeon and a neurologist for intraoperative EEG monitoring may be necessary.

In addition to the above testing, discussion with the patient about the postoperative complications is extremely important; this includes the risks of cranial nerve injuries (IX, X, XI, XII), bleeding, infection, the possibility of a carotid bypass or vein grafts, stroke, and death.[29]

Embolization of carotid body tumors has been a controversial topic. It has been recommended by some surgeons when the size of the tumor exceeds 4 cm in size. Blood loss appears to be less with prior embolization of larger tumors. Bilateral arteriography can also be performed to delineate the vascularity of the tumor and to detect any tumors on the contralateral side.

A study by Zhang et al indicated that preoperative tumor embolization, carried out less than 48 hours before surgery, can significantly reduce blood loss, surgical time, and length of hospital stay in patients undergoing carotid body tumor resection. The investigators warned that embolization should be carried out only in vessels that can be subselectively catheterized and that do not permit contrast medium to freely reflux into the internal carotid artery. The study involved 32 patients, including 21 patients who underwent embolization and 11 who did not.[30]

In contrast, a literature review by Abu-Ghanem et al indicated that in carotid body tumor resection, preoperative embolization is no more effective than nonembolization in terms of estimated blood loss, length of surgery or of hospital stay, or risk of stroke, cranial nerve injury, or vascular injury.[31]

Previous
Next

Intraoperative Details

Effective communication between the surgeon and the anesthesiologist is of utmost importance throughout the surgery. Whether the patient is totally relaxed is the surgeon's preference. Some surgeons prefer paralysis to aid in muscle retraction and to facilitate the use of electrocautery. Others prefer the patient not be paralyzed, which, they feel, aids in the identification of the cranial nerves.

Different types of neck incisions have been described. The incision chosen is usually based on tumor size and extent. A horizontal incision in the mid neck affords excellent exposure both superiorly and inferiorly and typically provides excellent cosmesis. Some surgeons prefer an endarterectomy approach using a transverse cervical incision along the anterior border of the sternocleidomastoid. For larger tumors, many incisions have been described, including preauricular extension of the incision for tumors extending to the infratemporal fossa.

The most important step in tumor removal is superior and inferior control of the blood vessels. This includes identification of the internal jugular vein, common, and internal carotid arteries and placing vessel loops on each. (In a report on 11 patients with Shamblin II or III carotid body tumors, Spinelli et al described a surgical technique in which resection of the tumors from the internal carotid artery was performed bloodlessly by clamping the external carotid artery [at its origin and distal branches] but not the internal carotid artery.[32] )

In cases in which the tumor is not involving the hypoglossal and vagus nerve, these nerves should be exposed and followed cranially. Identification of the accessory and glossopharyngeal nerve is also performed; in certain cases, the digastric muscle must be sacrificed for better exposure.

The small feeder vessels, together with some branches from the external carotid artery, are ligated. Dissection between the external and internal carotid artery exposes the larger feeding vessels, including the ascending pharyngeal artery, which is the main feeding vessel most of the time. This is followed by a craniocaudal dissection of the tumor from the carotid vessels. In tumors that are identified intraoperatively as Shamblin type III, the plane of dissection can be found on either the dorsolateral side of the internal carotid or the ventrolateral side of the external carotid.

Dissection of the carotid body tumor is performed in the subadventitial plane using either loupes or microscope, as described by Gordon-Taylor. This is supplemented with the use of bipolar cautery, which limits blood loss and controls bleeding.

Any injury to the carotid vessel requiring clamping of the common or internal arteries needs temporary heparinization with a low risk of complications; this is often followed by vascular reconstruction. van der Mey reported that 65% of patients who had their internal carotid artery ligated had a stroke and that 25% of patients who passed their balloon occlusion test had a delayed stroke.[33]

During removal of the carotid body tumor, care should be taken once again to avoid injuring the nerves, especially the superior laryngeal nerve, which has been reported to be the most injured nerve during dissection. This nerve is frequently involved with tumor posteriorly. Ideally, it can be identified and dissected as it exits the vagus nerve and courses medially towards the larynx.

