CASE REPORT Carotid Body Tumor As a Reversible Cause of Syncope
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CASE REPORT Carotid Body Tumor as a Reversible Cause of Syncope Paul Harlan Singh Janda, DO; Venkatachalam Veerappan, MD; Mark E. McKenzie, MD; and Neel V. Dhudshia, MD The causes of syncope are diverse and extensive; carotid In the current report, we present the case of a patient body tumors are an extremely rare cause of syncope. These with syncope who, after other causes had been ruled out, was rare neoplasms represent less than 0.5% of all head and neck found to have a right-sided carotid body tumor as the cause tumors. The authors present a case of a woman with syncope of her condition. We also review the history, presentation, who was found to have a right-sided carotid body tumor. epidemiology, etiology, diagnosis, management, and prog - After surgical resection was performed, she did not have nosis of this condition. any additional syncopal or near-syncopal events. The authors provide a review of the literature on the natural history, pre - Report of Case sentation, and preferred management of carotid body tumors. Our patient was a 75-year-old white woman with a medical his - With modern diagnostic tools and treatment options, most tory that included hypertension, type 2 diabetes mellitus, and patients with this diagnosis can expect to recover fully. a prolapsed bladder. She was admitted to the hospital with a J Am Osteopath Assoc . 2011;111(11):638-644 chief complaint of syncope and left hip pain secondary to her fall. She reported having approximately 4 or 5 near-syncopal episodes before this admission. These earlier events consisted yncope is defined as the abrupt loss of consciousness of a sensation of dizziness but did not result in loss of con - Swith loss of postural tone. Approximately one-third of sciousness or postural tone. However, they were increasing in 1 individuals will have a syncopal episode during their lifetime. frequency. The patient reported that the events were occa - 2 According to Manolis et al, syncope is the reason for up to sionally linked to her position (ie, standing) and occurred at dif - 5% of all emergency department admissions. However, the ferent times throughout the day. She had no history of seizures causes of syncope are diverse and extensive; syncopes that or stroke. originate from vasodepressor, cardiogenic, and orthostatic Her syncopal episode occurred while she was standing at 3 causes are among the most common. the sink in her bathroom. She reported experiencing a pro - Carotid body tumors are rare neoplasms that are also drome of dizziness before falling to the floor. She stated that referred to as carotid body paragangliomas because they arise she had a rapid recovery, crawled to the toilet for positional 4 from paraganglionic cells. Carotid body tumors are an support, and then called out to her family members, asking 4-6 extremely rare cause of syncope, representing less than 0.5% them to phone for emergency medical services. The patient was 7 of all head and neck tumors. We reviewed the literature and neither disoriented nor weak after the episode and had no 8,9 found few reports of patients with these tumors. associated bowel or bladder incontinence or any oropharyn - geal trauma. Although she had type 2 diabetes mellitus, she was normoglycemic at her home when she was tended to by emergency medical services. During physical examination at admission, the patient appeared to be her stated age and was in no acute distress; she was awake, alert, and oriented. Her vital signs were stable. Her neck was not obese, enabling the physician (P.H.S.J.) to palpate a small, pulsatile right-sided mass with some mild tender - From the departments of neurology (Drs Janda and Veerappan), internal ness to light pressure. Except for this finding, the physical medicine (Dr McKenzie), and cardiothoracic surgery (Dr Dhudshia) at Valley examination findings were within normal limits for the patient’s Hospital Medical Center in Las Vegas, Nevada. Financial Disclosures: None reported. age. Address correspondence to Paul Harlan Singh Janda, DO, Valley Hospital On day 1, several diagnostic studies were performed. An Medical Center, 620 Shadow Ln, Las Vegas, NV 89106-4119. electrocardiogram exhibited normal sinus rhythm; an echocar - E-mail: [email protected] diogram showed an ejection fraction of 60% with mild, con - Submitted March 1, 2011; final revision received August 10, 2011; accepted centric left ventricular hypertrophy. Her orthostatic vital signs August 11, 2011. were unremarkable. Plasma metanephrine levels were mea - 638 • JAOA • Vol 111 • No 11 • November 2011 Janda et al • Case Report CASE REPORT sured and were within normal limits. A computed tomo - patient was informed of the risks and benefits of the procedure graphic (CT) scan of the brain without contrast material and agreed to proceed. The operation was uncomplicated. enhancement was unremarkable. The patient’s heart rate was At resection, the tumor appeared to be a highly vascu - monitored telemetrically and