A rare case of paraganglioma arising in the vagus nerve and carotid body is reported. A tumor occupying the right parapharyngeal space was revealed incidentally by computed tomography in a 46 year-old woman. The tumor was located between the common carotid artery and the internal jugular vein and was removed transcervically with vagus nerve. After the operation, Horner's symptom and hoarseness occurred and thyroplasty was performed. Six months after the operation, a right neck mass occurred and angiography showed a high vascular tumor in the widened carotid bifurcation. The tumor was resected transcervically. The patient's elder brother had also exhibited a glomus-jugular tumor and had died of intracranial invasion. This case is the first familial case and the third multiple case of vagal paraganglioma reported in Japan.
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