What is the management approach for a carotid body tumor (carotid body tumour) with suspected endocrine activity?

From the Guidelines

Management of a carotid body tumor with suspected endocrine activity requires a multidisciplinary approach focused on preoperative stabilization and careful surgical planning, with the primary goal of minimizing morbidity, mortality, and improving quality of life. The first step is biochemical confirmation of catecholamine excess through 24-hour urinary metanephrines and plasma free metanephrines, as recommended by the clinical consensus guideline on the management of phaeochromocytoma and paraganglioma in patients harbouring germline sdhd pathogenic variants 1. Once confirmed, preoperative alpha-adrenergic blockade is essential, typically starting with phenoxybenzamine 10mg twice daily, gradually increasing to 20-40mg daily until adequate blood pressure control is achieved (systolic <140 mmHg, diastolic <90 mmHg), as supported by the study published in The Lancet Diabetes and Endocrinology 1.

• Key considerations in the management of carotid body tumors with suspected endocrine activity include:
  • Preoperative alpha-adrenergic blockade to prevent hypertensive crisis
  • Volume expansion with increased salt and fluid intake during alpha-blockade
  • Preoperative imaging with CT, MRI, or MRA to determine tumor extent and vascular involvement
  • Surgical resection as the definitive treatment, with careful intraoperative monitoring of hemodynamics
  • Postoperative monitoring for hypotension as catecholamine levels normalize

The approach to carotid body tumors with suspected endocrine activity should prioritize minimizing the risk of perioperative complications, such as hypertensive crisis and hemodynamic instability, as emphasized by the clinical consensus guideline 1. Surgical resection remains the definitive treatment, but should be approached with caution, considering the potential risks and benefits, and the patient's overall health status and quality of life. The management of these tumors should be individualized, taking into account the patient's specific needs and circumstances, as recommended by the clinical consensus guideline 1.

• Additional considerations in the management of carotid body tumors with suspected endocrine activity include:
  • The potential for baroreflex failure in patients with bilateral carotid body tumors
  • The risk of cranial neuropathies and other complications associated with surgical resection
  • The importance of long-term follow-up to monitor for recurrence and potential metastases, as recommended by the European Neuroendocrine Tumor Society expert consensus and recommendations for best practice for typical and atypical pulmonary carcinoids 1

From the Research

Management Approach for Carotid Body Tumor with Suspected Endocrine Activity

The management of carotid body tumors (CBTs) with suspected endocrine activity involves a multidisciplinary approach. Key considerations include:

• Preoperative evaluation to assess the tumor's size, relationship with the carotid arteries, and potential for endocrine activity 2
• Diagnostic modalities such as color-coded ultrasound (CCU), Somatostatin receptor scintigraphy (SRS), and dynamic contrast-enhanced ultrasound (CEUS) to evaluate the tumor and its extent 3, 4
• Surgical resection as the primary treatment option, with consideration for preoperative embolization to reduce bleeding and improve surgical outcomes 5, 6

Diagnostic Considerations

Diagnostic approaches for CBTs with suspected endocrine activity include:

• CCU and SRS to evaluate tumor size and extent, as well as to identify potential metastases or multicentricity 3
• CEUS to assess intratumoral microcirculation and perfusion characteristics 4
• Measurement of serum catecholamine levels to identify functional CBTs 2

Treatment Options

Treatment options for CBTs with suspected endocrine activity include:

• Surgical resection, which may involve reconstruction of the internal carotid artery with an interposition graft 5, 6
• Preoperative embolization to reduce bleeding and improve surgical outcomes 5, 6
• Radiotherapy in cases of confirmed malignancy or partially resectable lesions 6
• Careful preoperative evaluation, measurement of serum catecholamine, treatment for alpha- and beta-adrenergic blockade, and gentle intraoperative manipulation to avoid life-threatening complications 2