Previous
Next

Postoperative Details

In the immediate postoperative period, the patient should be carefully observed for any complication of the procedure, including postoperative hemorrhage or late stroke. In patients who underwent vascular reconstruction, ICU admission is recommended, along with heparinization.

Previous
Next

Follow-up

Patients should be closely observed for any local recurrence, although these are usually rare. If the patient has no morbidity from the initial surgery, contralateral tumors should also be resected. In patients with significant permanent morbidities to cranial nerves, radiation to the contralateral side might be considered. As always, a careful weighing of pros and cons must be done and presented to the patient. Operating on small tumors has a very low rate of complications and the long-term effects of radiation on younger patients is potentially a contraindication.

Previous
Next

Complications

Careful subadventitial dissection of the tumor and control of the proximal and distal carotids minimizes vascular complications. Of note is that the size of the tumor and involvement of the carotid artery predict the vascular complication risks. The highest risk is observed in tumors larger than 5 cm and/or grade 3 by Shamblin classification. These patients with large tumors also have more frequent postoperative cranial nerve injuries.[25]

The most commonly injured nerve is the superior laryngeal nerve. This nerve supplies the cricothyroid muscle and provides sensation to the supraglottic larynx. The patient postoperatively might suffer from some degree of aspiration and voice changes (inability to create high-pitched sounds).

Injury to the vagus nerve results in vocal cord paralysis with resultant hoarseness and increased aspiration risk. When combined with a superior nerve paralysis, as is the case with a high vagal injury, aspiration is a significant problem because the larynx not only does not function well but is also anesthetic. This may be compensated by the contralateral vocal cord over time. If the problem persisted, then vocal cord medialization procedures should be performed.

Speech and swallowing problems result from a hypoglossal nerve injury. If the nerve is accidentally cut, primary reanastomosis should be attempted first. If primary anastomosis fails, then other options include a greater auricular nerve graft.

Postoperative shoulder pain and weakness is typically a result of an accessory nerve injury. This results in significant disability for the patient.

First bite syndrome is another complication that occurs when the sympathetic supply to the ipsilateral parotid gland is severed. The resultant parotid gland has an unabated parasympathetic supply. To date, no successful treatment of this syndrome exists.

In cases of bilateral tumor excision with loss of the bilateral Hering nerves, patients experience labile blood pressure postoperatively, which is difficult to control medically. Concurrent excision of bilateral carotid body tumors should, therefore, be avoided, although staging the surgeries might help because of the compensation provided by the aortic receptors.

Previous
 
Contributor Information and Disclosures
Author

Mohamad R Chaaban, MD, MBA, MSCR, FACS, FAAOA Assistant Professor, Department of Otolaryngology, Section of Rhinology, Sinus and Skull Base Surgery, University of Texas Medical Branch at Galveston

Mohamad R Chaaban, MD, MBA, MSCR, FACS, FAAOA is a member of the following medical societies: Alpha Omega Alpha, American Academy of Allergy Asthma and Immunology, American Academy of Otolaryngic Allergy, American Academy of Otolaryngology-Head and Neck Surgery, American College of Surgeons, American Rhinologic Society, European Rhinologic Society, North American Skull Base Society

Disclosure: Nothing to disclose.

