Carotid body tumor: atypical angiogram of a functional tumor

Carotid body tumors (CBTs) are rare, usually benign, neoplasms of the extra-adrenal paraganglion system. They are almost always nonfunctional. The diagnosis is generally confirmed by an angiogram that shows a vascular tumor enlarging the space between the internal and external carotid arteries. A 55-year-old man with hypertension and episodes of flushing, palpitations, and dizziness was treated for a firm, nonmobile mass measuring 3 x 2 cm at the left carotid bifurcation. Plasma and urine catecholamines, and the vanilylmandelic acid/creatinine ratios were elevated. Carotid arteriograms showed a vascular mass displacing the vessels, but the space between the arteries was narrowed rather than enlarged, and an atherosclerotic plaque was present. At operation the CBT was removed by resection of the bifurcation and with a temporary shunt a saphenous vein graft was inserted between the common and internal carotid arteries. Pathologic examination revealed a typical paraganglionoma. Although most CBTs produce catecholamines, only 11 patients have been reported to have elevated plasma and urine levels, and most were symptomatic. Since these tumors slowly increase in size, early surgical removal is recommended, even in asymptomatic patients.