This document summarizes the case of a 47-year-old female patient presenting with a right neck mass. Imaging revealed bilateral carotid body tumors. Carotid body tumors are rare neuroendocrine tumors that typically arise from paraganglia near the carotid bifurcation. The patient's tumors were large and bilateral, presenting a surgical challenge. She underwent preoperative embolization followed by tumor resection via a transparotid approach, which resulted in temporary vocal cord paralysis. Her post-operative recovery required a PEG tube and vocal cord injections. Carotid body tumors are most often benign but can cause cranial nerve deficits if untreated or if the tumor is large.

1. Carotid Body Tumors
JASON LEPSE, MS4
UNIVERSITY OF KANSAS SCHOOL OF MEDICINE

2. H&P
 Background: 47 yo female who was referred to an outside ENT in August,
2014
 CC: Right neck mass
 HPI: Patient reported presence of neck mass for 9 years
 Initially fluctuated in size but recently began growing steadily
 Intermittent dull pain over right lateral neck sometimes extending to right year
 No fluctuation of mass with eating; no facial weakness, numbness, dysphagia,
odynophagia or voice change
 No tachycardia, diaphoresis, flushing or tachypnea
*Modified from documentation by Drs. Michael Baker, Kiran Kakarala and Paul Camarata

3. H&P
 PMH: Anemia, headaches and seasonal allergies
 PSH: Tonsillectomy and adenoidectomy
 Meds/Allergies: Aspirin and Tylenol; allergic to sulfa
 FH: Breast and uterine cancer in mother and sister
 SH: ½ ppd smoker for 35 years; occasional alcohol use
 ROS: + fatigue, hearing loss, tinnitus, palpitations, confusion, light-
headedness, headaches
*Modified from documentation by Drs. Michael Baker, Kiran Kakarala and Paul Camarata

4. H&P
 Exam
 Vitals: WNL
 General: Well-developed, well-nourished
 Ears: canals normal, TM intact and mobile; no evidence of fluid or infection
 Nose: Normal nasal mucosa, no masses/lesions
 Nasopharynx: No mass, lesion or drainage
 Oral Cavity: Clear and without signs of infection
 Pharynx: No mass or ulceration
 Larynx: Vocal cords mobile, no visible lesions
 Neck: Midline trachea without mass or lesion, large right side mass mobile (more horizontal than vertical) ,
firm, 4x5cm extending to the angle of mandible superiorly inferiorly to the level of cricoid, no TTP, non-
pulsatile
DDX: lymphadenopathy, branchial cleft cysts, salivary gland tumors, neurogenic
tumors, and aneurysms of the carotid artery.
*Modified from documentation by Drs. Michael Baker, Kiran Kakarala and Paul Camarata

5. Workup
 CT Neck with Contrast ordered by outside
ENT before referral to KU
 In the area of the carotid bifurcations there
are large enhancing masses bilaterally
 Right: 8x4x3 cm
 Left: 6x4 cm
 Masses compress but do not invade IJ
 Unremarkable parotid and submandibular
glands
 Diagnosis: Bilateral Carotid Body Tumors
 Definitive diagnosis would require histologic
verification
 Labs: Plasma-free metanephrines and 24-
hour urine metanephrines WNL

6. Carotid Body Tumors
 Type of paraganglioma, rare neuroendocrine tumors arising from extra-adrenal autonomic
paraganglia
 Parasympathetic derived
 Head and neck paragangliomas are less likely to be secretory (5%)1
 Most are sporadic but hereditary paragangliomas have been linked to mutations in the
succinate dehydrogenase (SDH) enzyme complex 2
 Sporadic tumors more common in women; hereditary tumors are equally distributed 3
 Mean age at diagnosis was 47 in a study of 236 patients 4
 Vast majority are benign; malignancy is only confirmed by presence of paraganglioma in
lymph nodes 1

7. Carotid Body Tumors
 Bilateral in approximately 10% of cases 5
 Fontaine’s sign: Exam finding in which mass is more
mobile in horizontal plane than vertical plane 6
 Lyre sign on angiography: splaying of the internal and
external carotid arteries by the mass 8
 Consider vagal paraganglioma if carotids are displaced
anteriomedially without splaying
 Salt and pepper appearance on MRI 8

8. Treatment
 Surgery remains the only definitive treatment with low
vascular morbidity 7
 If untreated, complications include dysphagia, deficits of
cranial nerves VII, IX, X, XI and XII, and hoarseness or a
Horner's syndrome may result from pressure on the vagus
or sympathetic nerves
 Shamblin III tumor and tumor size >3.2cm emerged as
predictive factors for early postoperative peripheral
neurological complications 7
 Post-operative nerve deficit is common (56%) but often
temporary (17% at 18 months)
7
Shamblin Classification

9. Course
 Patient saw surgeons prior to operation
 Underwent preoperative right carotid body tumor embolization
 Controversial with some studies reporting no benefit and others reporting
reduction in intraoperative blood loss and associated morbidity
 Right pre-auricular transparotid and transcervical approach to
parapharyngeal space tumor
 Failed swallow with evidence of right vocal cord paralysis
 PEG tube placed
 Underwent Vocal Cord Injection three days after resection
 Discharged with nectar-thick liquids and post-op swallow appointment

10. References
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Philadelphia: Wolters Kluwer Health /Lippincott Williams & Wilkins, 2014
 2: Dannenberg H, Dinjens WN, Abbou M, et al. Frequent germ-line succinate dehydrogenase subunit D gene
mutations in patients with apparently sporadic parasympathetic paraganglioma. Clin Cancer Res 2002; 8:2061.
 3: Lin D, Carty S, Young WF Jr. Paragangliomas: Epidemiology, clinical presentation, diagnosis, and histology.
UpToDate. 2015.
 4: Erickson D, Kudva YC, Ebersold MJ, Thompson GB, Grant CS, van Heerden JA, Young WF Jr. Benign
paragangliomas: clinical presentation and treatment outcomes in 236 patients. J Clin Endocrinol Metab.
2001;86(11):5210.
 5: Davidovic LB, Djukic VB, Vasic DM et-al. Diagnosis and treatment of carotid body paraganglioma: 21 years of
experience at a clinical center of Serbia. World J Surg Oncol. 2005;3 (1): 10
 6 :Boedeker CC, Ridder GJ, Schipper J. Paragangliomas of the head and neck: diagnosis and treatment. Fam Cancer.
2005;4(1):55.
 7: Lamblin, E., I. Atallah, E. Reyt, S. Schmerber, J.-L. Magne, and C.a. Righini. "Neurovascular Complications following
Carotid Body Paraganglioma Resection." European Annals of Otorhinolaryngology, Head and Neck Diseases (2016)
 8: Lee, K. J. Essential Otolaryngology: Head and Neck Surgery. Norwalk, CT: Appleton & Lange, 2012.