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A case of malignant carotid body chemodectoma with neighbouring lymph nodes metastases is reported. The case is noteworthy as it was accompanied by another jugulotympanic chemodectoma. There was also hereditary component as four of the patients forebears had been operated for cervical chemodectomas. An ultrastructural study showed the characteristic secretory granules. 32 other cases of tumors of carotid body and jugular glomus with metastases are reviewed.
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