Malignant carotid body tumor with systemic metastases.

Carotid body tumors (CBTs) are rare neoplasms of the carotid body. Most CBTs are benign; however, some can show malignant behavior. Malignant CBTs have an unpredictable history; often, there is no correlation between the histologic findings and the clinical behavior. They are usually diagnosed by the development of local recurrence or lymph node metastasis following total resection of the primary mass, or by the detection of distant metastasis. There are few reports of histopathologic confirmation of malignant CBTs. We report a rare case of malignant CBT with distant metastases, in which the diagnosis was confirmed by histopathology, and present a review of the literature.