Catecholamine-secreting infratemporal fossa paraganglioma

Extraadrenal paragangliomas are tumors of the paraganglion system, usually arising from the carotid bodies, the glomus jugulare, or the glomus tympanicum. These tumors are capable of secreting catecholamines which can cause severe hypertensive crises. This paper reports a case of a patient who suffered a cerebral vascular accident due to hypertension resulting from a catecholamine-secreting infratemporal fossa paraganglioma. Any patient with a history of paroxysmal hypertension, headaches, and palpitations should be evaluated for a catecholamine-secreting tumor. Diagnosis and management of these tumors is best accomplished by a team including a radiologist, an endocrinologist, an anesthesiologist, a pathologist, an otolaryngologist--head and neck surgeon, and when the tumor invades the cranial cavity, a neurosurgeon. In this case, precise radiographic mapping of the tumor extent and its blood supply, control of hypertension with adrenergic blocking agents, and depletion of catecholamine stores using alpha-methyl-p-tyrosine allowed total extirpation through a craniofacial approach and a successful outcome.