Case presentation identifying key diagnostic and treatment considerations for carotid body tumors

1. Ms. L
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. Ms. L’s Course
 Ms. L saw Dr. Kakarala, Dr. Vamanan and Dr. Camarata 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
 1: Johnson, Jonas T., Clark A. Rosen, and Byron J. Bailey. Bailey's Head and Neck Surgery--otolaryngology.
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.