Bilateral carotid body tumor management: tips, tricks, strategies, and problems

Carotid body tumors (CBTs) are rare neuroendocrine tumors with an annual incidence of 1 : 30 000. Bilateral carotid body tumors (BCBTs) account for 3-5% of all CBTs and are more frequently linked to familial syndromes and potential malignancy. BCBT management is still not universally standardized and depends on multiple factors, including patient's age, risk of malignancy, location, growth rate, size, and related Shamblin and Mehanna classifications.

Options of treatment include active surveillance, external beam radiation, and surgery. Surgery is the first-choice treatment, but it may not always be performed especially in elderly patients. Simultaneous BCBT resection is not suggested due to high intra-operative and postoperative risks. The decision to operate on the larger or smaller tumor first is still debated. Whenever feasible, treatment of the larger tumor first to reduce the tumor burden should be preferred but with higher risks of neurovascular injury. Conversely, starting with the smaller tumor first offers a lower risk at initial surgery but may complicate the management of the larger tumor later.

Surgery for BCBTs, whenever feasible, remains the most indicated treatment but poses a significant risk of neurovascular complications. Resection of the larger, often more symptomatic, and potentially problematic or malignant tumor, reduces the overall disease burden and mitigates risks of rapid progression but involves a higher immediate complication hazard. Multidisciplinary evaluation is essential for balancing surgical risks and long-term outcomes, prioritizing neurovascular preservation and reducing morbidity.