A diagnostic and therapeutic approach to paragangliomas of the larynx

Background: Differentiating paragangliomas from moderately Differentiated n euroendocrine carcinoma in the larynx is a difficult management problem. As the biological behavior of these 2 entities is different, we developed an algorithm for the preoperative diagnosis and treatment of this disease. Design: The sample case from which the algorithm was developed consisted of a 69-year-old man who was transferred to us after tracheostomy and an atte mpt at biopsy for airway obstruction secondary to a vascular mass. Biopsy r esulted in substantial bleeding. Flexible laryngoscopy showed a vascular ma ss of the supraglottis. X computed tomographic scan showed 2 vascular masse s at the carotid bifurcation and in the larynx. An arteriogram confirmed sy nchronous vascular tumors. Results: The arteriogram showed the superior thyroid artery to be the major feeder vessel to this mass, a situation commonly seen in paragangliomas bu t not other neuroendocrine tumors. The presence of synchronous lesions and a vascular mass based on the superior thyroid artery helped differentiate p araganglioma from the other neuroendocrine tumors. As the biological behavi or of paragangliomas is relatively benign, we performed a conservative supr aglottic laryngectomy and excision of the care rid body tumor. Histologic d iagnosis and immunohistochemical analysis confirmed the diagnosis of paraga nglioma. Conclusions: The vascular nature of neuroendocrine tumors prevents preopera tive pathological diagnosis. Radiologic features demonstrating a vascular m ass with a dominant feeder vessel by the superior or inferior thyroid arter y may help in the clinical diagnosis of paragangliomas of the larynx. Since paragangliomas are rarely malignant, a conservative surgical procedure sho uld suffice.