Asian Cardiovasc Thorac Ann 2002;10:173-175
© 2002 Asia Publishing EXchange Pte Ltd
Carotid Body Tumors
Hakki Aydo
an, MD,
Gökçen Orhan, MD,
Serap Aykut-Aka, MD,
ebnem Albeyolu, MD,
Okan Yücel, MD,
Murat Sargin, MD,
Onur Göksel, MD,
Ugür Filizcan, MD,
E Ergin Eren, MD
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Department of Cardiovascular Surgery Siyami Ersek Thoracic and Cardiovascular Surgery Center Istanbul, Turkey
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Gökçen Orhan, MD Tel: 90 216 349 9120 Fax: 90 216 348 9323 email: gokcenorhan{at}hotmail.com Dr. Erkin Cad. Zaman Sok. Güngör, Apt. No. 4/9 Dumlupinar Mah., Göztepe, Istanbul 81050, Turkey.
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ABSTRACT
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Carotid body paragangliomas were diagnosed by Doppler ultrasound, carotid artery angiography, and cranial computed tomography in a 35-year-old man with a mass in the neck and hearing loss, and in a 42-year-old man with headache, syncope, and a mass in the neck. They underwent successful surgical excision.
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INTRODUCTION
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Paragangliomas are painless tumors that grow very slowly. They originate from neural crest cells that have migrated with ganglionic cells from the autonomic nervous system.
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CASE REPORTS
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CASE 1
A 35-year-old man was admitted to a health center with a mass in the neck and hearing problems. The mass had appeared 6 years previously and grew progressively. On physical examination, a mobile painless 3 x 3-cm mass was found in the left jugular-carotid region. Routine laboratory investigations were within normal limits. A bilateral ultrasound study of the neck showed a solid mass of approximately 2.5 x 3.8 x 2.4 cm in size in the left submandibular region between the internal and external carotid arteries, with no enlarged local lymph nodes. Contrast-enhanced computed tomography demonstrated a homogeneous 4 x 4 x 5-cm mass located in the region extending from the posteroinferior part of the left parapharyngeal space to the area between the internal and external carotid arteries near the bifurcation of the internal carotid artery and the submandibular gland inferiorly (Figure 1
). Angiography of the carotid arteries revealed a hypervascular mass perfused by the facial branch of the external carotid artery. This finding was considered to be consistent with a diagnosis of glomus caroticum (Figure 2). Audiometric investigations showed a bilateral mild hearing defect. He was then referred to our center for operation.
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Figure 1. Computed tomography showing a homogeneous mass of 4 x 4 x 5 cm in size, extending from the posteroinferior part of the left parapharyngeal space to the carotid arteries and the submandibular gland.
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Figure 2. The angiographic image of the hypervascular mass was consistent with a diagnosis of glomus caroticum.
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Under general anesthesia, the carotid region was reached with a transcervical incision. During surgical exploration, a smooth homogeneous 3 x 4-cm mass was observed; it was classified as a grade-2 tumor according to the Shamblin classification system. The common, external, and internal carotid arteries were isolated. The hypoglossal nerve was located. The tumor was isolated with subadventitial dissections, and resected with distal and proximal clamps on the external carotid artery, due to attachment of the tumor to this artery. After total extirpation of the tumor, the external carotid artery was anastomosed in an end-to-end fashion. The patient was discharged from the intensive care unit on postoperative day 1, and discharged from the hospital on day 6. Six months later, magnetic resonance imaging showed no mass or vascular pathology.
CASE 2
A 42-year-old man with no family history of carotid body tumor, was admitted to a private health center with headache, syncope, neck pain, and a mass in the neck. The mass was palpated in the middle of the left cervical region, along with multiple enlarged lymph nodes. Routine blood tests were within normal limits. Cranial computed tomography was normal. Ultrasound examination of the neck detected a homogeneous hypoechoic solid tissue mass with a smooth contour and a thickness of 6 mm, around the left internal and external carotid arteries, along a 3-cm segment extending from the left carotid bifurcation. These findings were consistent with thrombus formation or a carotid body tumor. Magnetic resonance imaging revealed a homogeneous mass lesion around the left internal and external carotid arteries, in the region of the carotid bifurcation, consistent with a carotid body tumor. Cerebral angiography demonstrated an intraluminal stenosing lesion localized to the arterial wall in the left common carotid artery (Figure 3).
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Figure 3. Cerebral angiogram showing a stenosing lesion localized to the arterial wall in the left common carotid artery.
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Under general anesthesia, the carotid region was reached by a transcervical incision. A homogeneous tumor with a smooth contour and approximately 3 cm long was observed at the carotid bifurcation. The common, external, and internal carotid arteries were dissected and isolated, the hypoglossal nerve was located, and the tumor was isolated by subadventitial dissection. It was considered to be Shamblin grade 2. No additional vascular procedure was necessary. The patient was moved from the intensive care unit after 1 day, and discharged from the hospital on the 6th postoperative day with a total recovery.
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DISCUSSION
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Carotid body paragangliomas are slow growing (5 mm annually), painless masses localized in the neck, anterior to the sternocleidomastoid muscle at the level of the hyoid bone. As the tumor grows, dysphagia, odynophagia, dysphonia, and symptoms due to compression of cranial nerves 9 to 12 may be seen.1–3 Carotid arterial angiography is the most valuable diagnostic technique.2,4 It can detect multiple lesions, tumor size and vascularity, and the major vascular tributaries perfusing the tumor. Pathonomically, a specific perfusion increase (Lir sign) at the carotid bifurcation can be seen due to the tumor.2 Arteriographic imaging should be carried out bilaterally because of the possibility of bilateral tumors.1,2,5 In cases of bilateral tumors, serum catecholamine levels as well as urinary vanilmandelic acid and metanephrine levels should be assessed.4,6 Magnetic resonance imaging and contrast-enhanced computed tomography scans are additional noninvasive diagnostic tools. With magnetic resonance imaging, paragangliomas smaller than 0.8 cm in diameter can be detected.1,4 Biopsy is contraindicated in cases of paraganglioma.3,4
Paragangliomas are generally sporadic in character; they are hereditary with an autosomal dominant pattern in 7% to 9% of cases. Patients with a family history and hyper-tensive palpitation attacks with flushing should be assessed.4,6 Surgery is the first choice of treatment. Radio-therapy has been used as the sole treatment in a few cases, and the short-term results are disputable.7 Radiotherapy may be considered as an alternative modality for an elderly patient in poor general status, whereas surgical treatment is preferred in young patients.2–4
Tumor classification (Shamblin) is based on the size and the difficulty of surgical resection. A grade-1 tumor is small and easily resected from vascular elements. Grade 2 is a medium-sized tumor closely associated with vascular structures, which can be resected by careful subadventitial dissection. A grade-3 tumor is large and enfolded by the carotid arteries; it can be resected only by partial or total vascular resection requiring vascular replacement. A transcervical approach is usually preferred, but in superiorly localized carotid paragangliomas, a cranial basilar approach can be used.7 The most important principle is to preserve the integrity of the internal carotid artery. If necessary, the external carotid artery can be sacrificed.2–4 Defects in the common or internal carotid arteries must be repaired immediately and graft replace-ment may be necessary.1,8
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REFERENCES
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ABSTRACT
INTRODUCTION
