Dopamine-secreting adrenal ganglioneuroma presenting with paroxysmal hypertension attacks

Adrenal ganglioneuromas are rare tumors originating from the neural crest tissue of the sympathetic nervous system. The clinical presentation for most patients is asymptomatic, and most of these tumors are hormone silent. We report a case of dopamine-secreting adrenal ganglioneuroma associated with paroxysmal hypertensive attacks in an adult patient. A 46-year-old woman was admitted to our hospital with a 2-month history of right flank pain, and a 2-year history of paroxysmal hypertensive attacks associated with headaches, palpitations, nervousness, and sweating. Abdominal CT and MRI revealed a solid round tumor approximately 4 cm in diameter on the upper pole of the right kidney. Urinary levels of dopamine and homovanillic acid were slightly elevated, although urinary levels of metanephrine and normetanephrine were suppressed. The urinary levels of epinephrine, norepinephrine, and vanillylmandelic acid were within normal limits. Right adrenalectomy was performed for treatment purposes. Histological diagnosis of the tumor was a ganglioneuroma originating from the adrenal medulla. In conclusion, this is a case of dopamine-secreting adrenal ganglioneuroma associated with paroxysmal hypertensive attacks in an adult patient

Carotid body tumors and adrenal pheocchromocytomas in siblings of a turkish family

This is a report of 2 hypertensive siblings with a history of carotid body tumors and subsequent benign adrenal pheochromocytomas [pheos] in a family where the mother had died of possible adrenal carcinoma. The first case was a 35-year-old woman with paroxysmal hypertensive attacks and a right adrenal mass. She had earlier undergone surgery to remove bilateral carotid body tumors. Investigation revealed excessive excretion of catecholamines and their metabolites in the urine. Abdominal MRI and 131I-MIBG scintigraphy revealed a right adrenal tumor. Right adrenalectomy was performed. The second case, the 45-year-old brother of the first case, was found to have a left adrenal mass on abdominal MRI. Catecholamines and their metabolites in the urine were found to be increased. He had also had surgery to remove bilateral carotid body tumors of the neck. Left adrenalectomy was performed. Both siblings showed no evidence of other familial syndromes, such as multiple neoplasia type 2, von Hippel-Lindau disease or neurofibromatosis type 1. Although the combination of familial carotid body tumors and pheo is rare, a patient who remains hypertensive after removal of a carotid body tumor deserves a careful evaluation to exclude pheo. Such tumors may be extra-adrenal or multifocal