Carotid body paraganglioma (CBP) is a rare neuroendocrine tumor that typically develop at the carotid bifurcation. Although most CBPs are non-functional and slow growing, some pose a risk of local invasion and distant metastasis. Advances in genetic testing have revealed that up to 50% of paragangliomas (PGLs) are linked to germline mutations, most commonly in the succinate dehydrogenase (SDHx) genes. The clinical presentation of CBPs is often subtle, with patients noticing a painless, slowly enlarging mass in the neck. CBP diagnosis often relies on imaging (both anatomical and functional) and biochemical testing. In managing CBPs, surgical resection remains the standard treatment for localized tumor, but the procedure can be technically demanding due to high vascularity and close proximity of the tumor to cervical neurovascular structures. Other treatment options, especially for patients with unresectable or metastatic CBPs, include radiotherapy, radionuclide therapy, and chemotherapy. This review discussed current CBP management, from diagnosis to treatment and surveillance, highlighting the importance of individualized care based on tumor behavior, genetic background, and surgical risk.
Keywords: Carotid body; Genetic predisposition to disease; Head and neck neoplasms; Paraganglioma; Radionuclide imaging.