Paragangliomas of the head and neck. A report of three cases and a review of the Japanese literature.:-A Report of Three Cases and a Review of the Japanese Literature-

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Three cases of paraganglioma of the head and neck are presented, two intercarotid paragangliomas and one intravagal paraganglioma. A review of Japanese literature of paragangliomas of the head and neck revealed 196 cases, including our three cases. The sites of the tumors were intercarotid, jugulotympanic, laryngeal, intravagal, nasal, orbital, base of the tongue, and neck (not otherwise specified) ; the number of cases according to site was 100, 77, 5, 5, 2, 2, 1 and 2, respectively. The remaining two cases were sympathetic paraganglioma or extraadrenal pheochromocytoma of the neck. The age at diagnosis ranged from 3 to 82 years (median 40 years and mode in the 30s). The female to male ratio was 1.7 : 1 and female predominance was greatest in cases of intravagal paraganglioma. On the contrary, 4 of 5 patients with laryngeal paraganglioma were male. Multiple primary paragangliomas were diagnosed in four cases. A familial incidence was noted in two families. Twenty four cases had developed metastases. Five functioning paragangliomas were reported.