Coauthor(s)

Kerstin M Stenson, MD Professor, Department of Surgery, Section of Otolaryngology-Head and Neck Surgery, Director, Head and Neck Program, University of Chicago Division of the Biological Sciences, The Pritzker School of Medicine

Kerstin M Stenson, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Otolaryngology-Head and Neck Surgery, American College of Surgeons, American Medical Association, Association for Surgical Education, Chicago Medical Society, Society of University Otolaryngologists-Head and Neck Surgeons, American Society of Clinical Oncology

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Arlen D Meyers, MD, MBA Professor of Otolaryngology, Dentistry, and Engineering, University of Colorado School of Medicine

Arlen D Meyers, MD, MBA is a member of the following medical societies: American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, American Head and Neck Society

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Cerescan;RxRevu;SymbiaAllergySolutions<br/>Received income in an amount equal to or greater than $250 from: Symbia<br/>Received from Allergy Solutions, Inc for board membership; Received honoraria from RxRevu for chief medical editor; Received salary from Medvoy for founder and president; Received consulting fee from Corvectra for senior medical advisor; Received ownership interest from Cerescan for consulting; Received consulting fee from Essiahealth for advisor; Received consulting fee from Carespan for advisor; Received consulting fee from Covidien for consulting.

Additional Contributors

William M Lydiatt, MD Professor and Division Director, Head and Neck Surgical Oncology, Department of Otolaryngology-Head and Neck Surgery, University of Nebraska Medical Center

William M Lydiatt, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Otolaryngology-Head and Neck Surgery, American College of Surgeons, American Head and Neck Society, Nebraska Medical Association

Disclosure: Nothing to disclose.

References
  1. Georgiadis GS, Lazarides MK, Tsalkidis A, Argyropoulou P, Giatromanolaki A. Carotid body tumor in a 13-year-old child: Case report and review of the literature. J Vasc Surg. 2008 Apr. 47(4):874-880. [Medline].

  2. Sajid MS, Hamilton G, Baker DM;. A multicenter review of carotid body tumour management. Eur J Vasc Endovasc Surg. Aug/2007. 34:127-30. [Medline]. [Full Text].

  3. Shamblin WR, ReMine WH, Sheps SG, Harrison EG Jr. Carotid body tumor (chemodectoma). Clinicopathologic analysis of ninety cases. Am J Surg. 1971 Dec. 122(6):732-9. [Medline].

  4. Mitchell RO, Richardson JD, Lambert GE. Characteristics, surgical management, and outcome in 17 carotid body tumors. Am Surg. 1996 Dec. 62(12):1034-7. [Medline].

  5. Naughton J, Morley E, Chan D, Fong Y, Bosanquet D, Lewis M. Carotid body tumours. Br J Hosp Med (Lond). 2011 Oct. 72(10):559-64. [Medline].

  6. H Martin. Surgery of Head and Neck Tumors. Surgery of Head and Neck Tumors. New York: Hoeber-Harper Books; 1957.

  7. Sevilla Garcia MA, Llorente Pendas JL, Rodrigo Tapia JP, Garcia Rostan G, Suarez Fente V, Coca Pelaz A. [Head and neck paragangliomas: revision of 89 cases in 73 patients]. Acta Otorrinolaringol Esp. 2007 Mar. 58(3):94-100. [Medline].

  8. Kotelis D, Rizos T, Geisbusch P, Attigah N, Ringleb P, Hacke W, et al. Late outcome after surgical management of carotid body tumors from a 20-year single-center experience. Langenbecks Arch Surg. 2009 Mar. 394(2):339-44. [Medline].

  9. Jani P, Qureshi AA, Verma S, Walker L. Familial carotid body tumours: is there a role for genetic screening?. J Laryngol Otol. 2008 Sep. 122(9):978-82. [Medline].

  10. JL Netterville, KM Reilly D, Robertson, ME Reiber, WB Armstrong and P. Childs. Carotid body tumors: a review of 30 patients with 46 tumors. Laryngoscope. 1995. 105:114-126.

  11. Karatas E, Sirikci A, Baglam T, Mumbuc S, Durucu C, Tutar E. Synchronous bilateral carotid body tumor and vagal paraganglioma: a case report and review of literature. Auris Nasus Larynx. 2008 Mar. 35(1):171-5. [Medline].

