Asian Cardiovasc Thorac Ann 2001;9:208-211
© 2001 Asia Publishing EXchange Pte Ltd
Carotid Body Tumors (Paragangliomas)
Yahya Ünlü, MD,
Azman Ate
, MD,
Ahmet Özyazicio
lu, MD,
Necip Becit, MD,
Kemal Erol, MD1,
Münacettin Ceviz, MD,
brahim Yekeler, MD,
Ünsal Vural, MD,
Hikmet Koçak, MD
Department of Cardiovascular Surgery
1 Department of Cardiology Atatürk University Medical Faculty Erzurum, Turkey
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For reprint information contact: Yahya  nlü, MD Tel: 90 442 316 6333 Fax: 90 442 316 6340 email: yahyaunlu{at}hotmail.com Atatürk Mah. Universite Loj. 38, Blok No. 8, Erzurum 25170, Turkey.
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Abstract
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Carotid body tumors were diagnosed in 19 patients (13 females and 6 males) between 1977 and 2000. All but one were operated upon. The ages of the 18 surgically treated patients ranged from 17 to 65 years. Carotid body tumor was confirmed in 16 cases; the diagnosis was neurofibroma in 1 and tuberculosis lymphadenitis in 1. The carotid body tumors were resected without a shunt procedure. Eight patients underwent total resection, 6 had resection and saphenous vein interposition, 1 had partial resection, and 1 had carotid artery ligation with no resultant neurological deficit. One case of hypoglossal paralysis and one benign ipsilateral recurrence were detected. Contralateral recurrence was detected in 1 patient 4 years postoperatively. No mortality or malignant course was observed.
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Introduction
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Carotid body tumors are rare benign neoplasms accounting for less than 0.5% of all tumors, and they are the most common extra-adrenal paraganglioma.1 They develop from epithelioid cells derived from the neural crest. The carotid body is usually a minute oval structure of 0.1 to 0.5 cm in diameter located on the posterior aspect of the carotid bifurcation. The blood supply is mostly via a small vessel from the carotid bifurcation and branches of the external carotid artery. The carotid body is innervated by afferent branches of the glossopharyngeal and vagus nerves and the cervical sympathetic ganglia.2 There is specialized chemoreceptor tissue in the carotid body, which plays a complex homeostatic role in the control of arterial blood gases and pH.2 Chemoreceptors in carotid bodies are sensitive to hypoxia, hypercapnia, and acidosis, and the substantial blood flow allows these cells to participate in the control of respiration.1 Carotid body tumors result in symptoms related to their mass and compression on adjacent structures in the relatively confined space of the carotid sheath. The tumor is usually discovered on routine examination and noted to be pulsatile but not expandable. Biopsy is contraindicated because of the risks of bleeding and pseudoaneurysm.1–3 Approximately 10% of patients have bilateral lesions.
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Patients and Methods
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Between 1977 and 2000, 19 patients (13 females and 6 males) aged 17 to 65 years were diagnosed with carotid body tumors at this institute. A mass in the neck was the common symptom in all patients (Table 1
). The site and size of each tumor were established by imaging studies including color-flow duplex sonography in 7 (Figure 1), computed tomography in 17 (Figure 2), magnetic resonance imaging in 6 (Figure 3), selective angiography (before 1990) in 10 (Figure 4), and digital subtraction angiography (after 1990) in 9. No hormonally active tumor was detected on the basis of clinical and laboratory studies. Eighteen patients agreed to undergo surgical treatment.
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Figure 4. Selective angiography showing a carotid body tumor involving the carotid bifurcation. Note the splaying of the internal and external branches, and the dense vascularity of the tumor mass.
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Results
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In 16 of the 18 surgically treated cases, a carotid body tumor was confirmed by histopathology. One case was found to be a neurofibroma and another was diagnosed as tuberculosis lymphadenitis; these 2 patients were excluded from the study. There was no history of tuberculosis in the patient with tuberculosis lymphadenitis, and clinical signs in the lung or other organs could not be established.
