Parapharyngeal ganglioneuroma with neurofibromatosis: an unusual presentation

Ganglioneuroma is a rare benign tumor of the sympathetic nervous system that is seldom localized to the head and neck. Rarer still are cases of functional ganglioneuroma with catecholamine production. We report an unusual case of a large parapharyngeal space ganglioneuroma in a patient with neurofibromatosis that caused an intraoperative hypertensive crisis in the absence of a neuroblastomatous component - hitherto an undocumented complication.

We present a case of functional parapharyngeal ganglioneuroma and review the clinicopathologic and biochemical features of these rare tumors.

Fewer than 40 anecdotal cases of parapharyngeal ganglioneuromas have been reported, and there is a lack of adequate literature on the biology and appropriate treatment approach of these tumors.

Our case illustrates the importance of vigilant preoperative precautions in the management of neurogenic tumors, particularly ganglioneuromas. These usually nonfunctional tumors can result in sudden intraoperative and postoperative hypertensive crises even in the absence of any preoperative autonomic symptoms.