Carotid body tumor is an uncommon tumor of the head and neck. The coexistence of this entity with primary hyperparathyroidism is even more unusual, with only four cases having been previously recorded. This report describes a patient with a left inferior parathyroid adenoma and a right carotid body tumor. A common neuroectodermal origin is proposed as an explanation for the simultaneous occurrence of these tumors. The fact that this may represent yet another variable expression of the multiple endocrine neoplasia syndromes emphasizes the importance of careful endocrine screening in patients with carotid body tumors.
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