  12. Gardner P, Dalsing M, Weisberger E, Sawchuk A, Miyamoto R. Carotid body tumors, inheritance, and a high incidence of associated cervical paragangliomas. Am J Surg. 1996 Aug. 172(2):196-9. [Medline].

  13. Barnes L, Tse LLY, Hunt JL. Carotid Body Paragangliomas. Pathology and Genetics of Head and Neck Tumors. IARC. 2005. 364-365.

  14. Baysal BE, Myers EN. Etiopathogenesis and clinical presentation of carotid body tumors. Microsc Res Tech. 2002 Nov 1. 59(3):256-61. [Medline].

  15. de Franciscis S, Grande R, Butrico L, et al. Resection of Carotid Body Tumors reduces arterial blood pressure. An underestimated neuroendocrine syndrome. Int J Surg. 2014. 12 Suppl 1:S63-7. [Medline].

  16. Jansen JC, van den Berg R, Kuiper A, van der Mey AG, Zwinderman AH, Cornelisse CJ. Estimation of growth rate in patients with head and neck paragangliomas influences the treatment proposal. Cancer. 2000 Jun 15. 88(12):2811-6. [Medline].

  17. Isik AC, Imamoglu M, Erem C, Sari A. Paragangliomas of the head and neck. Med Princ Pract. 2007. 16(3):209-14. [Medline].

  18. Ghoreishi M, Akbar-Beigi A, Tahery D, Sehhat S. Fever as the main presenting symptom of a carotid body tumor. Arch Iran Med. 2008 Mar. 11(2):214-7. [Medline].

  19. Lack EE. Anatomy and physiology of peripheral arterial chemoreceptors. Pathology of adrenal and extra-adrenal paraganglia. Philadelphia: W.B Saunders; 1-14.

  20. Maxwell JG, Jones SW, Wilson E, Kotwall CA, Hall T, Hamann S. Carotid body tumor excisions: adverse outcomes of adding carotid endarterectomy. J Am Coll Surg. 2004 Jan. 198(1):36-41. [Medline].

  21. Terry A. Day John K. Joe. Primary Neoplasms of the neck. Cummings: Otolaryngology: Head & Neck Surgery. 4th. St Louis: Elsevier-Mosby; 2005. 113.

  22. Knight TT Jr, Gonzalez JA, Rary JM, Rush DS. Current concepts for the surgical management of carotid body tumor. Am J Surg. 2006 Jan. 191(1):104-10. [Medline].

  23. Straughan DM, Neychev VK, Sadowski SM, et al. Preoperative Imaging Features are Associated with Surgical Complications Following Carotid Body Tumor Resection. World J Surg. 2015 Aug. 39 (8):2084-9. [Medline].

  24. Rosa M, Sahoo S. Bilateral carotid body tumor: the role of fine-needle aspiration biopsy in the preoperative diagnosis. Diagn Cytopathol. 2008 Mar. 36(3):178-80. [Medline].

  25. Arya S, Rao V, Juvekar S, Dcruz AK. Carotid body tumors: objective criteria to predict the Shamblin group on MR imaging. AJNR Am J Neuroradiol. 2008 Aug. 29(7):1349-54. [Medline].

  26. Li-Shan L, Chang-Wei L, Heng G, Yue-Hong Z, Xing-Ming C, Yong-Jun L. Efficacy of surgical therapy for carotid body tumors. Chin Med Sci J. 2011 Dec. 26(4):241-5. [Medline].

  27. Makeieff M, Raingeard I, Alric P, Bonafe A, Guerrier B, Marty-Ane Ch. Surgical management of carotid body tumors. Ann Surg Oncol. 2008 Aug. 15(8):2180-6. [Medline].

  28. Sahin MA, Jahollari A, Guler A, Doganci S, Bingol H, Karaman B, et al. Results of combined preoperative direct percutaneous embolization and surgical excision in treatment of carotid body tumors. Vasa. 2011 Nov. 40(6):461-6. [Medline].

