Carotid Body TumorsJASON LEPSE, MS4UNIVERSITY OF KANSAS SCHOOL OF MEDICINE

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

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

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

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

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

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

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)

7Shamblin Classification

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

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.