Abstract:Carotid body tumor (CBT) is a rare paraganglioma arising from the carotid bifurcation and is generally characterized by benign biological behavior; however, a subset of patients may develop local invasion, recurrence, or distant metastasis. In recent years, advances in molecular biology and imaging techniques have substantially improved our understanding of the pathogenesis, diagnosis, and management of CBT. Current evidence indicates that chronic hypoxia and mutations in succinate dehydrogenase (SDH)-related genes jointly contribute to tumorigenesis, with SDH mutation-induced pseudohypoxia signaling representing a major research focus. Color Doppler ultrasonography, computed tomography angiography (CTA), and magnetic resonance angiography (MRA) remain the principal imaging modalities for diagnosis and preoperative evaluation. Compared with the traditional Shamblin classification, the Peking Union Medical College Hospital classification incorporates longitudinal anatomical extension, providing more accurate surgical risk stratification for complex tumors. In addition, the Grading System for Adrenal Pheochromocytoma and Paraganglioma (GAPP) facilitates assessment of malignant potential. Surgical resection remains the treatment of choice, whereas perioperative carotid management, preoperative embolization, and endovascular interventions continue to be topics of ongoing debate. Precision radiotherapy serves as an effective alternative for patients who are inoperable or at high surgical risk. This review summarizes recent advances in the pathogenesis, clinical manifestations, classification, diagnosis, treatment, and follow-up of CBT, aiming to provide updated evidence for clinical practice and future research.