exhibited a sinus cardiac rhythm larized, solid mass resembling a tuft of capillaries. It was sent throughout her hospital stay. to the pathologist for histologic analysis. Histologic findings On day 2, results from magnetic resonance (MR) imaging were typical of a carotid body tumor and indicated no signs of of the brain, performed with and without gadolinium con - malignancy. Sections of the tumor appeared highly vascular, trast enhancement, demonstrated no acute changes or with cells palisaded by surrounding blood vessels. There were abnormal enhancement; only scant chronic white matter numerous cell clusters in zellballen formation, separated by a changes were noted. An MR angiogram of the brain was unre - prominent vascular stroma that is pathognomonic for carotid markable. An electroencephalogram was within normal limits body tumors. Hematoxylin-eosin staining revealed chief cells— and showed no areas of focal slowing and no spikes or sharp type I cells that are APUD (amine precursor uptake and decar - wave activities. Results from duplex Doppler carotid ultra - boxylation) cells with copious cytoplasm and large round sonography revealed normal carotid and vertebral arteries; it nuclei ( Figure 2 ), and S100 staining revealed the elongated also demonstrated a 1.5-cm nodule that was near the thyroid type II supporting cells ( Figure 3 ) that are also found in carotid gland but situated between the bifurcation of the right common body tumors. carotid artery into the right internal and external carotid The patient had no postoperative complications and exhib - arteries. ited no nerve damage or deficits. She was closely followed On the third day, 3-dimensional CT angiography of the up for 15 months after hospital discharge and had no additional neck and CT of the neck with iopamidol contrast enhance - syncopal or near-syncopal episodes. ment (Isovue-370, 100 mL) was performed, and results from these tests demonstrated a 1.7-cm mass with an intense vascular Comment and Review of Literature blush at the bifurcation of the right common carotid artery Historical Background (Figure 1 ). The findings were deemed to be consistent with a A carotid body tumor is a rare neoplasm of the carotid body carotid body tumor. that was first described by Lushka in 1862. 10 In 1880, Riegner The vascular surgery department was consulted on day attempted the first surgical resection, which resulted in the 3 and recommended surgical resection of the tumor. The death of the patient. 11,12 Maydl’s resection attempt in 1886 A B Figure 1. Three-dimensional computed tomographic (CT) angiogram (A) and transverse CT scan (B) of the neck of a 75-year-old woman presenting with complaint of sycope. The images revealed a 1.7-cm carotid body tumor (arrows) at the bifurcation of the right common carotid artery. Janda et al • Case Report JAOA • Vol 111 • No 11 • November 2011 • 639 CASE REPORT Figure 2. Histologic analysis of a tumor in a 75-year-old woman revealed Figure 3. Histologic analysis of a tumor in a 75-year-old woman revealed numerous chief cells in the pathognomonic zellballen cluster formation elongated sustentacular cells that are pathognomonic for carotid body (arrow), separated by a vascular stroma (hematoxylin-eosin stain; orig - tumors (S100 stain; original magnification, ×40). inal magnification, ×40). was reportedly successful, but the procedure caused sub - the lesion and is caused by the external carotid artery. 11 Rarely, stantial, irreversible residual complications of aphasia and carotid body tumors secrete catecholamines, which can result hemiplegia in his patient. The first published report of resec - in hypertension. 11,19 Our patient’s metanephrine levels were tion of a carotid body tumor with preservation of the carotid within normal limits, and she did not exhibit any hemody - arteries was by Scuddder in 1903. 11,12 In 1940, Gordon-Taylor namic instability. The majority of patients with carotid body illustrated an appropriate plane of dissection for the proce - tumors are asymptomatic until the tumor enlarges; carotid dure. 13 In 1953, Morfit et al 14 described arterial reconstruction body tumors expand slowly, with a growth rate of 0.5 cm per techniques, offered operative recommendations, and provided year. 4,7,9 Syncope, dizziness, and transient ischemic attacks the first documented report of a successful carotid body tumor are very rare presentations. 20 More frequent presentations are resection. A major publication by Shamblin et al 15 provided a neck discomfort, dysphagia, or dyspnea due to the size of the reliable template for categorizing carotid body tumors based mass, or hoarseness, dysphagia, or tongue weakness due to cra - on the degree of encirclement of the carotid arteries, a system nial nerve involvement. 11 Specifically, the 8th through 12th that is still used today. cranial nerves may be involved. Involvement of the mandibular Although carotid body tumors have been reported in the branch of the seventh cranial nerve has been reported but is literature for many years, syncope has rarely been mentioned rare.