Abstract

Carotid body paragangliomas (CBP) are uncommon tumours that very rarely cause death. The genetic basis of tumours of hereditary origin (more than a third of the total) has been determined in the last few years, which has helped to identify the population at risk and to implement screening methods for an early diagnosis of tumours. The most common treatment of CBP has been surgery, which frequently causes significant morbidity. A significant number of paragangliomas do not experience any growth, which justifies a wait-and-see approach using annual image studies. Those tumours with significant growth must be treated. Radiotherapy has similar outcomes to surgery and has no neurological or vascular sequelae. However, surgery is indicated in Shamblin I/II carotid body tumours in young patients, in the rare malignant or hyper-functioning tumours, and in the failure of radiotherapy. Multiple tumours of familial origin should be treated conservatively.

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