Secreting vagal paraganglioma

Paragangliomas are rare tumors that derive from neural crest tissue. Vagal paragangliomas account for only 3% of all head and neck paragangliomas. Patients with vagal paragangliomas typically present with an asymptomatic neck mass and, less frequently, with cranial neuropathies. It is estimated that only 1% to 3% of all head and neck paragangliomas secrete catecholamines. The incidence of secreting vagal paragangliomas is even smaller. The diagnosis of a secreting paraganglioma involves the use of a screening test for serum catecholamines and a 24-hour urinary test for catecholamine metabolites. The identification and staging of these tumors can be performed through the use of MRI and/or CT scans and an octreotide scintigraphy. The mainstay treatment is surgical extirpation; however, preoperative medical blockade is critical to avoid a hypertensive crisis intra-operatively. We present two illustrative cases of secreting vagal paragangliomas involving a complex diagnostic and therapeutic algorithm.