Nonpulsatile carotid body tumor in a teenager.

A 16-year-old girl presented with a painful nonpulsatile neck mass. Obtaining a fine-needle aspiration biopsy specimen elicited shooting head pain; the results were nondiagnostic. Suspicion was high for a neurogenic tumor. Imaging studies, including contrast-enhanced computed tomography (CT) of the neck, were followed by cervical angiography (figure 1). These images demonstrated the classic findings of a carotid body tumor. The patient underwent preoperative embolization and an uneventful subadventitial dissection of a 3-cm carotid body tumor (figure 2).

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The carotid body is located at the carotid bifurcation. As a chemoreceptor, it is distinct from the carotid sinus, which is a baroreceptor. Carotid body tumors are benign masses that arise from the neural crest-derived paraganglia of the autonomic nervous system. They account for 60 to 70% of all paragangliomas of the head and neck.

Patients typically present during the fourth or fifth decade of life with a slowly growing, painless neck mass. Progressive enlargement of the tumor may lead to hoarseness, cranial nerve deficits, pain, vocal fold paralysis, and/or Horner's syndrome. Examination reveals a pulsatile, poorly defined anterior neck mass near the level of the hyoid; the tumor is not mobile in the vertical axis. Fine-needle aspiration and open biopsies should be avoided. The diagnosis is typically made via carotid angiography or magnetic resonance angiography, which shows splaying of the internal and external carotid arteries (lyre sign).

The treatment of choice is surgical excision. Preoperative embolization can significantly reduce bleeding and enhance visualization of tissue planes. After gaining both proximal and distal control of the carotid system, a subadventitial dissection should be performed to minimize significant blood loss. Surgical planning should account for the possible need for vascular repair and shunting. Radiotherapy is reserved for adjuvant and palliative therapies in cases in which the therapeutic goal is merely to arrest tumor growth.

Suggested reading

Mendenhall WM, Hinerman RW, Amdur RJ, et al. Treatment of paragangliomas with radiation therapy. Otolaryngol Clin North Am 2001 ;34:1007-20, vii-viii.

van der Mey AG, Jansen JC, van Baalen JM. Management of carotid body minors. Otolaryngol Clin North Am 2001;34:907-24, vi.

Ward PH, Liu C, Vinucla F, Bentson JR. Embolization: An adjunctive measure for removal of carotid body tumors. Laryngoscope 1988;98:1287-91.

From the Department of Otolaryngology, Charles R. Drew University of Medicine and Science, and the Head and Neck Cancer Center, Cedars-Sinai Medical Center, Los Angeles.