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.
Groblewski
Jan C
JC
Department of Otolaryngology, University of Pittsburgh School of Medicine, PA 15213, USA.
Thekdi
Apurva
A
Carrau
Ricardo L
RL
D000328:Adult; D005260:Female; D006258:Head and Neck Neoplasms / Q000175:diagnosis / Q000378:metabolism* / Q000601:surgery; D006801:Humans; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D009333:Neck / Q000601:surgery; D010235:Paraganglioma / Q000175:diagnosis / Q000378:metabolism* / Q000601:surgery; D014057:Tomography, X-Ray Computed; D014630:Vagus Nerve