According to the Shamblin classification, 3 patients had a type I carotid body tumor, 10 had type II, and 3 had type III (Table 1
). Total resection was performed in 8 patients, a further 6 had total resection and saphenous vein interposition, 1 had partial resection, and 1 underwent carotid artery ligation. No shunt procedures were employed. Regional lymph node was detected in 3 cases during the operation. The histopathologic diagnosis was nonchromaffin paraganglioma in all 16 patients. One case of hypoglossal paralysis and 2 benign recurrences were detected (Table 1). No patient died and no malignant course was observed.
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Discussion
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Although the term "tumor" has historically been assigned to these masses, they are not carcinomas, and the neoplastic process is actually hypertrophy of the carotid body tissue. Carotid body hypertrophy has been associated with chronic hypoxia and familial predisposition.4 Our patients all lived in a particular area at an altitude of 1,800 meters.
A carotid body tumor can be diagnosed by ultrasound, technetium isotope scintigraphy, computed tomography, or magnetic resonance imaging. Color-flow duplex sonography can easily delineate a hypervascular mass nestled in the carotid bifurcation, which differentiates it from a relatively nonvascular mass and is now the most widely utilized noninvasive method.5 In addition, this technique can provide information on tumor dimensions and demonstrate any coexistent carotid occlusive disease.2,5 Arteriography was once the diagnostic tool of choice for demonstrating a hypervascular mass arising in the crotch of the carotid bifurcation. It is still essential for identifying large tumors that may be easily embolized as a preparatory step for surgical excision.2,6,7 A carotid body tumor must be differentiated from bronchial cyst, parotid gland tumor, carotid artery aneurysm, lateral aberrant thyroid gland, malignant lymphoma, neuro-fibroma, tuberculosis lymphadenitis, and metastatic carcinoma.
Shamblin and colleagues8 classified carotid body tumors according to their gross relationship with the carotid vessels. Type I tumors account for 26% of cases and are localized masses that are easily removed. Type II tumors are adherent and partially surround the carotid vessels; they make up 46% of such tumors, and are removed with moderate difficulty. Type III tumors (27%) are adherent and completely surround the carotid vessels; they are removed with extreme difficulty and generally require resection of the internal carotid artery with vein graft interposition.9 Type III tumors are associated with a high complication rate (perioperative stroke and cranial nerve dysfunction).
Several methods of treating carotid body tumors have been proposed. The role of preoperative embolization of highly vascular carotid body tumors is controversial. Preoperative percutaneous catheter embolization of the lesion might be effective in reducing vascularity and improving the safety of surgical excision, with lower operative blood loss and decreased technical difficulties. However, percutaneous embolization may produce throm-bosis in the internal carotid artery or cerebral embolization. Therefore, this method should be used only as an adjunct to operative resection.2,10,11 Schick and colleagues12 performed arterial embolization and showed by arterio-gram that the size of the tumor decreased by 30% to 90%.
Once a paraganglioma is discovered, surgical excision is the treatment of choice unless the patient has a specific contraindication such as terminal illness, advanced age, or high anesthetic or operative risks.13 This tumor is radioresistant, thus adjuvant radiotherapy after partial resection is of questionable benefit and most tumors subsequently demonstrate progressive enlargement.14,15 The lesion is considered to be malignant if there is infiltration of the paraganglionic tissue into adjacent lymph nodes or distant metastasis, and approximately 10% of reported cases meet this definition of malignancy. In follow-up studies at the Memorial Cancer Center, malignancy was detected in 9% of 43 patients over a 20-year period.10–12 No malignant course was observed in our study.
The external carotid artery should be ligated only if it is invaded.2,11 As in our single case, Padberg and colleagues16 ligated the carotid arteries in 3 patients without causing any neurological deficit, although abrupt obstruction of the carotid arteries caused paralysis in 30%. Chronic narrowing of the internal carotid artery may not produce paralysis due to collateral circulation.17 However, Chamorro Sanchez and colleagues18 reported transient ischemic attacks in a patient with bilateral carotid body tumors. Cowley19 reported 12% mortality and a 30% incidence of paralysis after carotid ligation. A donor saphenous vein site should be prepared as complicated arterial reconstruction is required in approximately 25% of cases.11,19 Carotid shunts should also be available because clamping of the internal carotid artery may be necessary.
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References
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Abstract
Introduction
