Paraganglioma represent vascular neoplastic tumors that arise from chemoreceptors located in the wall of blood vessels or are associated with specific nerves. The site of origin defines the name given to these tumors, and they most commonly occur at the carotid bifurcation where they are known as carotid body tumors (CBT). A malignant form of CBT, malignant CBT is uncommon. We report herein on a case of malignant CBT in a 70-year-old woman. The patient complained of a lateral cervical mass on the right side four years previously. The tumor had become gradually larger and tender. Enhanced CT scan and carotid angiography revealed a vascular-rich tumor of the right carotid bifurcation. Fine-needle aspiration biopsy of the mass showed variation in nuclear size. A resection was performed after vascular embolization. Histopathologically, the tumor cells were positive for chromogranin A, synaptophysin and CD56. Scattered sustentacular cells were positive for S-100 protein. Moreover the specimens showed vessel invasion and extracapsular tumor invasion. This case was diagnosed as malignant CBT taking both the clinical findings and the histopathological sides into consideration. The patient had no problem after the operation. Observation of the patient’s progress is due to be performed carefully in the future.