  29. Kim H, Cho YP, Moon KM, Kwon TW. Embolic stroke after carotid artery ligation during carotid body tumor resection. Vascular. 2011 Nov 18. [Medline].

  30. Zhang TH, Jiang WL, Li YL, et al. Perioperative approach in the surgical management of carotid body tumors. Ann Vasc Surg. 2012 Aug. 26(6):775-82. [Medline].

  31. Abu-Ghanem S, Yehuda M, Carmel NN, Abergel A, Fliss DM. Impact of preoperative embolization on the outcomes of carotid body tumor surgery: A meta-analysis and review of the literature. Head Neck. 2016 Apr. 38 Suppl 1:E2386-94. [Medline].

  32. Spinelli F, Massara M, La Spada M, et al. A simple technique to achieve bloodless excision of carotid body tumors. J Vasc Surg. 2014 May. 59(5):1462-4. [Medline].

  33. van der Mey AG, Jansen JC, van Baalen JM. Management of carotid body tumors. Otolaryngol Clin North Am. 2001 Oct. 34(5):907-24, vi. [Medline].

  34. Antonello M, Piazza M, Menegolo M, Opocher G, Deriu GP, Grego F. Role of the genetic study in the management of carotid body tumor in paraganglioma syndrome. Eur J Vasc Endovasc Surg. 2008 Nov. 36(5):517-9. [Medline].

  35. Drocos Kotelis, Timolaos Rizos, Philipp Geisbus, et al. Late Outcome after Surgical management of carotid body tumors from a 20-year single-center experience. Langenbecks Arch Surg. 2008.

  36. Gardner P, Dalsing M, Weisberger E, Sawchuk A, Miyamoto R. Carotid body tumors, inheritance, and a high incidence of associated cervical paragangliomas. Am J Surg. 1996 Aug. 172(2):196-9. [Medline].

  37. Hinerman RW, Amdur RJ, Morris CG, Kirwan J, Mendenhall WM. Definitive radiotherapy in the management of paragangliomas arising in the head and neck: a 35-year experience. Head Neck. 2008 Nov. 30(11):1431-8. [Medline].

  38. Janda PH, Veerappan V, McKenzie ME, Dhudshia NV. Carotid body tumor as a reversible cause of syncope. J Am Osteopath Assoc. 2011 Nov. 111(11):638-44. [Medline].

  39. Karatas E, Sirikci A, Baglam T, Mumbuc S, Durucu C, Tutar E. Synchronous bilateral carotid body tumor and vagal paraganglioma: a case report and review of literature. Auris Nasus Larynx. 2008 Mar. 35(1):171-5. [Medline].

  40. Pellitteri PK, Rinaldo A, Myssiorek D, Gary Jackson C, Bradley PJ, Devaney KO. Paragangliomas of the head and neck. Oral Oncol. 2004 Jul. 40(6):563-75. [Medline].

  41. Scanlon JM, Lustgarten JJ, Karr SB, Cahan JI. Successful devascularization of carotid body tumors by covered stent placement in the external carotid artery. J Vasc Surg. 2008 Nov. 48(5):1322-4. [Medline].

  42. van der Bogt KE, Vrancken Peeters MP, van Baalen JM, Hamming JF. Resection of carotid body tumors: results of an evolving surgical technique. Ann Surg. 2008 May. 247(5):877-84. [Medline].

 
Previous
Next
 
Proximal and distal control of the carotids with vessel loops.
Complete subadventitial removal of the tumor with intact carotids.
Carotid body tumor after total excision.
CT scan with IV contrast demonstrating a large left enhancing carotid body tumor extending into the parapharyngeal space to the oropharynx.
Preauricular excision extended from the neck in patients who have large tumors.
Proximal and distal control of the carotids is extremely important and can be difficult in larger tumors.
Postoperative picture after removal of the tumor.
Four-vessel angiography of a 57-year-old patient with bilateral carotid body tumors.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.