Cervical catecholamine-secreting paraganglioma in the pterygopalatine fossa

A 25-year-old man was admitted for examination to determine the cause of hypertension. High levels of noradrenaline in plasma and urine were seen, suggesting that the patient had an adrenal or extra-adrenal pheochromocytoma. However, whole-body scintigraphy using the isotope of [131I] meta-iodo-benzylguanidine (131I-MIBG) failed to find the presence of a catecholamine-secreting tumor. The highest level of noradrenaline was detected in plasma obtained from the left jugular vein after selective venous collections through femoral catheterization. Both the computed tomography and cerebral angiography showed a hen-egg-sized tumor located in the left pterygopalatine. After surgical removal of the tumor, high blood pressure and the levels of noradrenaline in plasma and urine were significantly decreased. Histopathological diagnosis was paraganglioma (catecholamine-secreting paraganglioma). The patient with cervical catecholamine-secreting paraganglioma is the first case where the tumor was isolated and located in the pterygopalatine fossa.